SELF-AsSESSMENT

Self-Assessment examination of theAmerican Academy of Dermatology* Identification No. 894-207

Learningobjectives: At the conclusion of this self-assessment learningactivity, physicianparticipants shouldbe able toassess their owndiagnostic and patient managementskills with respectto thoseof their colleagues in the field, use theresultsof the self-assessment to helpdeterminepersonal learningneeds that can be addressedthrough subsequentCMEinvolvement, and enhance their abilityto complywiththe requirements for certificationin the specialtyof dermatology.

InstructionsforCategoryI CME creditappearin thefrontadvertisingsection.Seelast page ofContents for pagenumber.

Instructions: In answering each question, refer to the specificdirections provided. Becauseit isoften necessary to provideinformationin questions occurring later in a seriesthat give away answers to earlier questions,please answerthe ques­tionsin each series in sequence.

QUESTIONS 1-10

A 73-year-old man had a 6-month history of anenlarging nodule arising with a scar on the nose (Fig.1). A basal cell carcinoma had been treated 30 yearspreviously in this location with radiation therapy.

1. The clinical differential diagnosis includes (Chooseas many as apply. All, some, or none ofthe choicesmay be correct.)a. basal cell carcinomab. squamous cell carcinomac. Merkel cell carcinomad. lymphomae. eccrine poroma

A biopsy specimen shows sheets of small blue cellsin the dermis (Fig. 2).

2. The histologicdifferential diagnosis includes (Chooseas many as apply. All, some, or none of the choicesmay be correct.)a. sebaceous carcinomab. basal cell carcinomac. Merkel cell carcinomad. metastatic small-cell carcinoma of the lunge. lymphoma

3. Immunocytochemical staining with low-molecular­weight cytokeratins (Fig. 3) shows (Choose singlebest response.)a. artifactual staining

lilaCTheSelf-Assessment examination issupported in partby anHO<!J educational grant from the Procter & Gamble Company,

Cincinnati, Ohio.

*Membersof the Self-Assessment Committeeof the American Acad­emyof Dermatology are: Kenneth J.Tornecki, MD, chairman, Bur­ton S. Belknap, MD, Mary R. Buchness, MD, Ponciano D. Cruz,MD, C. Ralph Daniel III, MD, Gary R. Kantor,MD,Francisco A.Kerdel,MD, TobiB. Richman,MD,Theodore Rosen, MD, RobertA. Schwartz, MD, Norman W. Walton III, MD, and Ronald G.Wheeland, MD.

b. mitochondrial stainingc. cytoplasmic positivityd. specific paranuclear positivitye. none of the above 145

146 Self-Assessment examination

4. Immunocytochemical staining with neuron-specificenolase (Fig. 4) shows (Choose singlebest response.)a. artifactual stainingb. diffuse perinuclear positivityc. cytoplasmic positivityd. specific paranuclear positivitye. none of the above

5. Electron microscopy of this lesion would demonstrte(Choose as manyas apply.All, some, or noneofthechoices may becorrect.)a. Birbeck granulesb. dense-core granulesc. fibrous bodiesd. numerous melanosomese. lipid-containing vacuoles

6. A correct diagnosis of Merkel cell carcinoma may beconfirmed by (Choose as many as apply. All, some,or noneof the choices may be correct.)a. positive juxtanuclear labeling of tumor cells with

low-molecular-weight cytokeratinsb. marked cytoplasmic reactivity with neuron-spe­

cific enolasec. marked cytoplasmic reactivity with chromog­

raninsd. focal presence of neurofilament proteins in a

paranuclear locatione. negative staining for leukocyte common antigen

Journal of the American Academy of DermatologyJuly 1994

A diagnosis of Merkel cell carcinoma has been es­tablished. The patient has no lymphadenopathy orhepatosplenomegaly.

7. Further evaluation of this patient should include(Choose as many asapply. All, some,or noneofthechoices may be correct.)a. baseline chest x-rayb. computed tomographic scan of thorax, abdomen,

and pelvisc. liver function testsd. carcinoembryonic antigen levelse. lymph node biopsy

Chest x-ray findings and liver function tests arewithin normal limits.

8. The patientundergoes widelocal excisionfollowedbyradiation therapy but a new left submandibular massdevelops 2 months after treatment. He undergoesunilateral prophylactic lymph node dissection fol­lowed by radiation therapy to the draining lymphnodes bilaterally. The likelihoodthat this patient willdie of his disease despite these aggressive measures is(Choose single best response.)a. 20%b.33%c.50%d.66%e.99%

9. The most effective chemotherapeutic regimens usedin the treatment of metastatic Merkel cell carcinoma(Choose as many as apply. All, some,or noneofthechoices may be correct.)a. are those used in the treatment of small-cell car­

cinoma of the lungb. are those used in the treatment of cervical carci­

nomac. most often produce a long-lasting clinical responsed. most often produce dramatic clinical responses of

short duratione. are also the treatment of choice for recurrent lo­

calor regional disease

10. The most common site of presentation of Merkel cellcarcinoma is the (Choose single best response}a. trunkb. lower extremitiesc. upper extremitiesd. head and necke. oral cavity

QUESTIONS 11-20

A 17-year-old Oriental boy had a slightly pruriticeruption on his chest, abdomen, axillae, upper arms,and shoulders of 4 years' duration. Physical exam­ination revealed confluent light brown hyperpig-

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mentation on his trunk (Fig. 5) and coalescingerythematous pinpoint papules in a reticulated pat­tern in the axillae (Fig. 6). Mild scaling was presentonly on the trunk. He was otherwise in good healthand was not taking any medications.

II. The most likely diagnosis in this patient is (Choosesingle best response.)a. pseudo-acanthosis nigricansb. tinea versicolorc. acanthosis nigricansd. confluent and reticulated papillomatosis of Goug­

erot and Carteaud (CRP)e. dyskeratosis congenita

12. Morphologically, the primary lesion in this disease isa (Choose single best response}a. maculeb. patchc. papuled. vesiclee. plaque

Self-Assessment examination 147

13. The two most common sites for disease to initiallydevelop include (Choose two best responses.)a. intermammary areab. shouldersc. axillaed. epigastric regione. upper extremities

14. The followinghistologic features are seen in this dis­ease : (Fig. 7) (Choose one or more responses.)a. hyperkeratosisb. papillomatosisc. focal acanthosisd. vascular dilation and edemae. occasionally decreased granular layer

15. Clinical and histologic reviews establish the diagno­sis of CRP in this patient. Each of the followingstatements is trueabout this conditionexcept(Choosesinglebestresponse.]a. Small erythematous papules rapidly evolve to

gray-brown hyperkeratotic verrucous papules thatmeasure 4 to 5 mm in diameter.

b. Disease typically progresses from intermammaryand epigastric regions toward axillae, neck, andback

c. Lesions are usually confluent centrally and retic­ulated peripherally.

d. Topical corticosteroids and keratolytics are goodapproaches to therapy.

e. Thickened gray-brown hyperpigmented skin withincreased skin markings may occur in the axillaeand neckline.

16. Clinical characteristics of CRP include (Choose oneor moreresponses.)a. usual age at onset approximately 21 years (range

11 to 36 years)b. female/male ratio of 2.5:Ic. greater frequency in black persons than in white

personsd. dominant inheritance patterne. absence of mucous membrane involvement

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17. The specific cause of confluent and reticulated pap­illomatosis is (Choose single best response.)a. a tissue reaction to Pityrosporum orbiculare col-

onizationb. a disorder of keratinizationc. unknownd. an underlying endocrine disordere. a genetically inherited disease

18. The three diseases considered as the papillomatosesinclude (Choose three best responses.)a. pseudo-acanthosis nigricansb. Dowling-Degos diseasec. CRPd. acanthosis nigricanse. dermatopathia pigmentosa reticularis

19. Features of acanthosis nigricans include (Choose oneor more responses.)a. definite association with endocrinopathyb. involvement of the entire body surfacec. mucous membrane involvementd. vast majority of cases around pubertye. hyperpigmented velvety plaques in neck and axil­

lae most common

Journal of the American Academy of DermatologyJuly 1994

20. Features of pseudo-acanthosis nigricans include thefollowing (Choose one or more responses.):a. occurrence in obese personsb. predominance in black personsc. frequent involvement of mucous membranesd. reversible condition with weight losse. definite association with endocrinopathy

QUESTIONS 21-33

A 49-year-old white woman had an eczematousdermatitis involving the right ear and right side ofthe neck (Fig. 8). She had been treated by her fam­ily physician with polymyxin B sulfate-bacitracinzinc-neomycin sulfate-hydrocortisone ointment(corticosporin) and desonide cream without im­provement.

21. The differential diagnosis of this eruption includes(Choose one or more appropriate responses.)a. seborrheic dermatitisb. allergic contact dermatitisc. subacute cutaneous lupus erythematosusd. psoriasise. tinea corporis

22. Which of the following laboratory examinationsmight be useful for diagnosis? (Choose one or moreappropriate responses.)a. Biopsy for routine histologic examinationb. Patch testing with standard screening patch test

tray (Hermal Pharmaceutical Laboratories)c. Potassium hydroxide examination of scaled. Serum for detection of antinuclear antibodye. All of the above

Histologic examination showed a spongiotic derma­titis. Screening patch testing revealed positive resultsto ethylenediamine, fragrance, and neomycin. Fungiwere not identified in affected scale and antinuclearantibodies were negative. The patient was treatedfor a presumed allergic contact dermatitis to neo­mycin with triamcinolone acetonide 0.025% oint­ment in a petrolatum base, which resulted in wors­ening of the dermatitis.

23. Diagnostic evaluation atthis pointinclude(s) (Chooseone or more appropriate responses.)a. patch testing to various corticosteroidsb. challenge with oral corticosteroidsc. patch test to vehicle and preservative allergensd. provocative use testing of corticosteroid ointmentse. all of the above

The patient was then patch tested to triamcinoloneacetonide 0.025% ointment and a moderate reaction

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was obtained (Fig. 9). The patient was treated withbetamethasone diproprionate ointment; the derma­titis cleared.

24. Under what circumstances should allergic contactdermatitis to corticosteroid preparations be sus­pected? (Choose oneormoreappropriate responses.)a. When the preparation does not improve the exist­

ing dermatitisb. When the preparation worsens the existing der­

matitisc. When the patient reports burning and stinging on

application of the productd. When there is slow resolution of an existing der­

matitise. When a formerly helpful preparation no longer

seems to have therapeutic value

25. In patch testing for corticosteroid sensitivitywhich ofthe following parameters should you consider?(Choose one or more appropriate responses.)a. Antiinflammatory nature of corticosteroidsb. High frequency of false-negative patch testsc. Corticosteroid concentration in the patch testd. The choice of vehiclee. Distinguishing cross-reactions from multiple sen­

sitizations

26. The incidence of allergic hypersensitivity to topicalcorticosteroids is highest when used for treatment of(Choose singlebest response.)a. lichen simplex chronicusb. psoriasisc. stasis dermatitisd. nummular eczemae. seborrheic dermatitis

27. Which of the followinghas been found tobe a markerfor hydrocortisone sensitivity? (Choose single bestresponse]a. Tixocortal pivalateb. Hydrocortisone-17-butyratec. Hydrocor tisone acetated. Triamcinolone acetonidee. 6-Chloroprednisone

28. Which of the following is (are) the cause(s) of aller­gic contact dermatitis due to the vehicle in topicalmedicaments? (Choose oneor more appropriate re­sponses.)a. Stearyl alcoholb. Propylene glycolc. Parabensd. Ethylenediamine hydrochloridee. Polysorbate 60

29. Which ofthe following preservatives, a known sensi­tizer in many over-the-counter corticosteroid prepa-

Self-Assessment examination 149

rations, is virtually hypoallergenic when applied tonondermatitic skin? (Choose single best response]a. Formaldehydeb. Parabensc. Quaternium 15d. Sorbic acide. Germall 115

30. Which of the following creams is totally free of allpreservatives? (Choosesingle best response]a. Fluocinonide (Lidex)b. Fluocinonide (Lidex E)c. Triamcinolone (Kenalog)d. Desoximetasone (Topicort)e. Fluocinolone acetonide (Synalar)

31. In a patient with a known corticosteroid sensitivitywhich, if any, route of administration could likelyre­sult in an immunologic reaction to that agent?(Choose one or more appropriate responses.}a. Oral routeb. Topical routec. Intralesional routed. Parenteral routee. None of the above

32. Cross-reactivity between corticosteroids has been re­ported for which of the following? (Choose one ormore appropriate responses.)a. Agents within a groupb. Cross-reactions to systemic corticosteroids in the

same classc. Cross-reactionsto intraarticular corticosteroids in

the same classd. Cross-reactivity between groupse. Cross-reactivity to all corticosteroids once sensi­

tized to anyone in a group

33. Which of the following belong(s) to the four basiccorticosteroid classes as proposed by Dooms-Goos­sens? (Choose all appropriate responses]a. The hydrocortisone groupb. The betamethasone groupc. The triamcinolone acetonide groupd. The hydrocortisone-7-butyrate groupe. The desoximetasone group

QUESTIONS 34-45

An 85-year-old man has many purple to blackpapules, some of them crusted, on the right side ofhis chest (Figs. 10 and 11). Lesions began appear­ing 4 months ago. He had also reported unsteadinessof gait of 2 weeks' duration.

34. Which entity is least likely in this case? (Choose sin­gle best response.)a. Kaposi's sarcoma

150 Self-Assessment examination

b. Bacillary angiomatosisc. Hemangiomasd. Primary melanomae. Metastatic melanoma

35. A biopsy specimen (Figs. 12 and 13) is most consis­tent with (Choose singlebest response.)a. congenital nevusb. epidermotropic metastatic melanomac. hemangiomad. Kaposi's sarcomae. hamartoma

36. Possible explanations for the lack of a clinically dis­cernibleprimary lesion includethe following (Choosesingle best response]:a. The primary lesion may have been destroyed

inadvertently or by inadequate treatment in thepast.

b. The primary lesion underwent spontaneous reso­lution for immunologic reasons.

c. The primary lesion remains in the skin, but is in­distinguishable from other pigmented lesions or istoo small or deeply placed in the adnexa to allowfor clinical detection.

d. The primary lesion is in an internal organ andtherefore eludes clinical detection.

e. All of the above

Journal of the American Academy of DermatologyJuly 1994

37. The frequency of metastatic melanoma without aclinically discernible primary lesion has been re­ported as (Choose single best response.)a. <1%b. 1%-15%c. 15%-25%d.25%-40%e. none of the above

38. Reasonable explanations for the localization of le­sions in this patient include (Choose single bestresponse.]a. dissemination of cells from primary lesion via

blood vesselsb. dissemination of cells from primary lesion via

lymphaticsc. neurotropism of cells from primary lesiond. all of the abovee. none of the above

39. Which of the following is the least likely site for met­astatic melanoma? (Choose singlebest response]

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a. Liverb. Lungc. Thyroidd. Bonee. Brain

40. Which of the following is the most likely site formetastatic melanoma?(Choose single bestresponse.)a. Lungb. Pericardiumc. Liverd. Gastrointestinal tracte. Brain

41. The most common site for a primary lesion of malig­nant melanoma in men is (Choose single bestresponse.)a. head and neckb. armsc. legsd. hands and feete. trunk

42. The most common decade of life for malignant mel­anoma to be identified is the (Choose single bestre­sponse.)a. fourthb. fifthc. sixth

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d. seventhe. eighth

43. Therapeutic options that may prolong survival in thispatient include (Choose as many as apply.All, some,or none of the responses may be correct.)a. systemic immunotherapyb. laser ablationc. surgical excision of all visible lesionsd. surgical excision combined with axillary lymph

node dissectione. local irradiation

44. New chemotherapeutic agents under investigationinclude (Choose single best response.)a. detrorubicinb. taxolc. vindesined. dibromodulcitole. all of the above

45. Further evaluation of this patient should include(Choose as many as apply. All, some, or none oftheresponses may be correci.)a. palpation of lymph nodesb. bone marrow biopsyc. computed tomographic scan of chest/abdomend. computed tomographic scan of heade. stool occult blood determination