Self-Assessment

Self-Assessment examination of the AmericanAcademy of Dermatology* Identification No, 889·207

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 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, 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 1-12

A 36-year-old woman has darkly pigmented ma­cules on most of the left leg (Fig. 1). On closeinspection these lesions are within a light tan, rela­tively well-defined macular area (Fig. 2). The lesionhas been present since childhood. The linear verticalsurgical scar is unrelated.

1. Hyperpigmentation of darker speckles that are flat,raised, or both within a macular cafe-au-lait spot ismost commonly called (Choose the single bestresponse.)a. speckled lentiginous nevusb. segmentallentiginosisc. nevus spilusd. Becker's nevuse. zosteriform lentiginous nevus

2. Wood's light examination of this patient would(Choose as many as apply. All, some, or none ofthechoices may be correct.)a. accentuate the difference in color of the cafe-au­

lait portion of the nevus spilus in comparison tonormal skin

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 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 Kerde\, MD, Donald PaulLookingbill, MD, Emily F. Omura, MD, Warren W. Piette, MD,Dennis C. Polley, DO, Sharon Ann Smith Raimer, MD, and Ken­neth Joseph Tomecki, MD.

b. make the speckles look darker against the cafe­au-Iait background

c. possibly not be useful if the patient's nonnal skinis too dark (skin types V, VI)

d. make the speckles lighter against the cafe-au-laitbackground

e. make the lesions unnoticeable

3. Each ofthefollowing statements is true about thesizeand distribution ofnevus spilus except itmay (Choosethe single best response.)a. have a segmental distributionb. have a zosteriform distributionc. occur on mucosal surfacesd. be less than 1 cm or greater than 10 cme. occur in a divided form along the upper and lower

eyelids

4. Each of the following statements is true about nevusspilus except which two?a. It may be present at birth.b. It is found most commonly on the trunk and

extremities.c. It may arise during early childhood.d. It is more common in boys.e. Within one lesion the speckles may be either mac­

ular or papular, but not both.

S. Fig. 3demonstrates the light microscopic appearanceof a raised, dark speckle from a nevus spilus. Fig. 4displays the findings from an area of surroundingmacular hyperpigmentation. On histologic examina­tion you can expect to see each of the following ex­cept (Choose the single best response.)a. decreased number of melanocytes in the pig­

mented background with each melanocyte beingdendritic, strongly dopa-positive, and containingmany stage IV melanosomes

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b. lentiginous melanocytic hyperplasia within thedark flat speckles of the nevus spilus

c. collections of nevus cells in the epidermis, dermis,or both of the raised areas in the nevus spilus

d. epithelioid cell-spindle cell nevi in the raised areasof the nevus spilus

e. nevus cells present in the tan background pigmen­tation of the nevus spilus

6. Melanin macroglobules may be seen within the cyto­plasm of me1anocytes from the cafe-au-lait-like ar­eas of nevus spilus (Fig. 4). These also may be foundin eachofthe following except (Choose the singlebestresponse.)

a. normal skinb. cafe-au-lait macules of neurofibromatosisc. melanotic macules of Albright's syndromed. tinea versicolore. ephelides

7. Which two facts are true regarding nevus spilus?a. Its prevalence in adults is approximately 2%.b. It is found more frequently in patients with mel­

anomas.c. The evidence for an association between nevus

spilus and melanoma is conclusive.d. There is some evidence that nevus spilus may be

related to neurofibromatosis.e. There is a higher incidence of melanomas in rela­

tives of patients with nevus spilus.

For each numbered item choose the most appro-priate lettered response:

a. Segmentallentiginosisb. Nevus spilusc. Bothd. Neither

8. Background is normal skin

9. Can occur at birth

10. More prevalent in males

11. Absence of nevus cells on histologic examination

12. Presence of nevus cells on histologic examination

QUESTIONS 13-24

A 42-year-old black woman has a slate-gray ma­cule 60 X 45 em on the anterolateral left thigh (Fig.5). The lesion is uniformly pigmented with relativelydistinct borders; it has been present since birth andhas not changed. Wood's light examination revealsno enhancement of the lesion.

13. Which of the following can be concluded from

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Wood's light findings? (Choose the single bestresponse.)a. Pigment is located only in the epidermis.b. Pigment is located only in the dennis.c. Pigment is located in the epidermis and dermis,

but mostly in the epidermis.d. Pigment is located in the epidermis and dermis,

but mostly in the dennis.e. None of the above is correct.

Histopathologic examination ofthe lesion (Fig. 6)shows numerous wavy dendritic pigmented cellswith evenly dispersed melanin granules scatteredsparsely throughout the reticular dermis. Melano­phages are absent.

14. The most likely diagnosis isa. giant dermal melanocytosis (persistent mongolian

spot)b. cafe-au-Iait spotc. giant nevus spilusd. congenital "garment" nevocellular nevuse. nevus of Ito

15. Each of the following statements is true about typi­cal mongolian spots except (Choose the single bestresponse.)a. they can occur in extrasacral areasb. most disappear within the first 5 years of lifec. lesions on the lumbosacral area sometimes are

mistaken for child abused. they usually appear after the first year of lifee. they are thought to derive from melanocytes of

neural crest origin that are retained in the dermiswhile migrating to the epidermis during fetal de­velopment

16. Approximately what percentage of white infantshave mongolian spots? (Choose the single bestresponse.)a.O.l%b.0.5%c.l0%d.25%e.50%

17. Each of the following is an example of dermal mel­anocytosis except (Choose the single best response.)a. blue nevusb. nevus fuscoceruleus ophthalmomaxillarisc. nevus fuscoceruleus acromiodeltoideusd. Becker's nevuse. mongolian spot

18. Which two of the following disorders have rarelybeen associated with malignant degeneration?a. blue nevus

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b. mongolian spotc, nevus of Otad. melasmae, melanotic macules of Albright's syndrome

19. Each of the following statements is true about persis-

tent mongolian spots except (Choose the single bestresponse.)a. they are usually solitary, although there may be

more than one lesionb, most are more than 3 em in diameter

Volume 21Number 1July 1989

c. they occur in approximately 4% of Japanese mend. they are found most frequently on the buttocke. they appear blue-gray because of the Tyndall

phenomenon in which longer wavelengths of visi­ble light are absorbed by the dermal melanin,whereas the shorter wavelength (blue) is reflectedback to the viewer's eye

20. Each of the following statements is true about the ul­trastructure of mongolian spots except (Choose thesingle best response.)a. dermal melanocytes in mongolian spots possess an

extracellular sheath that is primarily filamentousb. the sheath of melanocytes in a regressing mongo­

lian spot is reduced and the plasma membranedisintegrates, thereby discharging melanin gran­ules into the dermis

c. melanocytes with a reduced filamentous sheathare not found in the mongolian spots of fetuses

d. the illamentous sheaths ofthe dermal melanocytesin the persistent mongolian spots are not reduced

e. dermal melanocytes in persistent mongolian spotsare found localized around the blood vessels in thedermis

For each numbered item choose the most appro-priate lettered response:

a. Persistent mongolian spotb. Typical mongolian spotc. Bothd. Neither

21. Can frequently appear after age 5

22. Found most frequently in the sacrococcygeal region

23. Found most frequently in the shoulder region

24. Color is influenced by the amount of melanin in der­mal melanocytes as well as their number and loca­tion.

QUESTIONS 25-36

Two years ago this 56-year-old woman had tensebullae on an erythematous base located primarily onthe medial aspects of both upper and lower extrem­ities (Fig. 7). In some areas the lesions were erythe­matous plaques associated with pruritus. Themucosal surfaces were not involved. Laboratoryevaluations showed a normal complete blood cellcount, negative antinuclear antibody and anti-DNA,and normal liver profile. She was not taking oralmedications.25. Your differential diagnosis should include (Choose as

many as apply. All. some, or none oJthe choices maybe correct.)a. porphyria cutanea tarda

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b. pemphigusc. erythema multiformed. bullous pemphigoide. epidermolysis bullosa acquisita

A biopsy specimen ofskin adjacent to a blister re­vealed a spongiotic epidermis with underlying pap­illary dermal edema and an inflammatory cell infil­trate consisting of lymphocytes, neutrophils, andoccasionallyeosinophils (Fig. 8). Direct immuno­fluorescence of perilesional skin showed prominentlinear deposits of IgG (Fig. 9) and C3 at the base­ment membrane zone.

26. Which two diseases can best fit this histologic pat­tern?a. Porphyria cutanea tardab. Pemphigusc. Erythema multiformed. Bullous pemphigoide. Epidermolysis bullosa acquisita

The patient was treated with oral corticosteroids indoses as high as 100 mg of prednisone daily withpoor response. She also received dapsone in doses ashigh as 200 mg daily to no avail. After the treatmentwas discontinued, the disease eventually remittedspontaneously and the patient was without symp­toms until recently when she had blisters anderosions, with no inflammatory base located prima­rily on extensor aspects and frequently traumatizedareas of the extremities. On healing these lesionsshowed an atrophic surface frequently studded withmilia (Figs. 10 and 11).

27. On the basis ofthis new episode the differential diag­nosis should include (Choose as many as apply. All,some, or none of the choices may be correct.)a. bullous pemphigoidb. porphyria cutanea tardac. hereditary dystrophic epidermolysis bullosad. epidermolysis bullosa acquisitae. pemphigus

The patient has no history of photosensitivity or al­cohol use. The uroporphyrin levels in urine are nor­maL There is no family history of mechanobullousdisorders. A new specimen of skin is obtained forexamination that shows a paucity of inflammatorycells in the dermis with fibrosis separating append­ageal structures. Examination for direct immuno­fluorescence shows a similar pattern as seen in thepatient's first specimen.

28. On the basis of this information you can limit yourdiagnosis to (Choose single best response).

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a. bullous pemphigoidb. porphyria cutanea tardac. hereditary dystrophic epidermolysis bullosad. epidermolysis bullosa acquisitae. pemphigus

Indirect immunofluorescence titers of serum frompatients with epidermolysis bullosa acquisita canhelp differentiate it from bullous pemphigoid.

29. What percentage of patients with epidermolysis bul­losa acquisita will have circulating anti-basementmembrane zone autoantibodies? (Choose single bestresponse.)a.lO%b.90%c.25%-50%d. Nonee. 100%

30. The epithelial substrate that is used routinely toscreen for the presence of anti-basement membranezone autoantibodies in patients with suspected epi­dermolysis bullosa acquisita or bullous pemphigoid is(Choose single best response.)a. mouse liverb. monkey esophagusc. a blister from the patientd. normal human skine. none of the above

31. A special indirect immunofluorescence techniquethat uses as substrate normal human skin split alongthedermoepidermaljunction (Fig. 12) may discrim­inate the staining patterns ofbullous pemphigoid andepidermolysis bullosa acquisita when significant ti­ters of anti-basement membrane zone autoanti­bodies are present in serum. The technique that ar­tifactually creates a subepidermal split in the normalhuman skin substrate for this assay uses (Choose sin­gle best response.)a. sodium chloride solutionb. strong alkalic. acetic acidd. split-dopa techniquee. diastase

32. With this technique (described in question 31) youexpect the following pattern of immunofluorescencestaining for the two diseases: (Choose as many asapply. All, some, or none ofthe choices may be cor­rect.)a. In bullous pemphigoid the stain will be in the der­

mal side.

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Dermatology

b. In bullous pemphigoid the stain will be in the epi­dermal side.

c. In epidermolysis bullosa acquisita the stain will be·in the dermal side.

d. In epidermolysis bullosa acquisita the stain will bein the epidermal side.

e. In bullous pemphigoid the staining pattern inspeckled whereas in epidermolysis bullosa ac­quisita is linear.

33. What other confirmatory diagnostic methods areavailable to differentiate between bullous pemphig­oid and epidermolysis bullosa acquisita? (Choose asmany as apply. All, some. ornone ofthe choices maybe correct.)a. DNA hybridizationb. Immunoblottingc. Immunoelectron microscopyd. Isoenzymatic degradatione. None of the above

34. The epidermolysis bullosa acquisita antigen identi­fied by immunoblotting is a polypeptide with molec­ular weights (of) (Choose as many as apply. All,some. or none of the choices may be correct.)a. 290 and 145 kDb. 220 leDc. identical to laminind. with characteristics of type VII procollagene. with characteristics of type IV collagen

35. Epidermolysis bullosa acquisita has been associatedwith the following systemic disorders: Choose asmany as apply. All. some, or none ofthe choices maybe correct.)a. systemic lupus erythematosusb. inflammatory bowel diseasec. multiple endocrinopathy syndromed. rheumatoid arthritise. all of the above

36. Epidermolysis bullosa acquisita has been notoriouslyresistant to different treatments. Recently one treat­ment has shown promising, albeit preliminary, re­sults. This treatment is (Choose single best response.)a. pulsed oral corticosteroidsb. prednisone and cyclophosphamidec. dapsoned. cyclosporinee. azathioprine