SARCOIDOSIS OF THE NOSE By PETER O'BRIEN,F.R.C.S. 1 From Canniesburn Hospital, Glasgow PATIENTS with cutaneous manifestations of sarcoidosis will occasionally seek treatment for cosmetic reasons. This paper is presented with a view to describing the condition as it affects the nose, as a result of some limited experience with four cases. The first case of sarcoidosis mentioned in the literature presented with cutaneous signs. In I889, Ernest Besnier of Paris, described a patient with a skin eruption of the face and hands. The nose was involved "being double its normal volume, of a livid purplish red colour, and shining surface, with dilated sebaceous gland orifices, and shallow erosions in front of the nostrils ". To this condition he gave the name lupus pernio because of its chilbl0ain-like appearance. In I899, Caesar Boeck of Oslo described a patient with a skin eruption of the face and legs. These patches were elevated and of a red colour. The superficial lymph nodes were enlarged and he concluded from the histology of a node biopsy that the condition was due to a proliferation of connective tissue cells. He thus called the condition "multiple benign sarkoid ". Besnier's case is regarded as the same condition, though histological evidence is lacking. Sarcoidosis is a generalised disease which most frequently involves the respiratory tract but also involves the lymphatic system, liver, spleen, gastrointestinal tract, skin and eye. Most cases do not have symptoms and are detected by routine chest radiography, which reveals hilar lymphadenopathy. In J. G. Scadding's monograph Sarcoidosis (x967) the author analysed 275 cases, 5 per cent having cutaneous signs. The skin is involved in women more commonly than in men, the peak incidence being between the ages of 25 and 35 years. The basic histopathology of sarcoidosis is the non-caseating epithelioid cell tubercle. The lesion is made up of numerous aggregations of tubercles composed of mononuclear phagocytes derived from the reticulo-endothelial system. These cell aggregations are often surrounded by a layer of lymphocytes ; occasionally Langhans type giant cells are present. The absence of caseation is the feature which distinguishes the sarcoid tubercle from that of tuberculosis but central necrosis may occur. The lesions can resolve, the tubercles being replaced by masses of hyaline collagen. This histology is essentially that of sarcoidosis in any anatomical site, in fact, sarcoidosis is recognised by the monotonous repetition of these histological characteristics (Pinner, I938). When the skin is involved, all layers tend to be infiltrated up to the epidermis, which is never involved and always remains intact. On the face, the nose and cheek prominences are most often affected. The nose tends to be swollen, especially the tip, dilated veins being prominent on the shiny surface. It is this lesion which tends to resemble the sebaceous rhinophyma with which it can be confused. The cause of the sarcoidosis is still not known. It was thought initially to be a derivative of tuberculosis, but whether it is infective, neoplastic or an autoimmune process has not been determined. Medical treatment, employing corticosteroids and antimalarial drugs has been the only treatment used for the cutaneous sarcoid. Corti- costeroids and corticotrophin are suppressive in their effects ; skin sarcoidosis will clear with prolonged therapy but recurrence is invariable as soon as the suppressive dose is withdrawn. Scadding (I967) states " Suppression of extensive fixed sarcoid such as lupus pernio may require doses large enough to cause serious side effects ". This type of treatment has been advocated for severe disfigurement. Local treatment with hydrocortisone ointment is not effective. Antimalarial drugs such as mepacrine and chloroquin have been used to suppress skin sarcoidosis, but nearly all patients so treated have subsequently relapsed. x Present address: Wessex Centre for Plastic and Maxillo-facial Surgery~ Odstock IrIospital~ Salisbury, Wilts.
Transcript
S A R C O I D O S I S OF THE N O S E
By PETER O'BRIEN, F.R.C.S. 1 From Canniesburn Hospital, Glasgow
PATIENTS with cutaneous manifestations of sarcoidosis will occasionally seek treatment for cosmetic reasons. This paper is presented with a view to describing the condition as it affects the nose, as a result of some limited experience with four cases.
The first case of sarcoidosis mentioned in the literature presented with cutaneous signs. In I889, Ernest Besnier of Paris, described a patient with a skin eruption of the face and hands. The nose was involved "being double its normal volume, of a livid purplish red colour, and shining surface, with dilated sebaceous gland orifices, and shallow erosions in front of the nostrils ". To this condition he gave the name lupus pernio because of its chilbl0ain-like appearance. In I899, Caesar Boeck of Oslo described a patient with a skin eruption of the face and legs. These patches were elevated and of a red colour. The superficial lymph nodes were enlarged and he concluded from the histology of a node biopsy that the condition was due to a proliferation of connective tissue cells. He thus called the condition "multiple benign sarkoid ". Besnier's case is regarded as the same condition, though histological evidence is lacking.
Sarcoidosis is a generalised disease which most frequently involves the respiratory tract but also involves the lymphatic system, liver, spleen, gastrointestinal tract, skin and eye. Most cases do not have symptoms and are detected by routine chest radiography, which reveals hilar lymphadenopathy. In J. G. Scadding's monograph Sarcoidosis (x967) the author analysed 275 cases, 5 per cent having cutaneous signs. The skin is involved in women more commonly than in men, the peak incidence being between the ages of 25 and 35 years.
The basic histopathology of sarcoidosis is the non-caseating epithelioid cell tubercle. The lesion is made up of numerous aggregations of tubercles composed of mononuclear phagocytes derived from the reticulo-endothelial system. These cell aggregations are often surrounded by a layer of lymphocytes ; occasionally Langhans type giant cells are present. The absence of caseation is the feature which distinguishes the sarcoid tubercle from that of tuberculosis but central necrosis may occur. The lesions can resolve, the tubercles being replaced by masses of hyaline collagen. This histology is essentially that of sarcoidosis in any anatomical site, in fact, sarcoidosis is recognised by the monotonous repetition of these histological characteristics (Pinner, I938).
When the skin is involved, all layers tend to be infiltrated up to the epidermis, which is never involved and always remains intact. On the face, the nose and cheek prominences are most often affected. The nose tends to be swollen, especially the tip, dilated veins being prominent on the shiny surface. It is this lesion which tends to resemble the sebaceous rhinophyma with which it can be confused.
The cause of the sarcoidosis is still not known. It was thought initially to be a derivative of tuberculosis, but whether it is infective, neoplastic or an autoimmune process has not been determined. Medical treatment, employing corticosteroids and antimalarial drugs has been the only treatment used for the cutaneous sarcoid. Corti- costeroids and corticotrophin are suppressive in their effects ; skin sarcoidosis will clear with prolonged therapy but recurrence is invariable as soon as the suppressive dose is withdrawn. Scadding (I967) states " Suppression of extensive fixed sarcoid such as lupus pernio may require doses large enough to cause serious side effects ". This type of treatment has been advocated for severe disfigurement. Local treatment with hydrocortisone ointment is not effective. Antimalarial drugs such as mepacrine and chloroquin have been used to suppress skin sarcoidosis, but nearly all patients so treated have subsequently relapsed.
x Present address: Wessex Centre for Plastic and Maxillo-facial Surgery~ Odstock IrIospital~ Salisbury, Wilts.
$ARCOIDOSIS OF THE NOSE 243
Case x.--This patient, aged 58 (Figs. I and 2) had sarcoidosis diagnosed 12 years ago, at which time she had skin plaques on the face and legs. The diagnosis was supported by a Kveim test, chest radiography showing hilar adenopathy and confirmed by histological diagnosis
FIG. I
C a s e I .
FIG. 2
Preoperative appearance.
FIG. 3 FIG. 4
Case I. Appearance four months postoperatively.
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from an involved lymph gland. The nose had been treated with X-ray therapy, local steroid injections and a prolonged course of antimalarial drugs with no improvement. At operation, the tip of the nose was cut down in a manner similar to that of a sebaceous rhinophyma, great care being taken to avoid exposing the nasal cartilage or penetrating the nasal wall. This was
FIG. 5 Case I. Pathology.
continued until the nose looked a reasonable size. The raw surface was dressed with tulle gras. Within a week islands of epithelium were evident on the bare area and these continued to grow until the nose was completely covered by new epithelium. Healing was complete in four weeks. Figures 3 and 4 show the appearance four months post-operatively.
The histology is shown in Figure 5. The dermis and subcutaneous tissues are full of the epithelioid cell granulomas containing scanty giant cells, characteristic of sarcoidosis. A lymphocytic infiltration is noted at the periphery of most of the granulomas. The thinned out epidermis is intact.
Case 2 .nThis patient (Figs. 6 and 7) had sarcoidosis of the nose diagnosed clinically in r966. At that time she presented to the dermatology clinic complaining of swelling and
FIG. 6 FIG. 7
Case 2. Preoperative appearance.
SARCOIDOSIS OF THE NOSE 245
discolouration of the tip of the nose. The nasal tip was considerably swollen and of a livid blue colour, and numerous small veins were evident as were the dilated sebaceous gland orifices. The columella was similarly involved being thickened and discoloured. Biopsy of the nasal tip revealed histology of sarcoidosis. In June I967, the nasal tip was pared down to a normal size and a postauricular full thickness skin graft was applied to the defect. A small portion of the graft did not take successfully but healing was complete within three to four weeks. The
final shape of the nose satisfied the patient, but it has been necessary to disguise the livid blue colour showing through the graft with cosmetics. Sections of the excised specimen showed a thin attenuated epidermis overlying the dermis con- taining large infiltrates of pale epithelioid cells. There was no evidence of caseation and giant cells were absent. Lymphocytes were present in narrow
FIG. 8 FIG. 9
Case 2. Appearance two years postoperat ively.
incomplete bands around the islands of epithelioid cells. Since the operation two years ago, several small areas of the graft have been noticed to be a little indurated. The thickening and induration of the columella is still present and a similar area has appeared on the adjoining upper lip (Figs. 8 and 9).
Case 3.--(Figs. Io and I x.) This patient aged 56 had sarcoidosis diagnosed seven years ago when she presented complaining of discolouration of the tip of the nose.
Examination showed the nasal tip to be swollen and indurated. There were enlarged veins over the tip of.the nose and the sebaceous orifices were quite prominent. This infiltration extended well into the columella which was considerably thickened. There was an area similarly affected on the left cheek prominence. The clinical diagnosis of sarcoidosis of the skin was supported by chest radiography, which showed bilateral hilar lymphadenopathy.. Mantoux test was negative on several occasions. Over this seven-year period she was given several courses of antimalarial drugs and steroids which seemed to control the lesions during their active phase, but recurrence of swelling and vascularity was common upon withdrawal of this treatment. A biopsy of the involved area of the left cheek confirmed a diagnosis of sarcoidosis.
As the swelling was not marked it was decided to observe the patient for the time being.
Case 4.--(Figs. i2 and ~3.) This lady aged 4o presented complaining of swelling and discolouration of the nose, progressive over xo years. On examination, the nose was a purple colour, most pronounced at the tip. I t was symmetrically enlarged and indurated, the indura- tion extending along the columella. Numerous dilated veins were present over the nose and spread out on to the adjacent cheek. Unfortunately she refused to attend for further investigation.
246 BRITISH JOURNAL OF PLASTIC SURGERY
FIG. IO
Case 3.
FIG. I I
FIG. I2
Case 4.
F:G. :3
SARCOIDOSIS OF THE NOSE 247
DISCUSSION
Of these four cases diagnosed as cutaneous sarcoidosis, three are supported by histology. Although no definite conclusions regarding diagnosis and treatment can be stated from such a small series, several points emerge which are worthy of comment. These patients are all female. This is in distinction to sebaceous rhinophyma. From the series of 57 cases of rhinophyma (Matron et al., 1962) only 7 per cent were female. It is possible that cases of sarcoidosis have been thought to be sebaceous rhinophyma and treated as such.
From the clinical point of view there are several features which may help to differen- tiate sarcoid from sebaceous rhinophyma. The infiltration of the no~e tends to extend well down the columella towards its base in the case of sarcoidosis. In one case it extended on to the upper lip. On the other hand, with sebaceous rhinophyma the swelling extends just to the distal end of the columella, the major part of it is not involved, presumably due to the relative lack of sebaceous glands in the columella. I f sarcoidosis is suspected other features of the condition can be sought. Cutaneous infiltration of the cheek, arms, or legs may be present, chest radiograph may reveal enlarged hilar glands and the Kveim test is usually positive. A Mantoux test may help to differentiate the condition from lupus vulgaris. The diagnosis from a biopsy provides conclusive evidence of sarcoidosis.
Surgical treatment of sarcoidosis of the nose has been carried out in two of these cases following failed medical treatment. In Case I, in which the nose was pared down and allowed to epithelialise spontaneously, the result after four months is quite satis- factory. The growth of epithelium was slow when compared with that which occurs following excision of sebaceous rhinophyma, but after four weeks the nose was covered by thin but healthy-looking epithelium.
It is surprising that islands of epithelium should appear and coalesce when the dermis and subcutaneous tissue is replaced to a great extent by sarcoid tissue. Also, this new epithelium would appear to grow over any remaining areas of incompletely excised sarcoidosis.
In Case 2, which was treated by excision and full thickness skin grafting, the appearance satisfied the patient but after two years some small indurations of recurrence are present in the graft. One larger nodule on the left side of the nose is evident in Figure 9- It is not of such a size to interfere with the appearance of the patient and is being observed at the present moment. No decision has been reached regarding the treatment of recurrence but it seems that further excision and either allowing spontaneous epithelialisation or skin grafting would produce a satisfactory result.
SUMMARY
Four cases of sarcoidosis of the nose are described, two of which have been treated surgically.
I would like to express my thanks to Mr J. S. Tough and Mr T. Gibson for their help in producing this paper and for the use of their clinical material.
REFERENCES BESNIER, E. (1889). Lupus pernio de la face. Annls Derm. Syph., IO, 333. BOECK, C. (I899). Multiple benign sarkoid of the skin. ft. cutan. Dis., I7, 543- MATTON~ G., PICKRELL, K., HUGER, W. and POUND, E. (1962). The surgical treatment of
rhinophyma. Plastic reconstr. Surg., 30, 4o3-414. PINNER, M. (1938). lqon-caseating tuberculosis. Am. Rev. Tuber¢., 37, 69o. SCADDI~I~, J. G. (1967). "Sarcoidosis." London : Eyre and Spottiswoode.
