CME: CLINICAL PRACTICE AND ITS BASIS Clinical Medicine Vol 2 No 1 January/February 2002 9 Connective tissue disorders are uncommon conditions of unknown aetiology. They occur more often in women, affect skin and joints and at times also other organs, and are associ- ated with the production of auto- antibodies. They have a genetic back- ground, but are probably induced by a variety of environmental stimuli (Table 1) 1 . This review focuses on cutaneous manifestations. Lupus erythematosus Cutaneous lupus erythematosus (LE) occurs in discoid (DLE), systemic (SLE) and intermediate subacute cutaneous (SACLE) variants. These types are clini- cally similar and histologically indistin- guishable, with similar laboratory abnormalities (albeit to a lesser extent in DLE). However, there is a different age and sex distribution and the genetic associations are different. The risk of transformation of DLE to SLE overall is only 5%, but HLA B8-positive middle- aged women are at greater risk 2 . Discoid lupus erythematosus DLE affects twice as many women as men, mostly in their 30s, and takes a chronic relapsing course. In genetically predisposed individuals, the disease may be precipitated by a variety of environ- mental factors including trauma, stress, infections and particularly ultraviolet light 1 . DLE most commonly involves the face and scalp, but disseminated forms occur (Fig 1), and mucosa and nails may be involved. Red scaly patches heal with scarring and pigmentary change. Removal of the scales reveals horny plugs filling the follicular openings. Scarring alopecia is a problem for a third of patients. Raynaud’s phenomenon and joint pains may occur. Subcutaneous involvement (lupus profundus) and lesions resembling erythema multiforme are unusual presentations. The diagnosis of DLE depends on the characteristic clinical findings of photoexacerbated scaly lesions with follicular plugging and scarring, combined with typical histology of a lymphocytic infiltrate around dermal vessels and appendages and vacuolation of the basal layer of the epidermis. Treatment First-line treatment consists of a sun- block and a potent topical steroid, for example, clobetasol propionate or intralesional steroid injections. Failing this, oral antimalarials alone (usually hydroxychloroquine) or in combination with topical steroids should be tried. The list of third-line treatments is long and poorly evaluated, but includes thalidomide, oral gold, oral retinoids, dapsone and immunosuppressants for severe cases 3 . Systemic lupus erythematosus SLE is a chronic episodic disease, affecting all races but is particularly common in young black American women. It has a peak onset in early adult life and a female to male ratio of 8:1. Diagnostic criteria are summarised in Table 2. There is impaired cell-mediated immunity and an exaggerated humoral response with the production of anti- nuclear antibodies. The role of auto- antibodies in pathogenesis remains unclear. Some antibodies are more disease-specific (Table 3). Anti-Ro anti- bodies are implicated in the development of the characteristic rash seen in subacute cutaneous LE and neonatal LE. They are also responsible for producing the Dermatology Edited by Vanessa Venning DM FRCP Consultant Dermatologist, Churchill Hospital, Oxford, and Peter Mortimer MD FRCP Professor of Dermatological Medicine, St George’s Hospital Medical School, London Sabine Sommer MD MRCP, Specialist Registrar in Dermatology Mark JD Goodfield MD FRCP, Consultant Dermatologist Department of Dermatology, Leeds General Hospital Clin Med JRCPL 2002;2:9–14 Connective tissue disease and the skin Trauma Sunburn Exposure to cold Hormonal changes Mental stress Infection Drugs Chemicals Table 1. Triggers of connective tissue diseases. Connective tissue diseases are triggered by environmental stimuli in genetically predisposed patients Women are more commonly affected than men Skin manifestations are often the presenting feature of a multisystem disorder Diagnosis and classification are not always easy; incomplete, as well as overlap, syndromes exist Treatments are not curative, but disease control may be achieved by topical or systemic immune modulatory drugs Key Points KEY WORDS: CPD, lupus, scleroderma, dermatomyositis, cutaneous disease
Transcript
CME CLINICAL PRACTICE AND ITS BASIS
Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002 9
Connective tissue disorders areuncommon conditions of unknownaetiology They occur more often inwomen affect skin and joints and attimes also other organs and are associ-ated with the production of auto-antibodies They have a genetic back-ground but are probably induced by avariety of environmental stimuli(Table 1)1 This review focuses oncutaneous manifestations
(SACLE) variants These types are clini-cally similar and histologically indistin-guishable with similar laboratory abnormalities (albeit to a lesser extent inDLE) However there is a different ageand sex distribution and the geneticassociations are different The risk oftransformation of DLE to SLE overall isonly 5 but HLA B8-positive middle-aged women are at greater risk2
Discoid lupus erythematosus
DLE affects twice as many women asmen mostly in their 30s and takes achronic relapsing course In geneticallypredisposed individuals the disease maybe precipitated by a variety of environ-mental factors including trauma stressinfections and particularly ultravioletlight1
DLE most commonly involves the faceand scalp but disseminated forms occur(Fig 1) and mucosa and nails may beinvolved Red scaly patches heal withscarring and pigmentary changeRemoval of the scales reveals horny plugsfilling the follicular openings Scarringalopecia is a problem for a third ofpatients Raynaudrsquos phenomenon andjoint pains may occur Subcutaneousinvolvement (lupus profundus) andlesions resembling erythema multiformeare unusual presentations
The diagnosis of DLE depends on thecharacteristic clinical findings ofphotoexacerbated scaly lesions withfollicular plugging and scarringcombined with typical histology of alymphocytic infiltrate around dermalvessels and appendages and vacuolationof the basal layer of the epidermis
Treatment
First-line treatment consists of a sun-block and a potent topical steroid forexample clobetasol propionate orintralesional steroid injections Failingthis oral antimalarials alone (usuallyhydroxychloroquine) or in combinationwith topical steroids should be tried Thelist of third-line treatments is long andpoorly evaluated but includesthalidomide oral gold oral retinoidsdapsone and immunosuppressants forsevere cases3
Systemic lupus erythematosus
SLE is a chronic episodic diseaseaffecting all races but is particularlycommon in young black Americanwomen It has a peak onset in early adultlife and a female to male ratio of 81
Diagnostic criteria are summarised inTable 2 There is impaired cell-mediatedimmunity and an exaggerated humoralresponse with the production of anti-nuclear antibodies The role of auto-antibodies in pathogenesis remainsunclear Some antibodies are moredisease-specific (Table 3) Anti-Ro anti-bodies are implicated in the developmentof the characteristic rash seen in subacutecutaneous LE and neonatal LE They arealso responsible for producing the
DermatologyEdited by Vanessa Venning DM FRCP Consultant Dermatologist Churchill Hospital Oxford and Peter Mortimer MD FRCP Professor ofDermatological Medicine St Georgersquos Hospital Medical School London
Sabine Sommer MD MRCP SpecialistRegistrar in Dermatology
Connective tissue diseases are triggered by environmental stimuli in geneticallypredisposed patients
Women are more commonly affected than men
Skin manifestations are often the presenting feature of a multisystem disorder
Diagnosis and classification are not always easy incomplete as well as overlapsyndromes exist
Treatments are not curative but disease control may be achieved by topical orsystemic immune modulatory drugs
Key Points
KEY WORDS CPD lupus scleroderma dermatomyositis cutaneous disease
congenital heart-block seen in thiscondition4 In addition to the sameexacerbating factors as seen in DLEthere is also a drug-induced form
Skin involvement occurs in 80 ofpatients with SLE and is the presentingsign of disease in up to 25 of patients5The commonest skin sign is a photo-sensitive facial erythema with finescaling (the so-called butterfly rash) (Fig2) but the presentation may be diverse(Table 4)6
Treatment
Photoprotection and medications usedto treat DLE may also be useful in SLEalthough antimalarials are generally lesseffective The management of the skin inSLE is usually dictated by the need tocontrol the systemic manifestations ofthe disease
Intermediate subacutecutaneous variants7
There are two well recognised patterns ofskin disease in this subtype The firstand most common has red annularlesions with central clearing Lesions areusually made worse by light and 75 ofpatients are Ro-antibody positive Thesecond variant is indistinguishable fromwidespread DLE Ro-antibodies tend tobe positive patients may have associatedarthritis but rarely develop severeinternal organ disease
Treatment
Sun protection is critical in the manage-ment and lesions respond rapidly to oralsteroids or dapsone7
The antiphospholipid antibodysyndrome8
About 23 of patients with SLE haveantiphospholipid antibodies some ofwhich interfere with components of the coagulation cascade (the so-calledlsquolupus anticoagulantrsquo) although similarantibodies occur in patients withoutSLE A proportion of patients with thelupus anticoagulant develop the anti-phospholipid syndrome with paradoxical
10 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
CME Dermatology
Fig 1 Disseminated erythematosus squamous plaques of discoidlupus erythematosus
10 Immunological disorder LE cells false +ve syphilis serology ( 6 month) anti-DNA or anti-Sm antibody
11 Antinuclear antibodies
LE = lupus erythematosus
Table 2 American Rheumatism Association criteria for diagnosis of systemic lupuserythematosus (four of these features are necessary to diagnose SLE)
Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002 11
CME Dermatology
thrombotic episodes In the skin thesemay present as livedo reticularisthrombophlebitis ulcers and skininfarcts or gangrene
Scleroderma
Sclerosis of the skin (scleroderma) is asymptom not a diagnosis Cutaneoussclerosis is a characteristic feature ofprogressive systemic sclerosis but alsooccurs in several other settings (Table 5)
Ro (SSA) CentromereANAANF Nuclear RNP La (SSB) Sm (ENA) () Other
DLE 35 (homogeneous Rarespeckled)
SACLE 80 (homogeneous 75speckled)
SLE 100 (homogeneous Sometimes 30 6 Drug-induced casesspeckled) histones 50ndash70
double-stranded DNA ISystemic sclerosis 90 (speckled nucleolar) 50 20 Topoisomerase
(SCl 70)
Mixed connective tissue disease 100 (speckled) High titre diagnostic 100 high titre 6
Dermatomyositis Sometimes (speckled) Sometimes 20 Jo1
DLE = discoid lupus erythematosus SACLE = subacute cutaneous lupuserythematosus
Table 4 Cutaneous features of systemiclupus erythematosus in 73 patients6
Scleroderma produces indurated hardshiny tethered skin with loss ofappendages (ie hair and sweat glands)
Morphoea
Morphoea or localised sclerodermausually presents as a plaque of induratedmauve skin which later becomes waxyand either pale or hyperpigmented withabsent hair and sweating (Fig 3) Youngadults aged 20ndash40 are affected with athreefold preponderance in women Thecause is unknown but may be provokedby trauma or infection
Linear morphoea may involve sub-cutaneous tissues producing atrophy asin en coup de sabre morphoea Extensivemorphoea may mimic system sclerosisbut differs in the absence of systemicdisease Raynaudrsquos phenomenon andhand lesions Autoantibodies are usuallynegative Plaques around joints cancause contractures and impairedmobility
Treatment
Plaque morphoea lesions may improvespontaneously but local steroids topicaland oral vitamin D analogues local orsystemic psoralen ultraviolet A (PUVA)light therapy can be used
Eosinophilic fasciitis9
Eosinophilic fasciitis a scleroderma-likesyndrome appears to be a separateentity It presents with pain and swellingof the distal limbs followed by deepinduration often accompanied byeosinophilia both within the lesion andin the blood Biopsy shows dermalsclerosis with inflammation and fibrosisof the deep fascia Spontaneousremissions occur and systemic steroidsare helpful
Systemic sclerosis
Diagnostic criteria of this rare multi-system disorder are outlined in Table 610
The female to male ratio is between 3and 61 with a peak onset in the 40sThere is debate over the target tissue butthe vascular endothelial cell appears tobe involved early in the disease leadingto vascular occlusion tissue ischaemiaand fibrosis due to increased collagenformation The observed autoimmunitymay be a primary abnormality or due tocell damage Antinuclear antibodies arealmost invariably present and extractablenuclear antigens occur Anti-centromereantibodies occur in patients withlsquolimitedrsquo disease in whom sclerosis tendsto be peripheral and internal organinvolvement uncommon SCl 70 (anti-topoisomerase) occurs with severedisease
12 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
Occupational exposure eg PVC organic solvents (perchlorethylene used in dry cleaning)
Iatrogenic eg chronic GVH disease bleomycin
GVH = graft versus host disease PVC = polyvinyl chloride
Table 5 Causes of cutaneous sclerosis
Fig 3 Plaque of morphoea showing central white atrophy and surrounding mauve border
Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002 13
CME Dermatology
Unlike morphoea the skin involve-ment is generalised and irreversibleInternal organ involvement is variableClinical features include Raynaudrsquosphenomenon early swelling and laterhardening and tightening of the skin ofthe fingers frequently with cutaneouscalcinosis and digital ulceration (Fig 4)Facial involvement produces loss ofexpression lines a beaked nose andrestricted opening of the mouthTelangiectasia and hyper- or hypo-pigmentation are seen Oesophagealinvolvement causes dysphagia andgastro-oesophageal reflux The prognosisdepends on the degree of internal organinvolvement
Treatment
Currently no treatment is known to alterthe outcome of the disease but sympto-matic management is important Oralsteroids penicillamine colchicinevasodilators immunosuppressantsprostacyclin infusions and phototherapyhave all been used with symptomaticbenefit11
Mixed connective tissuedisease12
The presence of the U1-RNP antibodydefines mixed connective tissue diseasewhich has features of SLE systemicsclerosis dermatomyositis and rheuma-toid arthritis Evolution into a definitedisease entity may occur but overallprognosis tends to be good
Criterion Features
Major Scleroderma proximal to the digits or at least 2 of the minor criteria
Minor (i) Sclerodactyly
(ii) Digital pitted scarring
(iii) Bilateral basal pulmonary fibrosis
Table 6 American RheumatismAssociation criteria for the diagnosis ofsystemic sclerosis
Fig 4 Spindling of the fingers and digital infarcts in systemic sclerosis
Fig 5 Gottronrsquos papules overlying knuckles in dermatomyositis
Dermatomyositis
Dermatomyositis has a peak onset before the age of 10 and a second peakbetween 40 and 60 years Mauve-red(lsquoheliotropersquo) is the distinctive colour ofthe rash and oedema is prominentPhotosensitivity frequently producespuffy mauve discolouration of the eye-lids and other areas Flat topped lesionsoverlie the knuckles and other bonyprominences (Gottronrsquos papules) (Fig 5)The nailfolds show dilated capillaryloops and ragged cuticles Less typicalare bullous urticarial or psoriasiformlesions or reticulate telangiectaticerythema with atrophic scarring andpigmentary changes (poikiloderma)Vasculitis and panniculitis can occurwhile calcinosis may follow or occurspontaneously Calcinosis is a good signfor survival but makes functionalrecovery less likely It affects more than50 of children and 15 of adults withdermatomyositis Any or all muscletypes including respiratory and cardiacmay be involved A rheumatoid patternof arthritis and pulmonary fibrosisoccurs particularly in those with anti-Jo1 antibodies
The diagnosis depends upon theassociation of a typical rash with muscleweakness although muscle and skinbiopsy can be normal Electro-myography muscle enzyme levels andmagnetic resonance imaging may behelpful in establishing the presence of
muscle disease Early blood vesseldamage appears to be secondary to thepresence of anti-endothelial cell anti-bodies Circulating immune complexeshave been demonstrated in 70 ofpatients An associated malignancy ispresent in 25ndash40 of patients over40 years it is possible that an abnormalimmunological response to the neo-plasm may cause the disease Childhooddermatomyositis is not associated withneoplasia13 Two-thirds of deaths are dueto underlying malignancy the rest topulmonary or cardiac failure
Treatment
Treatment of the underlying malignancyis essential Systemic steroids azathio-prine and methotrexate are helpful andmay be required for many years Not allpatients respond to therapy
References
1 Rowell NR Goodfield MJD The lsquoconnec-tive tissue diseasesrsquo In Champion RHBurton JL Burns DA Breathnach SM (eds)Textbook of dermatology 6th edn OxfordBlackwell Science 19982437ndash76
2 Scott A Rees EG The relationship of sys-temic lupus erythematosus and discoidlupus erythematosus Arch Dermatol 195979422ndash35
4 Petri M Watson R Hochberg MC Anti-Roantibodies and neonatal lupus Review
Rheum Dis Clin North Am 198915335ndash605 Tuffanelli DL Dubois EL Cutaneous man-
ifestations of systemic lupus erythematosusArch Dermatol 196490377ndash86
6 Yell JA Mbuagbaw J Burge SM Cutaneousmanifestations of systemic lupus erythe-matosus Br J Dermatol 1996135355ndash62
7 Callen JP Klein J Subacute cutaneous lupuserythematosus Clinical serologicimmunogenetic and therapeutic consider-ations in seventy-two patients ArthritisRheum 1988311007ndash13
8 Khamashta MA Hughes GR Anti-phospholipid syndrome Br Med J 1993307883ndash4
9 Bennett RM Herron A Keogh LEosinophilic fasciitis Case report andreview of the literature Ann Rheum Dis197736354ndash9
10 Preliminary criteria for the classification ofsystemic sclerosis (scleroderma) Sub-committee for scleroderma criteria of theAmerican Rheumatism AssociationDiagnostic and Therapeutic CriteriaCommittee Arthritis Rheum 198023581ndash90
11 Alarcon-Segovia D Ibanez G KershenobichD Rojkind M Letter Treatment ofscleroderma Lancet 1974i1054ndash5
12 Rowell NR Overlap in the connective tissuediseases Semin Dermatol 19854136ndash42
13 Maugars YM Berthelot JM Abbas AAMussini JM et al Long term prognosis of 69 patients with dermatomyositis orpolymyositis Clin Exp Rheumatol 199614263ndash74
Address for correspondence Dr Sabine Sommer Specialist Registrar GeneralInfirmary Great George Street Leeds LS1 3EX
14 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
CME Dermatology
congenital heart-block seen in thiscondition4 In addition to the sameexacerbating factors as seen in DLEthere is also a drug-induced form
Skin involvement occurs in 80 ofpatients with SLE and is the presentingsign of disease in up to 25 of patients5The commonest skin sign is a photo-sensitive facial erythema with finescaling (the so-called butterfly rash) (Fig2) but the presentation may be diverse(Table 4)6
Treatment
Photoprotection and medications usedto treat DLE may also be useful in SLEalthough antimalarials are generally lesseffective The management of the skin inSLE is usually dictated by the need tocontrol the systemic manifestations ofthe disease
Intermediate subacutecutaneous variants7
There are two well recognised patterns ofskin disease in this subtype The firstand most common has red annularlesions with central clearing Lesions areusually made worse by light and 75 ofpatients are Ro-antibody positive Thesecond variant is indistinguishable fromwidespread DLE Ro-antibodies tend tobe positive patients may have associatedarthritis but rarely develop severeinternal organ disease
Treatment
Sun protection is critical in the manage-ment and lesions respond rapidly to oralsteroids or dapsone7
The antiphospholipid antibodysyndrome8
About 23 of patients with SLE haveantiphospholipid antibodies some ofwhich interfere with components of the coagulation cascade (the so-calledlsquolupus anticoagulantrsquo) although similarantibodies occur in patients withoutSLE A proportion of patients with thelupus anticoagulant develop the anti-phospholipid syndrome with paradoxical
10 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
CME Dermatology
Fig 1 Disseminated erythematosus squamous plaques of discoidlupus erythematosus
10 Immunological disorder LE cells false +ve syphilis serology ( 6 month) anti-DNA or anti-Sm antibody
11 Antinuclear antibodies
LE = lupus erythematosus
Table 2 American Rheumatism Association criteria for diagnosis of systemic lupuserythematosus (four of these features are necessary to diagnose SLE)
Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002 11
CME Dermatology
thrombotic episodes In the skin thesemay present as livedo reticularisthrombophlebitis ulcers and skininfarcts or gangrene
Scleroderma
Sclerosis of the skin (scleroderma) is asymptom not a diagnosis Cutaneoussclerosis is a characteristic feature ofprogressive systemic sclerosis but alsooccurs in several other settings (Table 5)
Ro (SSA) CentromereANAANF Nuclear RNP La (SSB) Sm (ENA) () Other
DLE 35 (homogeneous Rarespeckled)
SACLE 80 (homogeneous 75speckled)
SLE 100 (homogeneous Sometimes 30 6 Drug-induced casesspeckled) histones 50ndash70
double-stranded DNA ISystemic sclerosis 90 (speckled nucleolar) 50 20 Topoisomerase
(SCl 70)
Mixed connective tissue disease 100 (speckled) High titre diagnostic 100 high titre 6
Dermatomyositis Sometimes (speckled) Sometimes 20 Jo1
DLE = discoid lupus erythematosus SACLE = subacute cutaneous lupuserythematosus
Table 4 Cutaneous features of systemiclupus erythematosus in 73 patients6
Scleroderma produces indurated hardshiny tethered skin with loss ofappendages (ie hair and sweat glands)
Morphoea
Morphoea or localised sclerodermausually presents as a plaque of induratedmauve skin which later becomes waxyand either pale or hyperpigmented withabsent hair and sweating (Fig 3) Youngadults aged 20ndash40 are affected with athreefold preponderance in women Thecause is unknown but may be provokedby trauma or infection
Linear morphoea may involve sub-cutaneous tissues producing atrophy asin en coup de sabre morphoea Extensivemorphoea may mimic system sclerosisbut differs in the absence of systemicdisease Raynaudrsquos phenomenon andhand lesions Autoantibodies are usuallynegative Plaques around joints cancause contractures and impairedmobility
Treatment
Plaque morphoea lesions may improvespontaneously but local steroids topicaland oral vitamin D analogues local orsystemic psoralen ultraviolet A (PUVA)light therapy can be used
Eosinophilic fasciitis9
Eosinophilic fasciitis a scleroderma-likesyndrome appears to be a separateentity It presents with pain and swellingof the distal limbs followed by deepinduration often accompanied byeosinophilia both within the lesion andin the blood Biopsy shows dermalsclerosis with inflammation and fibrosisof the deep fascia Spontaneousremissions occur and systemic steroidsare helpful
Systemic sclerosis
Diagnostic criteria of this rare multi-system disorder are outlined in Table 610
The female to male ratio is between 3and 61 with a peak onset in the 40sThere is debate over the target tissue butthe vascular endothelial cell appears tobe involved early in the disease leadingto vascular occlusion tissue ischaemiaand fibrosis due to increased collagenformation The observed autoimmunitymay be a primary abnormality or due tocell damage Antinuclear antibodies arealmost invariably present and extractablenuclear antigens occur Anti-centromereantibodies occur in patients withlsquolimitedrsquo disease in whom sclerosis tendsto be peripheral and internal organinvolvement uncommon SCl 70 (anti-topoisomerase) occurs with severedisease
12 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
Occupational exposure eg PVC organic solvents (perchlorethylene used in dry cleaning)
Iatrogenic eg chronic GVH disease bleomycin
GVH = graft versus host disease PVC = polyvinyl chloride
Table 5 Causes of cutaneous sclerosis
Fig 3 Plaque of morphoea showing central white atrophy and surrounding mauve border
Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002 13
CME Dermatology
Unlike morphoea the skin involve-ment is generalised and irreversibleInternal organ involvement is variableClinical features include Raynaudrsquosphenomenon early swelling and laterhardening and tightening of the skin ofthe fingers frequently with cutaneouscalcinosis and digital ulceration (Fig 4)Facial involvement produces loss ofexpression lines a beaked nose andrestricted opening of the mouthTelangiectasia and hyper- or hypo-pigmentation are seen Oesophagealinvolvement causes dysphagia andgastro-oesophageal reflux The prognosisdepends on the degree of internal organinvolvement
Treatment
Currently no treatment is known to alterthe outcome of the disease but sympto-matic management is important Oralsteroids penicillamine colchicinevasodilators immunosuppressantsprostacyclin infusions and phototherapyhave all been used with symptomaticbenefit11
Mixed connective tissuedisease12
The presence of the U1-RNP antibodydefines mixed connective tissue diseasewhich has features of SLE systemicsclerosis dermatomyositis and rheuma-toid arthritis Evolution into a definitedisease entity may occur but overallprognosis tends to be good
Criterion Features
Major Scleroderma proximal to the digits or at least 2 of the minor criteria
Minor (i) Sclerodactyly
(ii) Digital pitted scarring
(iii) Bilateral basal pulmonary fibrosis
Table 6 American RheumatismAssociation criteria for the diagnosis ofsystemic sclerosis
Fig 4 Spindling of the fingers and digital infarcts in systemic sclerosis
Fig 5 Gottronrsquos papules overlying knuckles in dermatomyositis
Dermatomyositis
Dermatomyositis has a peak onset before the age of 10 and a second peakbetween 40 and 60 years Mauve-red(lsquoheliotropersquo) is the distinctive colour ofthe rash and oedema is prominentPhotosensitivity frequently producespuffy mauve discolouration of the eye-lids and other areas Flat topped lesionsoverlie the knuckles and other bonyprominences (Gottronrsquos papules) (Fig 5)The nailfolds show dilated capillaryloops and ragged cuticles Less typicalare bullous urticarial or psoriasiformlesions or reticulate telangiectaticerythema with atrophic scarring andpigmentary changes (poikiloderma)Vasculitis and panniculitis can occurwhile calcinosis may follow or occurspontaneously Calcinosis is a good signfor survival but makes functionalrecovery less likely It affects more than50 of children and 15 of adults withdermatomyositis Any or all muscletypes including respiratory and cardiacmay be involved A rheumatoid patternof arthritis and pulmonary fibrosisoccurs particularly in those with anti-Jo1 antibodies
The diagnosis depends upon theassociation of a typical rash with muscleweakness although muscle and skinbiopsy can be normal Electro-myography muscle enzyme levels andmagnetic resonance imaging may behelpful in establishing the presence of
muscle disease Early blood vesseldamage appears to be secondary to thepresence of anti-endothelial cell anti-bodies Circulating immune complexeshave been demonstrated in 70 ofpatients An associated malignancy ispresent in 25ndash40 of patients over40 years it is possible that an abnormalimmunological response to the neo-plasm may cause the disease Childhooddermatomyositis is not associated withneoplasia13 Two-thirds of deaths are dueto underlying malignancy the rest topulmonary or cardiac failure
Treatment
Treatment of the underlying malignancyis essential Systemic steroids azathio-prine and methotrexate are helpful andmay be required for many years Not allpatients respond to therapy
References
1 Rowell NR Goodfield MJD The lsquoconnec-tive tissue diseasesrsquo In Champion RHBurton JL Burns DA Breathnach SM (eds)Textbook of dermatology 6th edn OxfordBlackwell Science 19982437ndash76
2 Scott A Rees EG The relationship of sys-temic lupus erythematosus and discoidlupus erythematosus Arch Dermatol 195979422ndash35
4 Petri M Watson R Hochberg MC Anti-Roantibodies and neonatal lupus Review
Rheum Dis Clin North Am 198915335ndash605 Tuffanelli DL Dubois EL Cutaneous man-
ifestations of systemic lupus erythematosusArch Dermatol 196490377ndash86
6 Yell JA Mbuagbaw J Burge SM Cutaneousmanifestations of systemic lupus erythe-matosus Br J Dermatol 1996135355ndash62
7 Callen JP Klein J Subacute cutaneous lupuserythematosus Clinical serologicimmunogenetic and therapeutic consider-ations in seventy-two patients ArthritisRheum 1988311007ndash13
8 Khamashta MA Hughes GR Anti-phospholipid syndrome Br Med J 1993307883ndash4
9 Bennett RM Herron A Keogh LEosinophilic fasciitis Case report andreview of the literature Ann Rheum Dis197736354ndash9
10 Preliminary criteria for the classification ofsystemic sclerosis (scleroderma) Sub-committee for scleroderma criteria of theAmerican Rheumatism AssociationDiagnostic and Therapeutic CriteriaCommittee Arthritis Rheum 198023581ndash90
11 Alarcon-Segovia D Ibanez G KershenobichD Rojkind M Letter Treatment ofscleroderma Lancet 1974i1054ndash5
12 Rowell NR Overlap in the connective tissuediseases Semin Dermatol 19854136ndash42
13 Maugars YM Berthelot JM Abbas AAMussini JM et al Long term prognosis of 69 patients with dermatomyositis orpolymyositis Clin Exp Rheumatol 199614263ndash74
Address for correspondence Dr Sabine Sommer Specialist Registrar GeneralInfirmary Great George Street Leeds LS1 3EX
14 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
CME Dermatology
Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002 11
CME Dermatology
thrombotic episodes In the skin thesemay present as livedo reticularisthrombophlebitis ulcers and skininfarcts or gangrene
Scleroderma
Sclerosis of the skin (scleroderma) is asymptom not a diagnosis Cutaneoussclerosis is a characteristic feature ofprogressive systemic sclerosis but alsooccurs in several other settings (Table 5)
Ro (SSA) CentromereANAANF Nuclear RNP La (SSB) Sm (ENA) () Other
DLE 35 (homogeneous Rarespeckled)
SACLE 80 (homogeneous 75speckled)
SLE 100 (homogeneous Sometimes 30 6 Drug-induced casesspeckled) histones 50ndash70
double-stranded DNA ISystemic sclerosis 90 (speckled nucleolar) 50 20 Topoisomerase
(SCl 70)
Mixed connective tissue disease 100 (speckled) High titre diagnostic 100 high titre 6
Dermatomyositis Sometimes (speckled) Sometimes 20 Jo1
DLE = discoid lupus erythematosus SACLE = subacute cutaneous lupuserythematosus
Table 4 Cutaneous features of systemiclupus erythematosus in 73 patients6
Scleroderma produces indurated hardshiny tethered skin with loss ofappendages (ie hair and sweat glands)
Morphoea
Morphoea or localised sclerodermausually presents as a plaque of induratedmauve skin which later becomes waxyand either pale or hyperpigmented withabsent hair and sweating (Fig 3) Youngadults aged 20ndash40 are affected with athreefold preponderance in women Thecause is unknown but may be provokedby trauma or infection
Linear morphoea may involve sub-cutaneous tissues producing atrophy asin en coup de sabre morphoea Extensivemorphoea may mimic system sclerosisbut differs in the absence of systemicdisease Raynaudrsquos phenomenon andhand lesions Autoantibodies are usuallynegative Plaques around joints cancause contractures and impairedmobility
Treatment
Plaque morphoea lesions may improvespontaneously but local steroids topicaland oral vitamin D analogues local orsystemic psoralen ultraviolet A (PUVA)light therapy can be used
Eosinophilic fasciitis9
Eosinophilic fasciitis a scleroderma-likesyndrome appears to be a separateentity It presents with pain and swellingof the distal limbs followed by deepinduration often accompanied byeosinophilia both within the lesion andin the blood Biopsy shows dermalsclerosis with inflammation and fibrosisof the deep fascia Spontaneousremissions occur and systemic steroidsare helpful
Systemic sclerosis
Diagnostic criteria of this rare multi-system disorder are outlined in Table 610
The female to male ratio is between 3and 61 with a peak onset in the 40sThere is debate over the target tissue butthe vascular endothelial cell appears tobe involved early in the disease leadingto vascular occlusion tissue ischaemiaand fibrosis due to increased collagenformation The observed autoimmunitymay be a primary abnormality or due tocell damage Antinuclear antibodies arealmost invariably present and extractablenuclear antigens occur Anti-centromereantibodies occur in patients withlsquolimitedrsquo disease in whom sclerosis tendsto be peripheral and internal organinvolvement uncommon SCl 70 (anti-topoisomerase) occurs with severedisease
12 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
Occupational exposure eg PVC organic solvents (perchlorethylene used in dry cleaning)
Iatrogenic eg chronic GVH disease bleomycin
GVH = graft versus host disease PVC = polyvinyl chloride
Table 5 Causes of cutaneous sclerosis
Fig 3 Plaque of morphoea showing central white atrophy and surrounding mauve border
Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002 13
CME Dermatology
Unlike morphoea the skin involve-ment is generalised and irreversibleInternal organ involvement is variableClinical features include Raynaudrsquosphenomenon early swelling and laterhardening and tightening of the skin ofthe fingers frequently with cutaneouscalcinosis and digital ulceration (Fig 4)Facial involvement produces loss ofexpression lines a beaked nose andrestricted opening of the mouthTelangiectasia and hyper- or hypo-pigmentation are seen Oesophagealinvolvement causes dysphagia andgastro-oesophageal reflux The prognosisdepends on the degree of internal organinvolvement
Treatment
Currently no treatment is known to alterthe outcome of the disease but sympto-matic management is important Oralsteroids penicillamine colchicinevasodilators immunosuppressantsprostacyclin infusions and phototherapyhave all been used with symptomaticbenefit11
Mixed connective tissuedisease12
The presence of the U1-RNP antibodydefines mixed connective tissue diseasewhich has features of SLE systemicsclerosis dermatomyositis and rheuma-toid arthritis Evolution into a definitedisease entity may occur but overallprognosis tends to be good
Criterion Features
Major Scleroderma proximal to the digits or at least 2 of the minor criteria
Minor (i) Sclerodactyly
(ii) Digital pitted scarring
(iii) Bilateral basal pulmonary fibrosis
Table 6 American RheumatismAssociation criteria for the diagnosis ofsystemic sclerosis
Fig 4 Spindling of the fingers and digital infarcts in systemic sclerosis
Fig 5 Gottronrsquos papules overlying knuckles in dermatomyositis
Dermatomyositis
Dermatomyositis has a peak onset before the age of 10 and a second peakbetween 40 and 60 years Mauve-red(lsquoheliotropersquo) is the distinctive colour ofthe rash and oedema is prominentPhotosensitivity frequently producespuffy mauve discolouration of the eye-lids and other areas Flat topped lesionsoverlie the knuckles and other bonyprominences (Gottronrsquos papules) (Fig 5)The nailfolds show dilated capillaryloops and ragged cuticles Less typicalare bullous urticarial or psoriasiformlesions or reticulate telangiectaticerythema with atrophic scarring andpigmentary changes (poikiloderma)Vasculitis and panniculitis can occurwhile calcinosis may follow or occurspontaneously Calcinosis is a good signfor survival but makes functionalrecovery less likely It affects more than50 of children and 15 of adults withdermatomyositis Any or all muscletypes including respiratory and cardiacmay be involved A rheumatoid patternof arthritis and pulmonary fibrosisoccurs particularly in those with anti-Jo1 antibodies
The diagnosis depends upon theassociation of a typical rash with muscleweakness although muscle and skinbiopsy can be normal Electro-myography muscle enzyme levels andmagnetic resonance imaging may behelpful in establishing the presence of
muscle disease Early blood vesseldamage appears to be secondary to thepresence of anti-endothelial cell anti-bodies Circulating immune complexeshave been demonstrated in 70 ofpatients An associated malignancy ispresent in 25ndash40 of patients over40 years it is possible that an abnormalimmunological response to the neo-plasm may cause the disease Childhooddermatomyositis is not associated withneoplasia13 Two-thirds of deaths are dueto underlying malignancy the rest topulmonary or cardiac failure
Treatment
Treatment of the underlying malignancyis essential Systemic steroids azathio-prine and methotrexate are helpful andmay be required for many years Not allpatients respond to therapy
References
1 Rowell NR Goodfield MJD The lsquoconnec-tive tissue diseasesrsquo In Champion RHBurton JL Burns DA Breathnach SM (eds)Textbook of dermatology 6th edn OxfordBlackwell Science 19982437ndash76
2 Scott A Rees EG The relationship of sys-temic lupus erythematosus and discoidlupus erythematosus Arch Dermatol 195979422ndash35
4 Petri M Watson R Hochberg MC Anti-Roantibodies and neonatal lupus Review
Rheum Dis Clin North Am 198915335ndash605 Tuffanelli DL Dubois EL Cutaneous man-
ifestations of systemic lupus erythematosusArch Dermatol 196490377ndash86
6 Yell JA Mbuagbaw J Burge SM Cutaneousmanifestations of systemic lupus erythe-matosus Br J Dermatol 1996135355ndash62
7 Callen JP Klein J Subacute cutaneous lupuserythematosus Clinical serologicimmunogenetic and therapeutic consider-ations in seventy-two patients ArthritisRheum 1988311007ndash13
8 Khamashta MA Hughes GR Anti-phospholipid syndrome Br Med J 1993307883ndash4
9 Bennett RM Herron A Keogh LEosinophilic fasciitis Case report andreview of the literature Ann Rheum Dis197736354ndash9
10 Preliminary criteria for the classification ofsystemic sclerosis (scleroderma) Sub-committee for scleroderma criteria of theAmerican Rheumatism AssociationDiagnostic and Therapeutic CriteriaCommittee Arthritis Rheum 198023581ndash90
11 Alarcon-Segovia D Ibanez G KershenobichD Rojkind M Letter Treatment ofscleroderma Lancet 1974i1054ndash5
12 Rowell NR Overlap in the connective tissuediseases Semin Dermatol 19854136ndash42
13 Maugars YM Berthelot JM Abbas AAMussini JM et al Long term prognosis of 69 patients with dermatomyositis orpolymyositis Clin Exp Rheumatol 199614263ndash74
Address for correspondence Dr Sabine Sommer Specialist Registrar GeneralInfirmary Great George Street Leeds LS1 3EX
14 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
CME Dermatology
Scleroderma produces indurated hardshiny tethered skin with loss ofappendages (ie hair and sweat glands)
Morphoea
Morphoea or localised sclerodermausually presents as a plaque of induratedmauve skin which later becomes waxyand either pale or hyperpigmented withabsent hair and sweating (Fig 3) Youngadults aged 20ndash40 are affected with athreefold preponderance in women Thecause is unknown but may be provokedby trauma or infection
Linear morphoea may involve sub-cutaneous tissues producing atrophy asin en coup de sabre morphoea Extensivemorphoea may mimic system sclerosisbut differs in the absence of systemicdisease Raynaudrsquos phenomenon andhand lesions Autoantibodies are usuallynegative Plaques around joints cancause contractures and impairedmobility
Treatment
Plaque morphoea lesions may improvespontaneously but local steroids topicaland oral vitamin D analogues local orsystemic psoralen ultraviolet A (PUVA)light therapy can be used
Eosinophilic fasciitis9
Eosinophilic fasciitis a scleroderma-likesyndrome appears to be a separateentity It presents with pain and swellingof the distal limbs followed by deepinduration often accompanied byeosinophilia both within the lesion andin the blood Biopsy shows dermalsclerosis with inflammation and fibrosisof the deep fascia Spontaneousremissions occur and systemic steroidsare helpful
Systemic sclerosis
Diagnostic criteria of this rare multi-system disorder are outlined in Table 610
The female to male ratio is between 3and 61 with a peak onset in the 40sThere is debate over the target tissue butthe vascular endothelial cell appears tobe involved early in the disease leadingto vascular occlusion tissue ischaemiaand fibrosis due to increased collagenformation The observed autoimmunitymay be a primary abnormality or due tocell damage Antinuclear antibodies arealmost invariably present and extractablenuclear antigens occur Anti-centromereantibodies occur in patients withlsquolimitedrsquo disease in whom sclerosis tendsto be peripheral and internal organinvolvement uncommon SCl 70 (anti-topoisomerase) occurs with severedisease
12 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
Occupational exposure eg PVC organic solvents (perchlorethylene used in dry cleaning)
Iatrogenic eg chronic GVH disease bleomycin
GVH = graft versus host disease PVC = polyvinyl chloride
Table 5 Causes of cutaneous sclerosis
Fig 3 Plaque of morphoea showing central white atrophy and surrounding mauve border
Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002 13
CME Dermatology
Unlike morphoea the skin involve-ment is generalised and irreversibleInternal organ involvement is variableClinical features include Raynaudrsquosphenomenon early swelling and laterhardening and tightening of the skin ofthe fingers frequently with cutaneouscalcinosis and digital ulceration (Fig 4)Facial involvement produces loss ofexpression lines a beaked nose andrestricted opening of the mouthTelangiectasia and hyper- or hypo-pigmentation are seen Oesophagealinvolvement causes dysphagia andgastro-oesophageal reflux The prognosisdepends on the degree of internal organinvolvement
Treatment
Currently no treatment is known to alterthe outcome of the disease but sympto-matic management is important Oralsteroids penicillamine colchicinevasodilators immunosuppressantsprostacyclin infusions and phototherapyhave all been used with symptomaticbenefit11
Mixed connective tissuedisease12
The presence of the U1-RNP antibodydefines mixed connective tissue diseasewhich has features of SLE systemicsclerosis dermatomyositis and rheuma-toid arthritis Evolution into a definitedisease entity may occur but overallprognosis tends to be good
Criterion Features
Major Scleroderma proximal to the digits or at least 2 of the minor criteria
Minor (i) Sclerodactyly
(ii) Digital pitted scarring
(iii) Bilateral basal pulmonary fibrosis
Table 6 American RheumatismAssociation criteria for the diagnosis ofsystemic sclerosis
Fig 4 Spindling of the fingers and digital infarcts in systemic sclerosis
Fig 5 Gottronrsquos papules overlying knuckles in dermatomyositis
Dermatomyositis
Dermatomyositis has a peak onset before the age of 10 and a second peakbetween 40 and 60 years Mauve-red(lsquoheliotropersquo) is the distinctive colour ofthe rash and oedema is prominentPhotosensitivity frequently producespuffy mauve discolouration of the eye-lids and other areas Flat topped lesionsoverlie the knuckles and other bonyprominences (Gottronrsquos papules) (Fig 5)The nailfolds show dilated capillaryloops and ragged cuticles Less typicalare bullous urticarial or psoriasiformlesions or reticulate telangiectaticerythema with atrophic scarring andpigmentary changes (poikiloderma)Vasculitis and panniculitis can occurwhile calcinosis may follow or occurspontaneously Calcinosis is a good signfor survival but makes functionalrecovery less likely It affects more than50 of children and 15 of adults withdermatomyositis Any or all muscletypes including respiratory and cardiacmay be involved A rheumatoid patternof arthritis and pulmonary fibrosisoccurs particularly in those with anti-Jo1 antibodies
The diagnosis depends upon theassociation of a typical rash with muscleweakness although muscle and skinbiopsy can be normal Electro-myography muscle enzyme levels andmagnetic resonance imaging may behelpful in establishing the presence of
muscle disease Early blood vesseldamage appears to be secondary to thepresence of anti-endothelial cell anti-bodies Circulating immune complexeshave been demonstrated in 70 ofpatients An associated malignancy ispresent in 25ndash40 of patients over40 years it is possible that an abnormalimmunological response to the neo-plasm may cause the disease Childhooddermatomyositis is not associated withneoplasia13 Two-thirds of deaths are dueto underlying malignancy the rest topulmonary or cardiac failure
Treatment
Treatment of the underlying malignancyis essential Systemic steroids azathio-prine and methotrexate are helpful andmay be required for many years Not allpatients respond to therapy
References
1 Rowell NR Goodfield MJD The lsquoconnec-tive tissue diseasesrsquo In Champion RHBurton JL Burns DA Breathnach SM (eds)Textbook of dermatology 6th edn OxfordBlackwell Science 19982437ndash76
2 Scott A Rees EG The relationship of sys-temic lupus erythematosus and discoidlupus erythematosus Arch Dermatol 195979422ndash35
4 Petri M Watson R Hochberg MC Anti-Roantibodies and neonatal lupus Review
Rheum Dis Clin North Am 198915335ndash605 Tuffanelli DL Dubois EL Cutaneous man-
ifestations of systemic lupus erythematosusArch Dermatol 196490377ndash86
6 Yell JA Mbuagbaw J Burge SM Cutaneousmanifestations of systemic lupus erythe-matosus Br J Dermatol 1996135355ndash62
7 Callen JP Klein J Subacute cutaneous lupuserythematosus Clinical serologicimmunogenetic and therapeutic consider-ations in seventy-two patients ArthritisRheum 1988311007ndash13
8 Khamashta MA Hughes GR Anti-phospholipid syndrome Br Med J 1993307883ndash4
9 Bennett RM Herron A Keogh LEosinophilic fasciitis Case report andreview of the literature Ann Rheum Dis197736354ndash9
10 Preliminary criteria for the classification ofsystemic sclerosis (scleroderma) Sub-committee for scleroderma criteria of theAmerican Rheumatism AssociationDiagnostic and Therapeutic CriteriaCommittee Arthritis Rheum 198023581ndash90
11 Alarcon-Segovia D Ibanez G KershenobichD Rojkind M Letter Treatment ofscleroderma Lancet 1974i1054ndash5
12 Rowell NR Overlap in the connective tissuediseases Semin Dermatol 19854136ndash42
13 Maugars YM Berthelot JM Abbas AAMussini JM et al Long term prognosis of 69 patients with dermatomyositis orpolymyositis Clin Exp Rheumatol 199614263ndash74
Address for correspondence Dr Sabine Sommer Specialist Registrar GeneralInfirmary Great George Street Leeds LS1 3EX
14 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
CME Dermatology
Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002 13
CME Dermatology
Unlike morphoea the skin involve-ment is generalised and irreversibleInternal organ involvement is variableClinical features include Raynaudrsquosphenomenon early swelling and laterhardening and tightening of the skin ofthe fingers frequently with cutaneouscalcinosis and digital ulceration (Fig 4)Facial involvement produces loss ofexpression lines a beaked nose andrestricted opening of the mouthTelangiectasia and hyper- or hypo-pigmentation are seen Oesophagealinvolvement causes dysphagia andgastro-oesophageal reflux The prognosisdepends on the degree of internal organinvolvement
Treatment
Currently no treatment is known to alterthe outcome of the disease but sympto-matic management is important Oralsteroids penicillamine colchicinevasodilators immunosuppressantsprostacyclin infusions and phototherapyhave all been used with symptomaticbenefit11
Mixed connective tissuedisease12
The presence of the U1-RNP antibodydefines mixed connective tissue diseasewhich has features of SLE systemicsclerosis dermatomyositis and rheuma-toid arthritis Evolution into a definitedisease entity may occur but overallprognosis tends to be good
Criterion Features
Major Scleroderma proximal to the digits or at least 2 of the minor criteria
Minor (i) Sclerodactyly
(ii) Digital pitted scarring
(iii) Bilateral basal pulmonary fibrosis
Table 6 American RheumatismAssociation criteria for the diagnosis ofsystemic sclerosis
Fig 4 Spindling of the fingers and digital infarcts in systemic sclerosis
Fig 5 Gottronrsquos papules overlying knuckles in dermatomyositis
Dermatomyositis
Dermatomyositis has a peak onset before the age of 10 and a second peakbetween 40 and 60 years Mauve-red(lsquoheliotropersquo) is the distinctive colour ofthe rash and oedema is prominentPhotosensitivity frequently producespuffy mauve discolouration of the eye-lids and other areas Flat topped lesionsoverlie the knuckles and other bonyprominences (Gottronrsquos papules) (Fig 5)The nailfolds show dilated capillaryloops and ragged cuticles Less typicalare bullous urticarial or psoriasiformlesions or reticulate telangiectaticerythema with atrophic scarring andpigmentary changes (poikiloderma)Vasculitis and panniculitis can occurwhile calcinosis may follow or occurspontaneously Calcinosis is a good signfor survival but makes functionalrecovery less likely It affects more than50 of children and 15 of adults withdermatomyositis Any or all muscletypes including respiratory and cardiacmay be involved A rheumatoid patternof arthritis and pulmonary fibrosisoccurs particularly in those with anti-Jo1 antibodies
The diagnosis depends upon theassociation of a typical rash with muscleweakness although muscle and skinbiopsy can be normal Electro-myography muscle enzyme levels andmagnetic resonance imaging may behelpful in establishing the presence of
muscle disease Early blood vesseldamage appears to be secondary to thepresence of anti-endothelial cell anti-bodies Circulating immune complexeshave been demonstrated in 70 ofpatients An associated malignancy ispresent in 25ndash40 of patients over40 years it is possible that an abnormalimmunological response to the neo-plasm may cause the disease Childhooddermatomyositis is not associated withneoplasia13 Two-thirds of deaths are dueto underlying malignancy the rest topulmonary or cardiac failure
Treatment
Treatment of the underlying malignancyis essential Systemic steroids azathio-prine and methotrexate are helpful andmay be required for many years Not allpatients respond to therapy
References
1 Rowell NR Goodfield MJD The lsquoconnec-tive tissue diseasesrsquo In Champion RHBurton JL Burns DA Breathnach SM (eds)Textbook of dermatology 6th edn OxfordBlackwell Science 19982437ndash76
2 Scott A Rees EG The relationship of sys-temic lupus erythematosus and discoidlupus erythematosus Arch Dermatol 195979422ndash35
4 Petri M Watson R Hochberg MC Anti-Roantibodies and neonatal lupus Review
Rheum Dis Clin North Am 198915335ndash605 Tuffanelli DL Dubois EL Cutaneous man-
ifestations of systemic lupus erythematosusArch Dermatol 196490377ndash86
6 Yell JA Mbuagbaw J Burge SM Cutaneousmanifestations of systemic lupus erythe-matosus Br J Dermatol 1996135355ndash62
7 Callen JP Klein J Subacute cutaneous lupuserythematosus Clinical serologicimmunogenetic and therapeutic consider-ations in seventy-two patients ArthritisRheum 1988311007ndash13
8 Khamashta MA Hughes GR Anti-phospholipid syndrome Br Med J 1993307883ndash4
9 Bennett RM Herron A Keogh LEosinophilic fasciitis Case report andreview of the literature Ann Rheum Dis197736354ndash9
10 Preliminary criteria for the classification ofsystemic sclerosis (scleroderma) Sub-committee for scleroderma criteria of theAmerican Rheumatism AssociationDiagnostic and Therapeutic CriteriaCommittee Arthritis Rheum 198023581ndash90
11 Alarcon-Segovia D Ibanez G KershenobichD Rojkind M Letter Treatment ofscleroderma Lancet 1974i1054ndash5
12 Rowell NR Overlap in the connective tissuediseases Semin Dermatol 19854136ndash42
13 Maugars YM Berthelot JM Abbas AAMussini JM et al Long term prognosis of 69 patients with dermatomyositis orpolymyositis Clin Exp Rheumatol 199614263ndash74
Address for correspondence Dr Sabine Sommer Specialist Registrar GeneralInfirmary Great George Street Leeds LS1 3EX
14 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
CME Dermatology
Dermatomyositis
Dermatomyositis has a peak onset before the age of 10 and a second peakbetween 40 and 60 years Mauve-red(lsquoheliotropersquo) is the distinctive colour ofthe rash and oedema is prominentPhotosensitivity frequently producespuffy mauve discolouration of the eye-lids and other areas Flat topped lesionsoverlie the knuckles and other bonyprominences (Gottronrsquos papules) (Fig 5)The nailfolds show dilated capillaryloops and ragged cuticles Less typicalare bullous urticarial or psoriasiformlesions or reticulate telangiectaticerythema with atrophic scarring andpigmentary changes (poikiloderma)Vasculitis and panniculitis can occurwhile calcinosis may follow or occurspontaneously Calcinosis is a good signfor survival but makes functionalrecovery less likely It affects more than50 of children and 15 of adults withdermatomyositis Any or all muscletypes including respiratory and cardiacmay be involved A rheumatoid patternof arthritis and pulmonary fibrosisoccurs particularly in those with anti-Jo1 antibodies
The diagnosis depends upon theassociation of a typical rash with muscleweakness although muscle and skinbiopsy can be normal Electro-myography muscle enzyme levels andmagnetic resonance imaging may behelpful in establishing the presence of
muscle disease Early blood vesseldamage appears to be secondary to thepresence of anti-endothelial cell anti-bodies Circulating immune complexeshave been demonstrated in 70 ofpatients An associated malignancy ispresent in 25ndash40 of patients over40 years it is possible that an abnormalimmunological response to the neo-plasm may cause the disease Childhooddermatomyositis is not associated withneoplasia13 Two-thirds of deaths are dueto underlying malignancy the rest topulmonary or cardiac failure
Treatment
Treatment of the underlying malignancyis essential Systemic steroids azathio-prine and methotrexate are helpful andmay be required for many years Not allpatients respond to therapy
References
1 Rowell NR Goodfield MJD The lsquoconnec-tive tissue diseasesrsquo In Champion RHBurton JL Burns DA Breathnach SM (eds)Textbook of dermatology 6th edn OxfordBlackwell Science 19982437ndash76
2 Scott A Rees EG The relationship of sys-temic lupus erythematosus and discoidlupus erythematosus Arch Dermatol 195979422ndash35
4 Petri M Watson R Hochberg MC Anti-Roantibodies and neonatal lupus Review
Rheum Dis Clin North Am 198915335ndash605 Tuffanelli DL Dubois EL Cutaneous man-
ifestations of systemic lupus erythematosusArch Dermatol 196490377ndash86
6 Yell JA Mbuagbaw J Burge SM Cutaneousmanifestations of systemic lupus erythe-matosus Br J Dermatol 1996135355ndash62
7 Callen JP Klein J Subacute cutaneous lupuserythematosus Clinical serologicimmunogenetic and therapeutic consider-ations in seventy-two patients ArthritisRheum 1988311007ndash13
8 Khamashta MA Hughes GR Anti-phospholipid syndrome Br Med J 1993307883ndash4
9 Bennett RM Herron A Keogh LEosinophilic fasciitis Case report andreview of the literature Ann Rheum Dis197736354ndash9
10 Preliminary criteria for the classification ofsystemic sclerosis (scleroderma) Sub-committee for scleroderma criteria of theAmerican Rheumatism AssociationDiagnostic and Therapeutic CriteriaCommittee Arthritis Rheum 198023581ndash90
11 Alarcon-Segovia D Ibanez G KershenobichD Rojkind M Letter Treatment ofscleroderma Lancet 1974i1054ndash5
12 Rowell NR Overlap in the connective tissuediseases Semin Dermatol 19854136ndash42
13 Maugars YM Berthelot JM Abbas AAMussini JM et al Long term prognosis of 69 patients with dermatomyositis orpolymyositis Clin Exp Rheumatol 199614263ndash74
Address for correspondence Dr Sabine Sommer Specialist Registrar GeneralInfirmary Great George Street Leeds LS1 3EX
14 Clinical Medicine Vol 2 No 1 JanuaryFebruary 2002
![DLE-35RA - Hobbico - Hobbico, Inc. - largest U.S ...manuals.hobbico.com/dle/dleg0435-manual.pdfDLE with Manual Choke Main Engine − 2.08lb [947g] ... DLE-35RA Gas Engine with DLE](https://static.documents.pub/doc/80x56/5abf1f1d7f8b9a3a428dcf03/dle-35ra-hobbico-hobbico-inc-largest-us-with-manual-choke-main-engine.jpg)
