Answers for Self-Assessment examination of the American Academy of Dermatology

.~. ~ ~'~,

-¢. Iq . t~ £~"

I I I IIIlll III Ill I

|OURNAL o f lhe

A m e R i c a N A c a D e m Y OF

D e r M a T O L O G Y I I II I I

Answers for Self-Assessment examination of the American Academy of Dermatology

Identification No. 887-210

O c t o b e r 1987 issue o f the JOURNAL OF THE AMERICAN ACAD-

EMY OF DERMATOLOGY

DISCUSSION OF QUESTIONS 1 AND 2

A summary of this patient's history, clinical findings, and laboratory data confirms the presence of the syndrome of yon Gierke's disease associated with excessive production of uric acid. This con- dition is unrelated to Fabry's disease and is not associated with hyperlipoproteinemia. It is not a manifestation of hereditary Down's syndrome. Therefore, a low cholesterol diet is of no value in the therapy of this patient, but all of the other treatment modalities mentioned are of benefit.

For this series the recommended choices are: 1, e; 2, d.

Richard K. Scher, M.D., Bay Shore, NY

REFERENCES Kelley WM, et al. Excessive production of uric acid in

type I glycogen storage disease. J Pediatr 1968;72:488. Moses SW, Gutmann A. Inborn errors of glycogn metabolism.

In: Schulman I, ed. Adv Pediatr 1972;19:95.

DISCUSSION OF QUESTIONS 3-5

This patient has the nail-patella syndrome, otherwise known as osteo-onychodysplasia. This is an autosomal dominant disorder characterized by variable combinations of nail dysplasia, skeletal deformities, and renal disease. It is not hormonally caused and therefore evaluation of the thyroid and parathyroid systems is not necessary. This con- dition is not associated with liver disease. My- cologic studies are not necessary since this con- dition is not associated with a fungal infection of the nails. Furthermore, the syndromes of dyskera- tosis congenita, pachyonychia congenita, and epidermolysis bullosa do not fit the clinical picture.

The most serious complication of this disorder is the development of nephrotic syndrome associated with hypertension and uremia as a result of focal glomerular scarring, Intimal proliferation and hy- alinization of the small- and medium-sized renal arteries with reduplication of the internal elastic lamina are seen. The nail changes are characteristic and should alert the physician to the associated systemic findings.

For this series the recommended choices are: 3, d; 4, b; 5, d.


REFERENCES

Leahy MD. The hereditary nephropatby of osteo-onychodysplasia nail patella syndrome. Am J Dis Child 1966; 112:237-41,

Morita T. Nail-patella syndrome. Arch Intern Med 1973; 131:271.

Zimmerman C, Horns I. A pathognomonic roentgen sign of familial onycho-osteodysplasia. Am J Roentgenol 1961; 86:478-83,


The diagnosis in this case is multiple gloman- giomas. Multiple tumors occur most frequently in children and are much more common in male than in female patients. They can be associated with pain, temperature changes, and hyperhidrosis. The persistence of these lesions and their increase in size, combined with the other symptoms, essen- tially rules out congenital capillary hemangiomas. The diagnosis here is confirmed histologically with the characteristic finding of the large vascular spaces with an irregular shape lined by a single layer of flat endothelial cells with cuboidal cells in close association. Blue rubber bleb nevus syndrome is unlikely because of the absence of gas- trointestinal involvement as well as the absence of anemia. Although the overlying skin in this syndrome may be hyperhidrotic, this alone is insuf- ficient to make this diagnosis.

39A

42A Self-Assessment examination answe~"

J o u r n a l o r the

American Academy of Dermatology

For this series the recommended choices are: 6, a; 7, c; 8, c.


REFERENCES Goodman TF, Abele DC. Multiple glomus tumors. A clinical

and electron microscopic study. Arch Dermatol 197I; 103:11-23.

Larsen FS, Hage E. Multiple glomus tumors. A report of a family in Denmark. Acta Derm Venereol (Stockh) 1979; 59:180-2.

Tarnowski W, Hashimoto K. Multiple glomus tumors. An ultrastructure study. J Invest Dermatol 1969;52:474-8.


Fig. 1 shows an obese female patient who, following bowel bypass surgery for her obesity, de- veloped characteristic features of the bowel bypass syndrome, consisting of a neutrophilic dermatitis associated with polyarthritis, tendonitis, malaise, fever, enteropathy, and cryoglobulinemia. The skin lesions consist of small, tender, erythematous papules appearing singly or in crops over the arms (shown), legs, and abdomen. Fig. 2 shows the purple papules of variable size characteristic of early lesions of Kaposi 's sarcoma. Fig. 3 shows large, indurated, variable-sized papules, some of which are ulcerated, characteristic of cutaneous lesions of Hodgkin's disease. Fig. 4 shows the fine, superficial, annular, scaly lesions typical of disseminated superficial actinic porokeratosis. Fig. 5 shows the follicular inflammation characteristic of Pseudomonas folliculitis.

For this series the suggested responses are: 9, d; 10, c; 11, b; 12, a; 13, e.

Paul S. Wolfish, M.D., Los Angeles, CA, Thomas A. Chapel, M.D., Dearborn, 3/11,

Thomas R. Wade, M.D., Atlanta, GA, and W. Clark Lambert, M.D., Ph.D., Newark, NJ

REFERENCES Benninghoff DL, Medina A, Alexander LL, et al. The mode

of spread of Hodgkin's disease to the skin. Cancer 1970;26:1135-40.

Chernosky ME, Freeman RG. Disseminated superficial actinic porokeratosis. Arch Dermatol 1967;96:611-24.

Ely PH, The bowel bypass syndrome: A response to bacterial peptidoglycans. J AM ACAD DERMATOL 1980;2:473-87.

Myskowski PL, Romano JF, Safai B. Kaposi's sarcoma in young homosexual men. Cutis 1982;29:31-4.

Sausker WF, Aeling JL, Fitzpatrick JE, Judson FM. Pseu-

domonas folliculitis acquired from a health spa whirlpool. JAMA 1978;239:2632-5.


Fig. 6 shows an implanted hair in the sole of the foot of a man who owned a dog (collie) and apparently stepped on a hair of the dog. Such lesions are commonly seen on the hands and fingers of hairdressers and barbers. Histologically, a foreign body reaction is present and the hair itself may be seen. Fig. 7 shows the characteristic bur- rowing lesion of carcinoma cuniculatum, which shows histologically crypts lined by deceptively bland-appearing squamous cells extending deep into the dermis. This process is locally invasive but slow to metastasize. Fig. 8 shows the foot of an infant with the subepidermal blistering process characteristic of epidermolysis bullosa dystrophicans. Histologically, a more noninflammatory subepidermal bullous process is seen. Fig. 9 shows the markedly hyperkeratotic lesion typical of kera- toderma palmare et plantare.

For this series the recommended responses are: 14, c; 15, d; 16, b; 17, a.

John R. Haserick, M.D., Pinehurst, NC, Ronald J. Barr, M.D., Davis, CA,

Isaac Willis, M.D., Atlanta, GA, and W. Clark Lambert, M.D., Ph.D., Newark, NJ

REFERNCES B auer EA, Briggaman RA. The mechanobullous diseases. In:

Fitzpatrick TB, et al, eds. Dermatology in general med- icine, 3rd ed. New York: McGraw-Hill, 1986:334-47.

Fred HD, et al. Keratosis palmaris et plantaris. Arch Intern Med 1964;113:866-71.

Jackson CE, Callies QC, Krull EA, Mehregan A. Hairy cutaneous malformations of palms and soles. Arch Dermatol 1975;111:1146-9.

Kao GF, Graham JH, Helwig EB. Carcinoma cuniculatum. Cancer 1982;49:2395-403.

DISCUSSION OF QUESTIONS 18 AND 19

Fig. 10 shows an infant with the subepidermal bullae and denuding skin characteristic of epidermolysis bullosa dystrophicans. This distribution and the clinical appearance of the lesions resemble those of staphylococcal scalded skin syndrome, which, however, shows more widespread involve-

44A Self-Assessment examination answers

Journal of the American Academy of

Dermatology

ment and less erythema than the presented case. It is due to an inherited defect in dermoepidermal structural elements. Fig. 11 shows an intact bulla typical of bullous impetigo, which may be caused by staphylococcus and may occur secondary to scabies; this was in fact true in the present case.

For this series the recommended responses are: 18, b; 19, b.

Ronald J Barr, M.D., Davis, CA, Thomas A. Chapel, M.D., Dearborn, MI, and W. Clark Lambert, M.D., Ph.D., Newark, NJ

REFERENCES Bauer EA, Briggaman RA. The mechanobullous diseases. In:

Fitzpatrick TB, et al, eds. Dermatology in general med- icine. 3rd ed. New York:McGraw-Hill, 1986:334-47.

Estes SA: Scabies: the diagnostic dilemma. Arizona Med 1978;35:477-9.

DISCUSSIONS OF QUESTIONS 20-22

Fig. 12 shows a deep, indurated, ulcerated process typical of epithelioid sarcoma. This malignant tumor of soft tissues, first described by Enzinger in 1970, tends to occur in young adults and pri- marily involves the superficial soft tissues of the hand, forearm, and pretibial regions. It is the most common sarcoma of the hand. The clinical dif- ferential diagnosis includes chronic cellulitis, gan- grene, superficial and deep fungal infections, sar- coidosis, and necrobrosis lipoidica. Histologically, one sees large nodules consisting of eosinophilic epithelioid cells with prominent central areas of necrosis or of hyalinized collagen. The epithelioid cells show pleomorphism and mitoses are quite common, which is of great help in formulating a correct diagnosis.

Fig. 13 shows a fleshy, exophytic, ulcerated, nodular tumor mass typical of a cutaneous lesion of Hodgkin's disease. Such cutaneous metastases are relatively uncommon, in contrast to nonspecific cutaneous manifestations of Hodgkin's disease such as pruritus, prurigolike papules, urti- caria, and exfoliative dermatitis. Histologically, the Reed-Sternberg cell, which is diagnostic of Hodgkin's disease and which, depending on the type, is easily demonstrated in deep lesions in many cases, is rarely found in the skin tumors.

For this series the recommended choices are: 20, b; 21, a; 22, d.

Paul S. Wolfish, M.D., Los Angeles, CA, Ronald J. Barr, M.D., Davis, CA, and W. Clark Lambert, M.D., Newark, NJ


Fig. 14 shows tumor composed of spindle- shaped cells in the "storiform" pattern characteristic of dermatofibrosarcoma protuberans. The term storiform is derived from the Latin storia, or mat, referring to a mat made of hemp or hemplike material similar to mats currently used as "wel- come mats" on doorsteps. The intertwining cells of the dermatofibrosarcoma protuberans are thought to resemble the coarse intertwining fibers of the mat. Dermatofibrosarcoma protuberans is an uncommon, locally invasive and destructive tumor that is slow to metastasize. Wide excision is necessary to prevent recurrence.

Fig. 16 shows a tumor composed of spindle- shaped cells between which erythrocytes are per- colating. The lesion shows no tendency to have a structure or any sort of order. These changes are characteristic of Kaposi's sarcoma. Kaposi's sarcoma occurs in several clinical settings, including acquired immunodeficiency syndrome (AIDS) and a classical type of presentation in elderly men of Mediterranean region ancestry. It is thought to be largely an angiomatosis rather than a straightfor- ward malignancy, with many new lesions arising de novo rather than as metastases from older lesions.

Fig. 17 shows a solid lesion composed o f bland spindle-shaped basophilic cells, the nuclei of which show a striking tendency to line up side by side in parallel rows (known as palisading; left side of figure). Thin-walled, dilated blood vessels are also found in the tumor. On the right is a less structured portion of the lesion, which also shows some mucin deposition (right side of figure). This lesion thus shows the characteristic features of a schwannoma, a neurilemmoma. Since there is no structure called a "neurilemma," the former term is preferred. Of the above, the former, more structured histologic presentation is known as an An- toni A area and the latter, less structured presentation as an Antoni B area (Antoni B areas are


Journal of tile American Academy of

Dermatology

often even less structured than seen here). Dilated, thin-walled blood vessels are often seen in schwannomas, especially in Antoni B areas, which may also show necrosis and hemorrhage. These thin- walled vessels contrast with the thick-walled mus- cular blood vessels often seen in leiomyomas (be- nign tumors of smooth muscle). Leiomyomas may, however, show palisading of nuclei, in that respect resembling schwannomas.

Fig. 18 shows a lesion composed of spindle- shaped cells resembling smooth muscle cells in a structured but ill-defined pattern. These are ac- tually myofibroblasts and the lesion is an example of congenital fibromatosis (infantile myofibromatosis), an unusual type of juvenile fibromatosis. Patients born with these nodules usually do well following simple surgical excision. Fig. 15 shows a tumor composed of smooth muscle cells forming fascicles. This lesion is an example of a smooth muscle hamartoma; it could also be considered a leiomyoma.

For this series the recommended choices are: 23, d; 24, c; 25, a.

Ronald J. Barr, M.D., Davis, CA, Paul S. Wolfish, M.D., Los Angeles, CA, and W, Clark Lambert, M.D., Ph.D., Newark, NJ

REFERENCES Chung EB, Erizinger FM. Infantile myofibromatosis. Cancer

1981;48:1807-18. Hajdn SI. Pathology of soft tissue tumors. Philadelphia:Lea

& Febiger, 1979:82-94. Hashimato K, Brownstein MH, Jacobile FA. Dermatofibro-

sarcoma protuberans. A tumor with perineural and en- doneural cell features. Arch Dermatol 1974;100:874-85.

Sian CS, Ryan SF. The ultrastructure of neurilemmona with emphasis on Antoni B tissue, Hum Path01 1981;12: 145-60.

Templeton AC, Bhana D. Prognosis in Kaposi's sarcoma. J Natl Cancer Inst 1975;55:1301-4.


Fig. 19 shows several large sporangia, some of which contain endospores, of Rhinosporidium see- beri in the submucosal tissue of a biopsy specimen obtained from the nasal cavity. The periodic acid- Schiff stain with picric acid counterstain clearly demonstrates the bright red organisms against a pale yellow background. Rhinosporidiosis is

endemic in Sri Lanka and India but has also been reported in South America, the Southern United States, and, rarely, in Europe. Multiple polyps of the nasal mucosa or, less commonly, of other mucous membranes are seen. The disease is most common in children and young adults with males more frequently affected than females.

Fig. 20 shows Actinomyces israelii, the prin- cipal cause of actinomycosis in man. In the he- matoxylin and eosin-stained section, the center of the granule often stains basophilic, surrounded by pink clublike structures, Around this there is ho- mogeneous eosinophilic material, probably composed of antigen-antibody complexes and debris from inflammatory cells; this amorphous pink de- posit is known as the Splendore-Hoeppli phenom- enon and is a nonspecific finding also seen around foreign body reactions, bacteria, fungi, and some helminths. The photomicrograph shows a "sulfur granule" of actinomycosis; other organisms may also show sulfur granules but with different mor- phology. Those of Allescheria boydii and Exophi- ala jeanselmei show hyphae rather than thin basophilic filaments as seen here; those of botryo- mycosis show cocci in the center.

Fig. 21 shows Histoplasma capsulatum, the cause of histoplasmosis, with the 2- to 4-Ix organisms present within histiocytes where they are surrounded by a halo, or pseudocapsule. Oc- casional larger organisms, up to 15 I~ in diame- ter, may occur. The organism stains well with the periodic acid-Schiff and Gomori's methena- mine silver stains. Cutaneous lesions due to this organism almost always reflect disseminated disease.

Fig. 22 shows Exophiala (Phialophora)jeanselmei, a fragment of brown-black grain from a typical Madura foot (mycetoma). Mycetomas are tumorlike skin lesions with grains such as this one in the associated exudate. They are usually considered to be in two groups, porokaryotic (due to Actinomyces israelii and bacteria) and eukaryotic (eumycetomas) (due to true fungi). A number of pathogens may be responsible for either type. Eu- mycetomas respond poorly to chemotherapy and affected extremities may have to be amputated.

Fig. 23 shows a dermatophytosis with pig-


Journal of the American Academy of

Dermatology

mented hyphae in the superficial stratum corneum, with deeper structures spared. These two features are diagnostic of tinea nigra palmaris, caused by Exophiala (Cladosporium) werneckii, the organism illustrated here. In Asia, CIadosporium man- soni may also cause tinea nigra.

For this series the recommended responses are: 26, c; 27, d; 28, e; 29, b; 30, a.

Ronald J. Barr, M.D., Davis, CA, and W. Clark Lambert, M.D., Ph.D,, Newark, NJ

REFERENCES Binford CH, Connor DH, eds, Pathology of tropical and ex-

traordinary diseases. Washington, DC: Armed Forces in- stitute of Pathology, 1978:vol 2.

Johnson WC. Parasitic infections and deep mycoses. In: Gra- ham JH, Johnson WC, Helnig EB, eds. Dermal pathology. Hagerstorm, MD: Harper & Row, 1972:451-75.

Salfelder K. Atlas of deep mycoses, Philadelphia: WB Saun- ders, 1980.

Documents

Answers for Self-Assessment examination of the American Academy of Dermatology