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IOURNAL o f t h e

A m e m c a N A c a D e m y OF

D e r M a T O L O G Y

Self-Assessment examination of the American Academy of Dermatology*

Identification No. 883-208

See "Instructions for Category 5(d) CME Credit" on page 37A of the front advertising section.

QUESTIONS 1-5

A 63-year-old man comes to your office with a long-standing lesion on the back (Fig. t). He says the lesion began many years previously and has always been painless. On palpation of the lesion, you are impressed by a sharply demarcated de- pression of the surface of the skin at the edge of the lesion.

1. You tell the patient that the diagnosis is (Choose single best response.) a. morphea b. epidermal nevus c. dermal (collagen) nevus d. atrophoderma of Pasini and Pierini e. anetoderma of Schweninger and Buzzi

2. The patient asks you for a prognosis. You tell him that the usual course of atrophoderma of Pasini and Pierini is to (Choose single best response.) a. expand slowly over 3 to 4 years and then

gradually involute spontaneously b. expand slowly over 10 to 20 years and then

gradually involute spontaneously c. expand slowly over 10 to 20 years and then

persist indefinitely d. expand rapidly over 3 to 6 months and then

expand slowly for up to 50 years e. continue to expand slowly over the lifetime of

the patient

3. The most common site of occurrence of this entity is the (Choose single best response.) a. neck b. back

The Self-Assessment examination is supported in part by an educa- tional grant from OWEN Laboratories, Fort Worth, TX.

*The members of the Self-Assessment Committee of the American Academy of Dermatology are: W. Clark Lambert, M.D., chair- man, Ronald J. Barr, M.D., Joseph B. Bikowski, M.D., Ralph J. Coskey, M.D., Richard L. De Villez, M.D., Ernesto Gonzalez, M.D., Ira H. Gouterman, M.D., Terry M. Jones, M.D., Thomas G. Olsen, M.D., Charles E. Reaves, M.D., Richard K. Scher, M.D,, Henry T. Shenfield, M.D., Thomas R. Wade, M.D., and Paul S. Wolfish, M.D.

.

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c. abdomen d. arms e. legs

The most common age of onset of this disease is (Choose single best response.) a. 5-10 years b. 10-30 years c. 30-40 years d. 40-60 years e. over 60 years

This disease usually occurs as a (an) (Choose sin- gle best response.) a. autosomal dominant trait b. autosomal recessive trait c. sex-linked recessive trait d. multigenic trait e. sporadically, with no known familial predis-

position

QUESTIONS 6-9

A 52-year-old white woman consults you with a

36-year history of "chronic e c z e m a . " She was previously treated by several physicians with topi- cal and systemic steroids without significant re- sponse. On examination, the patient is noted to have discrete, brownish, scaling papules over her chest, sacrum, and thighs. Her palms are studded with keratotic papules, and the fingernails are ridged and distally split. The oral mucosa is studded with white, umbil icated " co b b l e s tone" papules.

6. At this time you would (Choose the two best re- sponses.) a. obtain a family history b. treat with ACTH c. obtain a biopsy d. order a barium enema e. obtain a potassium hydroxide (KOH) examina-

tion of the scale

Biopsy material reveals an epidermis that is ir-

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regularly acanthotic with para- and hyperkerato- sis. There is formation of many clefts within the epidermis, with evidence of dyskeratotic and acan- tholytic epithelial cells. Pyknotic nuclei, each sur- rounded by a dense eosinophilic cytoplasm with an outer glistening membrane, are evident, as are small cells with elongated nuclei and scant cyto- plasm resembling parakeratotic cells.

7. This clinical presentation and biopsy confirm a di- agnosis of (Choose single best response.) a. epidermal nevus b. atopic dermatitis c. Dafter-White disease d. benign familial pemphigus e. arsenical keratoses

8. The literature reports some associations with Darier-White disease. True statements concerning

Volume 9 Number 2 August, 1983

this clinical entity include (Choose as many as are correct. All, some, or none of the answers may be appropriate.) a. susceptibility to Kaposi's varicelliform erup-

tion is reported b. summer exacerbations are common c. concern for occult malignancy is warranted d. affected individuals are often of small stature

and low intelligence e. severe vitamin deficiency is almost always

found

9. The most effective therapy of this entity to date has been reported with (Choose single best response.) a. vitamin A (200,000 U/day) b. Plaquenil (hydroxychloroquine sulfate) c. ACTH d. aromatic retinoids e. dapsone

QUESTIONS 10-14

A 56-year-old South American man presents with a 9-mdiath history of an enlarging annular erythematous lesion on his right forearm. Three other nodular lesions are present on his trunk.

10. Included in your differential diagnosis are all of the following, except (Choose single most appropriate response.) a. sarcoidosis b. fixed drug eruption c. lepromatous leprosy d. granuloma annulare e. tuberculosis

11. A biopsy of the lesion was performed (Fig. 2). Histopathologic findings found in lepromatous lep- rosy include all of the following, except (a)(the) (Choose single most appropriate response.) a. grenz zone b. foamy histiocytes showing fine, gray granular

material within their cytoplasm, representing phagocytized elastin

c. granulomatous infiltrate around nerves d. Fite stain showing numerous red-staining ba-

cilli within histiocytes e. demonstration of globi

12. Drugs which might be beneficial to the patient in- clude (Choose the three best responses.) a. isoniazid b. dapsone c. rifampicin

Self-Assessment examination 47A

d. streptomycin e. clofazimine

13. Which of the following skin tests might be positive in this patient.9 (Answer as many as are correct. All, some, or none of the choices may be appro- priate.) a. Mitsuda b. Montenegro c. Fernandez d. Frei e. Dick

14. Erythema nodosum leprosum (ENL), the com- monest reactional state in this disease (Choose the two most appropriate responses.) a. usually begins within 2 years of treatment b. rarely occurs in lepromatous leprosy c. manifests with crops of red, tender nodules on

the face and extremities d. cannot be distinguished histologically from

erythema nodosum e. is usually asymptomatic

QUESTIONS 15-18

A 73-year-old white woman comes to your office with a several-day history of tender, edema- tous, and indurated areas of the face. You note that she is slightly febrile (37.8 ~ C), and that the skin is smooth, taut, and shows exaggerated wrinkle folds. The skin along the mandible is boggy, with painful, deep, indurated nodular areas (Fig. 3). You obtain a biopsy of the mandibular area, taking care to obtain subcutaneous fat with the biopsy (Fig. 4).

15. The most likely correct diagnosis is (Choose single best response.) a. Sweet's syndrome b. sarcoidosis c. berylliosis d. paraffin granuloma e. silica granuloma

16. You would best confirm the diagnosis of silica granuloma by (the) (Choose single best response.) a. thioflavine T stain b. alizarin red S stain c. oil red 0 stain d. glyoxal-bis- 2-hydroxyaniline stain e. polarization microscopy

Upon further questioning, the patient admits to having received numerous facial injections with "l iquid s i l icone" in Mexico approximately 20

48A Self-Assessment examination Journal of the

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years previously. The patient asks why, if her pres- ent lesions are related to her previous "s i l icone" treatment, there have been no adverse effects until the sudden onset of her present problem.

17. You explain that (Choose single best response.) a. the subcutaneous lesions form a nidus for cal-

cium or urate deposition, leading to a sudden inflammatory reaction in some cases

b. the subcutaneous lesions have become sec- ondarily infected, causing the sudden inflam- matory process

c. the patient has suddenly become allergic to the deposits of foreign material, causing the sud- den inflammation

d. the silica has gradually dissolved and the col- loidal suspension so formed has induced the

inflammatory response by a nonimmunologic mechanism

e. the patient must be mistaken, and that the inflammation has been developing for several years

18. The usual treatment for silica granulomas is (to) (Choose single best response.) a. desensitization to colloidal silica b. systemic corticosteroids c. locally injected corticosteroids d. surgical excision of the lesions e. allow the lesions to subside spontaneously

QUESTIONS 19-25 This 30-year-old woman gives a greater than

15-year history of multiple lesions (Figs. 5 and 6)

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beginning on her wrists, w i th eventual involve- ment o f the a rms , legs, and chest area. The lesions first appear as small " p i m p l e s " with per iphera l extension and are more p rominen t after sun expo- sure. The lesions are often pruri t ic .

19. The most likely correct diagnosis is (Choose single best response.) a. idiopathic guttate hypomelanosis b. disseminated superficial actinic poroker-

atosis c. multiple fiat warts d. atrophic lichen planus e. none of the above

20. A skin biopsy is performed and reveals the features shown in Fig. 7. Now your diagnosis is (Choose single best response.) a. lupus erythematosus (LE) b. lichen sclerosus et atrophicus (LSA) c. disseminated superficial actinic porokeratosis

(DSAP) d. atrophic lichen planus (ALP) e. none of the above

21. Characteristic histologic features of DSAP include all of the following, except (a)(the) (Choose single most appropriate response.) a. thin, somewhat atrophic epidermis b. lichenoid dermal infiltrate c. presence of cornoid lamellae d. numerous telangiectatic blood vessels e. large numbers of eosinophils

22. In addition to DSAP, other variants of porokerato- sis include (Choose single best response.) a. solitary lesions (porokeratosis of Mibelli) b. dissemiffated nonactinic porokeratosis c. punctate porokeratosis (of the palms/soles) d. linear or zosteriform porokeratosis e. all of the above

23. DSAP is inherited by which of the following modes? (Choose single best response.) a. Autosomal dominant b. Autosomal recessive c. Sex-linked recessive d. Sex-linked dominant e. It is not hereditary

24. All of the following statements about DSAP are true, except (Choose single most appropriate re- sponse.) a. oral lesions have been reported b. the disease is more common in female indi-

viduals

25.

c. the histogenesis is thought to be the result of an abnormal "c lone" of epidermal cells

d. lesions predominate on extremities e. the disease exacerbates with sun exposure

Which of the following statements concerning the cornoid lamella is true? (Choose single best re- sponse.) a. It is characteristic of all variants of poroker-

atosis b. It is similar to other forms of epidermal reac-

tion patterns (i.e., epidermolytie hyperkerato- sis, focal acantholytic dyskeratosis, follicular mucinosis)

c. It is seen in lesions not related to porokeratosis d. It histologically is characterized by a vertical

column of parakeratotie stratum corneum e. All of the above are correct

QUESTIONS 26-29 A 60-year-old man presented with red, asymp-

tomatic nodules on the V area of his chest of 2 weeks ' duration (Fig. 8). Previous medical history revealed that this patient had had two lung lobe resections for poor ly differentiated squamous cell carcinoma of the lung. The workup for metastasis both times was negative. Six months fol lowing the second procedure, a course of chemotherapy was begun. After finishing this course, these nodules appeared.

Skin biopsy revealed nests of epithelial type cells in the dermis and subcutis. Nuclei of varied

shapes and sizes were noted. Numerous mitoses were present (Fig. 9).

26. In men, the most frequent two sites of (or types of) primary tumors to metastasize to the skin are (the) (Choose the two best responses.) a. large intestine b. kidneys c. oral cavity d. lungs e. melanoma

27. In women, the most frequent two sites of (or types of) primary tumors to metastasize to the skin are (Choose the two best responses.) a. breasts b. large intestine c. melanoma d. oral cavity e. kidney

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28. The two most commonly recognized tumors meta- static to skin in men before the primary carcinoma is known are those arising in (the) (Choose the two best responses.) a. breasts b. oral cavity (squamous cell carcinoma) c. kidney d. lung e. liver

29. Some types of metastatic cutaneous lesions often

allow the primary site to be identified. Tumors metastatic from which three of the below would be most likely to help the pathologist diagnose the primary site? (Choose the three best responses.) a. Gastrointestinal tract b. Kidney c. Choriocarcinoma d. Lung e. Stomach