S e l f - A s s e s s m e n l I I I I II I I II II

Self-Assessment examination of the American Academy of Dermatology* Identification No. 889-201

Learning objectives: At the conclusion of this self-assessment learning activity, physician participants should be able to assess their own diagnostic and patient management skills with respect to tho~o of their colleagues in the field, use the results of the self-assessment to help determine personal learning needs that can be addressed through subsequent CME involvement, and enhance their ability to comply with the requirements for certification in the specialty of dermatology.

Instructions for Category 1 CME credit appear in the front advertising section. See last page of Contents for page number.

Instructions." In answering each question, refer to the specific directions provided. Since it is often necessary to provide information in questions occurring later in a series that give away answers to earlier questions, please answer the questions in each series in sequence.

QUESTIONS 1-14

A 54-year-old white woman ingested eight diet pills containing phenylpropanolamine. One week later she noted pruritus of her feet and calves, which progressed to pain, erythema, and edema. She was admitted to the hospital 4 weeks later for presumed cellulitis but did not respond to antibiot- ics. A venogram was normal and did not show deep venous thrombosis. Cordlike, tender, purpuric nod- ules associated wtih a livedo pattern developed on both legs and subsequently ulcerated (Figs. 1 and 2). The patient's tempera ture was 39 ~ C. Hyperes- thesia and paresthesia developed in the feet.

1. The differential diagnosis should include (Choose as many as apply. All, some, or none o f the choices may be correct.) a. thromboangiitis obliterans (Buerger's disease) b. periarteritis nodosa e. murantic endocarditis with peripheral emboliza-

lion d. Wegener's granulomatosis e. sperotrichosis

2. Appropriate laboratory examinations to be per- formed include (Choose as many as apply. All, some, or none o f the choices may be correct.) a. hepatitis B surface antigen b. human immunodeficieney virus (HIV) serolo-

gY

~ The Self-Assessment examination is supported in part by an educational grant from the Procter & Gamble Compa- ny, Cincinnati, Ohio.

*Members of the Self-Assessment Committee of the American Academy of Dermatology are: Ernesto Gonzalez, MD, chairman, Rita S. Berman, MD, Jeffrey D. Bernhard, MD, Mark S. Bernhardt, MD, Michael E. Bigby, MD, Madeleine Duvic, MD, Gloria F. Graham, MD, Richard A. Johnson, MD, Francisco Kerdel, MD, Donald Paul Lookingbill, MD, Emily F. Omura, MD, Warren W. Piette, MD, Dennis C. Polley, DO, Sharon Ann Smith Raimer, MD, and Kenneth Joseph Tomeeki, MD.

c. wedge biopsy of lesional margin d. culture of biopsy specimen for fungi e. biopsy of inguinal lymph node. Labora tory studies revealed the following: ane-

mia, mild leukocytosis with 42% eosinophils; eryth- rocyte sedimentation rate, 93 m m / h r ; urinalysis, normal. Hepatitis B surface antigen, rheumatoid factor, antinuclear antibody, cryoglobulin, and V D R L test results and serum complement levels were all normal. A skin biopsy specimen showed thrombosis of a medium-sized vessel in the subcu- taneous fat (Fig. 3). Numerous inflammatory cells were demonstrated within the vessel wall and the immedia te surrounding adipose tissue (Fig. 4). Elastic stain demonstrated an internal elastic lami- n a with focal areas of disruption above the vessel (Fig. 5). Hemorrhage was present throughout t h e reticular dermis and subcutaneous fat.

3. Based on the preceding findings, your best working diagnosis is (Choose single best response.) a. classic periarteritis nodosa b. leukocytoclastic angiitis c. Wegener's granulomatosis d. Henoch-SchiSnlein purpura e. cutaneous periarteritis nodoea

4. Direct immunofluorescence in cutaneous periarteri- tis nodosa shows (Choose as many as apply. All, some, or none o f the choices may be correct.) a. characteristic pattern different from classic per-

iarteritis nodosa b. frequent IgM deposition c. frequent IgG and IgA deposition d. deposition of immunoreactants in superficial and

deep dermal vessels e. absence of IgG and IgA

5. Cutaneous periarteritis nodosa has been associated with each of the following findings, except (Choose single best response.)

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a. regional enteritis b. fl-streptococcal infections c. hepatitis B antigenemia d. arthralgias e. myalgias

6. Classic periarteritis nodosa has been associated with each of the following etiologic factors, except (Choose single best response.) a. fl-streptococcal infections b. hepatitis B antigenemia c. mixed cryoglobulinemia d. rheumatoid arthritis e. sesquiterpene lactone-induced dermatitis

7. The appropriate initial therapy for severe cases of cutaneous periarteritis nodosa is (Choose single best response.) a. high dosage of aspirin b. colchicine c. cyclophosphamide

d. oral daily prednisone e. subcutaneous heparin, followed by oral sodium

warfarin (Coumadin)

For each numbered item choose the most appropriate lettered response.

a. Cutaneous periarteritis nodosa b. Classic periarteritis nodosa c. Both d. Neither

8. Nodules are usually painless 9. Livedo reticularis appears as "star-bursts" around

nodules 10. Skin lesions do not ulcerate 11. Cutaneous nodules rarely follow the course of the

arteries 12. Presence of direct immunofluorescence pattern 13. Cutaneous nodules usually follow the course of the

arteries 14. Renal involvement

Volume 20 Number 1 January 1989 Self-Assessment examination 145

QUESTIONS 15-24

A 29-year-old previously healthy white woman developed asymptomatic palpable purpura of the ankles, extensor aspects of the extremities, but- tocks, and face, 1 week after a documented strep- tococcal pharyngitis (Fig. 6). She subsequently developed arthralgias and swelling of the ankles, knees, wrists, and fingers accompanied by abdom- inal pain, diarrhea with heine-positive stools, and hematemesis. Physical examination revealed no organomegaly nor lymphadenopathy but revealed a grade 2/6 systolic ejection murmur at the upper left sternal border. The woman was notably hyper- tensive and afebrile throughout the illness. Labora- tory evaluation included negative or normal blood urea nitrogen and creatinine test results, clotting studies and platelet count, complement studies, rheumatoid factor test results, and antinuclear

antibody profile. Urinalyses and 24-hour urine studies revealed marked hematuria and a 6 gm proteinuria.

15. The most likely diagnosis in this patient is (Choose single best response.) a. Waldenstr6m's purpura b. subacute bacterial endocarditis c. Henoch-Sch~Snlein purpura d. meningococcemia e. poststreptococcal glomerulonephritis

Histologic examination of a skin biopsy speci- men demonstrated a perivascular infiltrate within the superficial and mid reticular dermis containing neutrophils and karyorrhectic debris. Extravasated erythrocytes were present within the surrounding interstitium. The walls of some vessels displayed amorphous fibrinoid deposits (Fig. 7). Direct immunofluorescence studies showed deposits of

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IgA and C3 within blood vessels and fibrin within and around vessels.

16. The pathophysiology of this disease involves (Choose single best response.) a. deposition of IgA-containing immune complexes

in small blood vessels b. septic emboli from heart valve vegetations c. hyperviscosity of the serum d. cryofibrinogens e. cold agglutinins

17. Each of the following is true regarding this disease, except (Choose single best response.) a. it primarily affects children b. women are affected more frequently than

men c. its most common initial manifestations are pal-

pable purpura, abdominal pain, glomerulone- phritis, and arthritis

d. there is a seasonal predilection for winter and spring

e. clinical manifestations are similar in children and adults

18. Disease associations include (Choose as many as apply. All, some, or none o f the choices may be correct.) a. malignancy b. cryoglobulinemia c. upper respiratory tract infections d. familial Mediterranean fever e. all of the above

19. Precipitating factors include (Choose as many as apply. All, some, or none o f the choices may be correct. ) a. insect bites and stings b. immunizations and vaccinations c. cold exposure d. drugs, such as aspirin, penicillin, sulfonamides,

and thiazides e. all of the above

20. With gastrointestinal involvement, each of the fol- lowing is true, except (Choose single best response.) a. abdominal pain, which is the most common

gastrointestinal symptom, may precede the skin lesions in 10% to 15% of cases in children and adults

b. intussusception occurs with equal frequency in children and adults

c. rare complications include visceral infarction or perforation and pancreatitis

d. a protein-losing enteropathy can occur e. in some patients the initial manifestation is

occult or gross blood in the stool

21. With renal involvement, each of the following is true, except (Choose single best response.) a. the most common finding to indicate renal

involvement is hematuria b. the most common renal lesion is a focal prolifer-

ative glomerulonephritis c. evidence of renal disease usually subsides with

the acute illness in the majority of patients d. renal complications are much more common in

children than in adults e. poor prognostic factors include acute renal fail-

ure, hypertension, and a large percentage of crescents on renal biopsy

22. In general, the prognosis of this disease (Choose as many as apply. All, some, or none o f the choices may be correct.) a. most closely correlates with the severity of renal

involvement b. is dependent on extrarenal manifestations c. is good because eventual recovery without

sequelae occurs in 95% or more of patients, although recurrent attacks may ensue over a period of several years following onset

d. a and c e. all of the above

23. Direct immunofluorescent findings in this disease (Choose as many as apply. All, some, or none o f the choices may be correct.) a. occur in skin specimens of involved skin b. occur in skin specimens of uninvolved skin c. are frequently negative if obtained from skin

lesions older than 48 hours d. reveal granular deposits of IgA, C3, and fibrin in

the dermal capillary walls and dermal connec- tive tissue as well as mesangial regions of the glomemli

e. all of the above

24. The renal lesions in this disease are histologically identical to (Choose single best response.) a. poststreptococcal glomerulonephritis b. Berger's disease (IgA nephropathy) c. cryoglobulinemia d. systemic lupus erythematosus e. none of the above

QUESTIONS 25-34

A 28-year-old white woman, at initial presenta- tion, had a gradually enlarging, asymptomat ic scalp lesion that had been present for 8 years. The patient consulted a physician 5 years previously and was told the lesion was a cyst tha t did not require treatment. A red brown plaque, 8 cm in diameter with a cobblestone surface and a maximal

Volume 20 Number l January 1989 Self-Assessment examination 147

thickness of 1.5 cm, was noted on the right parietal area of the scalp. The surface of the skin was shiny, intact, and stretched over the plaque. The scalp hair appeared normal but its density was reduced in the lesion (Fig. 8).

25. The most likely clinical diagnosis is (Choose single best response.) a. cylindroma b. linear (en coup de sabre) circumscribed mor-

phea c. nevus sebaceus d. dermatofibrosarcoma protuberans e. cutis verticis gyrata

26. What tests or procedures should be ordered or performed? (Choose as many as apply. All, some, or none of the choices may be correct.) a. Glucose tolerance test b. Antibody studies for Borrelia burgdorferi c. X-ray of skull and soft tissue of the scalp d. Specimen of lesion for histopathology e. Culture of a section of the biopsy specimen for

fungus and atypical mycobacterium

X-ray examination of the skull and scalp showed no involvement of the calvarium and thickening of

the soft tissue of the scalp. Light microscopic examination revealed an expansile, storiform pro- liferation-of spindle cells, displacing the normal reticular dermal stroma, infiltrating the subcutane- ous fat, and dissecting laterally along the reticular dermal-subcutaneous fat interface (Figs. 9-11).

27. The most characteristic histologic feature of derma- tofibrosarcoma protuberans is (Choose single best response.) a. numerous large pleomorphic fibroblasts b. storiform and cartwheel pattern of fibroblasts c. large, bizarre, rnultinucleated giant cells d. proliferation of spindle-shaped cells forming nar-

row vascular slits e. spindle-shaped cells arranged in herringbone

pattern

28. The Bednar tumor is best characterized as derma- tofibrosarcoma protuberans with (Choose single best response.) a. additional feature of melanin-containing den-

dritic cells b. loci of calcification c. involvement of the palmar or plantar fascia d. areas of liposarcomatous differentiation

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e. large areas of hemorrhage within cystlike spaces and thin-walled vascular spaces

29. Which of the following apply to dermatofibrosarco- ma protubarans? (Choose as many as apply. All, some, or none of the choices may be correct.) a. Most commonly occur on the scalp b. Is predominantly a neoplasm of men older than

60 years c. Is easily cured with conventional radiotherapy d. Arises in sites of previous radiotherapy e. Metastases are rare

30. The following are characteristics that fit the biolog- ic pattern of behavior of dermatobibrosarcoma protuberans, except (Choose single best re- sponse.) a. affect predominantly patients in the fourth

decade b. no significant difference in incidence between

males and females c. commonly occurs on the trunk and extremities

but spares palms and soles d. high incidence of local recurrences, low inci-

dence of metastases e. high incidence of local recurrences, high inci-

dence of metastases

31. The clinical course of dermatobirosarcoma protu- berans is characterized by (Choose as many as apply. All, some, or none of the choices may be correct.) a. slow growth b. recurrence in 5% to 10% of cases after initial

surgery c. recurrence in up to 50% of eases after initial

surgery

d. average recurrence time of 6 months after excision.

e. average recurrence time of 33 months after excision

32. The frequency of recurrences of dermatofibrosarco- ma protuberans is closely associated with (Choose single best response.) a. histologic pattern of invasion b. diameter of neoplasm c. duration of lesion d. number of mitotic figures e. inadequate resection margins

33. Recommended initial therapy for dermatofibrosar- coma protuberans is (Choose the two best re- sponses.) a. surgical excision with conservative margins b. wide surg6cal excision including a surrounding

margin of at least 3 cm normal tissue c. Mobs micrographic surgery d. conventional radiotherapy followed by high-dose

intravenous methotrexate e. intralesional injection of interferon followed by

high-dose intravenous methotrexate

34. Birefringent, doubly refractile polarizable collagen is not observed in the stroma of which of the following fibroproliferative dermatoses? (Choose as many as apply. All, some, or none of the choices may be correct.) a. Dermatofibrosarcoma protuberans b. Dermatofibroma c. Fibromatosis d. All of the above e. None of the above