Self-Assessment
Self-Assessment examination of the AmericanAcademy of Dermatology* Identification No. 892·201
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 personal 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 page number.
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 58-year-old white man has many flesh-coloredand erythematous papules and nodules diffuselyscattered on the hands, arms, and legs (Figs. 1 and2). The papules are dome-shaped and some noduleshave a central keratinous plug; size ranges from 3 to12 mm in diameter. Papules on the chest were discrete and coalescing and spanned an area of20 X 20cm(Fig. 3). Disease had been presentfor 18 months.
1. Differential diagnosis includes each of the followingexcept (Choose single best response.)a. perforating dermatosisb. mycosis fungoidesc. multiple keratoacanthomasd. hypertrophic lichen planuse. hypertrophic lupus erythematosus
The patient had a history of chronic sun exposureand many episodes ofsunburn in his lifetime; he hadbeen treated for multiple actinic keratoses. His skindisease supposedly began acutely and lesions wereboth painful and itchy.2. On the basis ofthe history and askin biopsyspecimen
ofa representative papule (Fig. 4), the best diagnosisis (Choose single best response.)a. perforating dermatosisb. prurigo nodularis
TheSelf-Assessment examination is supported in part by aneducational grant from the Procter & Gamble Company,Cincinnati, Ohio.
*Members of the Self-Assessment Committee of the American Acad·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, Theodore Rosen, MD, Robert A. Schwartz, MD, and Ronald G.Wheeland, MD.
c. multiple keratoacanthomasd. hypertrophic lichen planuse. hypertrophic lupus erythematosus
The patient had an aortic valve replacement in 1983and now takes warfarin for anticoagulation. Thereis no personal or familial history of skin cancer, gas~trointestinal cancer, or other malignancy.3. Each of the following is appropriate in the evaluation
at this point except (Choose single best response.)a. stool examination for occult bloodb. general medical examinationc. chest x-rayd. complete blood cell count, renal and hepatic func
tion tests, urinalysise. colonoscopy
The patient has a normal physical examination,chest x-ray, and routine laboratory tests, including anegative stool hemoccult. The best diagnosis at thistime is multiple eruptive keratoacanthomas,Gryzbowski type.4. Therapeutic options for multiple eruptive keratoa~
canthomas include which of the following? (Chooseas many as apply. All, some, or none ojthe choicesmay be correct.)a. Cryosurgeryb. Retinoidsc. Electrodessication and curettaged. Intralesional 5-fluorouracile. Surgical excision
The patient received isotretinoin, 0.5 mg/kg/daytwice a day. Mter 3 months of therapy, he noticeddramatic improvement.5. Each of the following is true about retinoid therapy
for multiple eruptive keratoacanthomas except(Choose single best response.)
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a. isotretinoin has proved to be efficaciousb. etretinate has proved to be efficaciousc. maintenance therapy is often necessaryd. dosages of 0.50 to 0.75 mg/kg/day are useful for
induction therapye. therapy is tolerable and side effects are minimal
6. Ferguson-Smith type of multiple keratoacanthomas(Choose single best response.)a. is usually present in childhood or adolescenceb. is an acquired diseasec. occurs primarily on sun-exposed areasd. is typically characterized by many small pruritic
papulese. usually heals without scarring
7. Multiple keratoacanthomas have occurred in theMuir-Torre syndrome, a disease characterized bywhich ofthe following features? (Choose as many asapply. All, some, or none ofthe choices may be correct.)a. Benign sebaceous neoplasmsb. Malignant sebaceous neoplasmsc. Gastrointestinal malignancy
d. Pulmonary malignancye. Genitourinary malignancy
Directions for questions 8-10: For each numbereditem choose the appropriate lettered item.
a. Multiple eruptive keratoacanthomas, Grzybowskitype
b. Multiplekeratoacanthomas, Ferguson-Smith typec. Bothd. Neither
8. Pruritic skin disease
9. May involve the larynx and oral mucosa
10. Histopathologic features similar to the solitary type
QUESTIONS 11-21
A 37-year-old black woman has had multiple hemangiomas since early childhood. She has undergone many surgical procedures to resect several ofthe hemangiomas. A recent spontaneous fracture ofthe right wrist required insertion of a metal plate andscrews. Physical examination at that time revealed
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many compressible flesh-colored and blue papules,nodules, and tumors scattered diffusely on the rightupper extremity, right shoulder, and chest; theextremity was deformed (Figs. 5 and 6).
11. Differential diagnosis at this time includes which ofthe following? (Choose as many as apply. All, some,or none of the choices may be correct.)a. Maffucci's syndromeb. Kasabach-Merritt syndromec. Gorham's syndromed. KlippeI-Trenaunay syndromee. Blue rubber bleb nevus syndrome
12. Which of the following studies are necessary in theevaluation of this patient? (Choose as many as apply.
All, some, or none of the choices may be correct.)a. Platelet countb. X-ray of the shoulder and extremity
c. Skin biopsyd. Stool examination for occult bloode. Hemoglobin and hematocrit
Laboratory studies, including complete blood cellcount and serum acid phosphatase, alkaline phosphatase, calcium, and phosphorus determinations,were normal. X-ray ofthe right shoulder and upperextremity revealed soft tissue hemangiomatosis,with resorption of the distal ulna resembling a"licked candystick" (Fig. 7); chest x-ray film revealed deformed upper ribs. A skin biopsy specimenshowed typical features of a hemangioma. Hemoccult examination was negative.
13. The best diagnosis for this woman is (Choose singlebest response.)a. Maffucci's syndromeb. Kasabach-Merritt syndrome
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c. Gorham's syndromed. Klippel-Trenaunay syndromee. Blue rubber bleb nevus syndrome
14. Gorham's syndrome may exhibit each of the following except (Choose single best response.)a. hemangiomasb. lymphangiomasc. osteolysisd. spontaneous fracturese. enchondromas
15. Gorham's syndrome usually occurs (Choose singlebest response.)a. at birthb. in infancyc. in the first decade of lifed. in the second decade of lifee. in the third decade of life
16. Laboratory testing of patients with Gorham's syndrome usually reveals (Choose single best response.)a. normal or negative findingsb. anemiac. thrombocytopenia
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d. hypercalcemiae. hypophosphatemia
Directions for questions 17-20: Select the onelettered item that is most closely related to eachnumbered item.
a. Maffucci's syndromeb. Kasabach-Merritt syndromec. Cobb's syndromed. Blue rubber bleb nevus syndromee. Klippel-Trenaunay syndrome
17. Thrombocytopenia
18. Autosomal dominant inheritance
19. Dyschondroplasia
20. Gastrointestinal hemangiomas
21. The treatment of choice for Gorham's syndrome is(Choose single best response.)a. prosthetic devicesb. radiation therapyc. chemotherapyd. surgerye. ablation with tunable dye laser
QUESTIONS 22-30
22. Since infancy a 6-year-old girl has had lymphedemaand loose skin folds on the back of the neck (Figs. 8and 9). The most likely diagnosis is (Choose singlebest response.)a. Noonan's syndromeb. Turner's syndromec. Klinefelter's syndromed. Down syndromee. Treacher Collins syndrome
23. Karyotyping of the girl may reveal the following pattern(s) (Choose as many as apply. All, some, or noneof the choices may be correct.)a.47,XXYb.46,XXc.46,XYd. 46,XX with trisomy 21e.45,XO
24. Features of this syndrome commonly include whichof the following? (Choose as many as apply. All,some, or none of the choices may be correct.)a. Short statureb. Gonadal dysgenesisc. Multiple syringomasd. Multiple nevie. Hypoplastic nails
25. Early hormonal replacement with estrogen and prog-
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esterone is essential to improve (Choose as many asapply. All, some, or none ofthe choices may be correct.)a. ultimate statureb. lymphatic drainagec. onset of pubertyd. mental facultiese. all of the above
Directions for questions 26-30: For each numbereditem choose the most appropriate lettered item.
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a. Turner's syndromeb. ~oonan's syndromec. Bothd. Neither
26. Abnormal karyotype
27. Lymphedema
28. Short stature
29. Gonadal dysgenesis
30. Autosomal dominant inheritance
CORRECTION
In the Self-Assessment examination of the American Academy of Dermatologyappearing in the January 1991 issue (1991 ;24: 156-9), incorrect answers were givenfor questions 2-4. The correct answers are as follows: 2, a; 3, a, b; 4, b, c.