Self-Assessment Self-Assessment examination of the American Academy ofDermatology* Identification No. 892-203 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 those of their colleagues in the field, use the results of the self-assessment to help determine persona! 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 I 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. Because it is often necessary to provide information in questions occurring later in a series that give away answers to earlier questions, please answer the ques- tions in each series in sequence. QUESTIONS 1-10 A 22-year-old man has numerous asymptomatic papules on the nasolabial folds (Fig. I). The papules developed at puberty and are slowly increasing in size and number. 1. The differential diagnosis of these facial papules should include (Choose one or more responses.) a. neurofibromatosis b. Cowden's disease c. multiple trichoepithelioma d. basal cell nevus syndrome e. syringomas 2. A biopsy specimen from the right nasolabial fold (Fig. 2) reveals numerous keratotic cysts and baso- philic cells in a lacelike pattern and in solid aggre- gates. The histologic differential diagnosis should in- clude (Choose one or more responses.) a. neurofibromatosis b. Cowden's disease c. multiple trichoepithelioma d. basal cell nevus syndrome e. syringomas 3. Histologic features found more commonly in trich- oepithelioma than in basal cell carcinoma include (Choose one or more responses.) a. fibrotic stroma b. epidermal connections c. frondlike tumor formation The Self-Assessment examination is supported in part by an educational grant from the Procter & Gamble Company, Cincinnati, Ohio. *Members of the Self-Assessment Committee of the AmerieanAcad- emy of Dermatology are: Kenneth J. Tomecki, MD, chairman, Bur- ton S. Belknap, MD, Mary R. Buchness, MD, Ponciano D. Cruz, MD, C. Ralph Daniel III, MD, Gary R. Kantor, MD, Francisco A. Kerdel, MD, Melinda B. Musick, MD, Tobi B. Richman, MD, The- odore Rosen, MD, Robert A. Schwartz, MD, and Ronald G. Wheeland, MD. d. tumor-stroma clefts e. papillary mesenchymal bodies 4. Clinical and histologic review established a diagnosis ofmultiple trichoepitheliomas in this patient. Each of the following is true about trichoepitheliomas except (Choose single best response.) a. they most commonly affect the nasolabial folds and nose. b. they can affect the scalp, neck, and trunk. c. they affect men more frequently than women. d. onset of lesions is noted during the second decade. e. approximately 65% of patients have a family his- tory of this 5. Multiple trichoepitheliomas have been associated with (Choose single best response.) a. eccrine spiradenomas b. chondroid syringomas c. apocrine hidrocystomas d. multiple cylindromas e. trichofolliculomas Directions for questions 6-10: Select the one lettered item that is most closely related to each numbered item: a. Autosomal dominant b. Autosomal recessive c. X-linked dominant d. X-linked recessive e. Sporadic , 6. Multiple trichoepitheliomas 7. Solitary trichoepithelioma 8. Multiple cylindromas 9. Basal cell nevus syndrome 10. Cowden's disease QUESTIONS 11-20 A 28-year-old medical student recently returned from a trip to South America where he had been walking along and swimming in the Amazon River. 513
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Self-Assessment
Self-Assessment examination of the AmericanAcademyofDermatology* Identification No. 892-203
Learning objectives: At the conclusion of this self-assessment learning activity, physician participants should be able toassess their own diagnostic and patient management skills with respect to those of their colleagues in the field, use theresults of the self-assessment to help determine persona! learning needs that can be addressed through subsequent CMEinvolvement, and enhance their ability to comply with the requirements for certification in the specialty of dermatology.
Instructions for Category I CME credit appear in the front advertising section. See last page of Contents for pagenumber.
Instructions: In answering each question, refer to the specific directions provided. Because it is often necessary to provideinformation in questions occurring later in a series that give away answers to earlier questions, please answer the questions in each series in sequence.
QUESTIONS 1-10
A 22-year-old man has numerous asymptomaticpapules on the nasolabial folds (Fig. I). The papulesdeveloped at puberty and are slowly increasing insize and number.
1. The differential diagnosis of these facial papulesshould include (Choose one or more responses.)a. neurofibromatosisb. Cowden's diseasec. multiple trichoepitheliomad. basal cell nevus syndromee. syringomas
2. A biopsy specimen from the right nasolabial fold(Fig. 2) reveals numerous keratotic cysts and basophilic cells in a lacelike pattern and in solid aggregates. The histologic differential diagnosis should include (Choose one or more responses.)a. neurofibromatosisb. Cowden's diseasec. multiple trichoepitheliomad. basal cell nevus syndromee. syringomas
3. Histologic features found more commonly in trichoepithelioma than in basal cell carcinoma include(Choose one or more responses.)a. fibrotic stromab. epidermal connectionsc. frondlike tumor formation
The Self-Assessment examination is supported in part by aneducational grant from the Procter & Gamble Company,Cincinnati, Ohio.
*Members of the Self-Assessment Committee of the AmerieanAcademy of Dermatology are: Kenneth J. Tomecki, 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, Melinda B. Musick, MD, Tobi B. Richman, MD, Theodore Rosen, MD, Robert A. Schwartz, MD, and Ronald G.Wheeland, MD.
d. tumor-stroma cleftse. papillary mesenchymal bodies
4. Clinical and histologic review established a diagnosisofmultiple trichoepitheliomas in this patient. Each ofthe following is true about trichoepitheliomas except(Choose single best response.)a. they most commonly affect the nasolabial folds
and nose.b. they can affect the scalp, neck, and trunk.c. they affect men more frequently than women.d. onset of lesions is noted during the second decade.e. approximately 65% of patients have a family his
tory of this condition~
5. Multiple trichoepitheliomas have been associatedwith (Choose single best response.)a. eccrine spiradenomasb. chondroid syringomasc. apocrine hidrocystomasd. multiple cylindromase. trichofolliculomas
Directionsfor questions6-10: Select theone lettereditem that is most closely related to each numbereditem:
A 28-year-old medical student recently returnedfrom a trip to South America where he had beenwalking along and swimming in the Amazon River.
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For the last 2 weeks he has had painful papules onthe third toe and fourth interspace of the right foot(Figs. 3 and 4). Initial evaluation included a biopsy(Fig. 5).
11. The most likely diagnosis is (Choose single bestresponse.)a. myiasisb. larva currensc. tungiasisd. chiggerse. schistosomiasis
12. The causative organism of tungiasis is (Choose single best response.)a. Pulex irritansb. Trombicula alfreddugesic. Schistosoma mansonid. Tunga penetranse. Strongyloides stercoralis
13. Tunga penetrans is also known as (Choose one ormore responses.)a. jigger fleab. niguac. bicho de ped. chigoe fleae. sand flea
14. Tunga penetrans is an arthropod that belongs to theclass Insecta, order (Choose single best response.)a. Dipterab. Coleopterac. Hemiptera
d. Siphonapterae. Lepidoptera
15. Endemic area(s) for tungiasis is (are) (Choose one ormore responses.)a. Indiab. Africac. South Americad. Caribbean Islandse. Central America
16. Which of the following statement(s) is (are) true ofTunga penetrans? (Choose one or more responses.)a. Both sexes are bloodsuckers.b. Both the adult male and the gravid female may
burrow into the skin.c. Theunfed adult is approximately 0.1 em in length,
but the abdomen of the gravid female may swellto I em in diameter.
d. The female produces ova while embedded in thetissues of the host.
e. The life cycle of the flea has four stages.
17. Tungiasis may clinically exhibit (Choose one or moreresponses.)a. one or several papules on the foot or, less com
monly, papules on the perineum or buttocksb. a small, erythematous papule with a central black
dotc. an asymptomatic, whitened, pruritic or painful
pea-sized noduled. an acute paronychiae. plaques and nodules with a honey-combed appear
18. Histopathologic examination of tungiasis reveals aflea (Choose one or more responses.)a. surrounded by epidermis except for the abdominal
segment that penetrates the dermisb. surrounded by epidermis except for the head that
penetrates the dermisc. with thickened cuticle, internal parts and eggs, and
a thick band of striated muscled. with a dwarfed head partly enclosed by an en
gorged bodye. with a surrounding dermal infiltrate of lympho
cytes, plasma cells, and eosinophils
19. Tungiasis may be complicated by (Choose one ormore responses.)a. gangreneb. cellulitisc. myocarditisd. autoamputation of toese. tetanus
20. Appropriate therapy for tungiasis includes (Chooseone or more responses.)a. curettage or excision of the burrowsb. topical antibiotics
c. systemic antibioticsd. oral thiobendazole in heavily infested patientse. tetanus prophylaxis
QUESTIONS 21-34
A 7-year-old boy has discrete and confluent, red,scaling plaques on the trunk (Fig. 6), extremities,and hands (Fig. 7).
21. Differential diagnosis includes (Choose single bestresponse.)a. psoriasisb. erythrokeratodernniac. pityriasis rubra pilarisd. ichthyosis linearis circumflexae. Vohwinkel's syndrome
A skin biopsy specimen (Fig. 8) reveals a sawtoothed epidermis with orthokeratotic hyperkeratosis.Directionsfor questions 22-24: For each numbereditem, choose the single best lettered item.
a. Psoriasisb. Erythrokeratodermia variabilis
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c. Pityriasis rubra pilarisd. Ichthyosis linearis circumflexae. Vohwinkel's syndrome
22. Thin or absent granular layer, parakeratosis, elongated rete ridges, and Munro microabscesses
23. Focal parakeratosis at follicular openings
24. Increased granular layer and eosinophilic degeneration of the upper malphigian layer in some areas.
The histologic findings of psoriasis, pityriasis rubra pilaris, and ichthyosis linearis circumflexa areabsent. The findings are most consistent with erythrokeratodermia variabilis.
25. The inheritance of erythrokeratodermia variabilis is(Choose single best response.)a. autosomal recessiveb. autosomal dominantc. x-linked rec~<1,Sive
d. x-linked dominante. unknown
26. Erythrokeratodermia variabilis usually has its onsetat (Choose single best response.)a. puberty
b. birth or infancyc. adulthoodd. menopausee. old age
27. Diseas~~ usually considered to be keratodermias include (Choose one or more responses.)a. genodermatose en cocardesb. symmetric progressive erythrokeratodermiac. Mendes da Costa diseased. Franceschetti-Jadassohn syndromee. Cross-McKusick-Breen syndrome
28. Features of Cross-McKusick-Breen syndrome include (Choose one or more responses.)a. autosomal dominant inheritanceb. microphthalmiac. gingival fibrosisd. athetosise. normal intelligence
29. Features of the Franceschetti-Jadassohn syndromeinclude (Choose one or more responses.)a. trichorrhexis nodosab. reticulated hyperpigmentationc. autosomal dominant inheritance
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d. hypohidrosise. molluscoid pseudotumors
Directions for questions 30-32: For each numbered item, select the most appropriate lettereditem.
a. Targetoid erythematous plaques with central hyperkeratosis
b. Well-defined persistent circular ichthyosiformplaques that develop in adulthood
c. Palmoplantar scaling, erythematous plaques thatdesquamate centrifugally
d. Symmetric, scaling plaques on the elbows andknees
e. Verrucous plaques inside the mouth30. Erythrokeratolysis hiemalis31. Genodermatose en cocardes
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32. Pityriasis rotunda
33. Erythrokeratodermias may be associated with(Choose one or more responses.)a. deafnessb. neuropathyc. physical retardationd. cornea verticillatae. dermatochalasis
34. Helpful therapy for erythrokeratodennia might include (Choose one or more responses.)a. salicylic acidb. emollientsc. aromatic retinoidsd. cyclosporinee. lactic acid
