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SELF-ASSESSMENT MINI-PROGRAM LAMAR S. OSMENT, M.D.

From the Division of Dermatology, Medical College of the University of Alabama, Birmingham, Alabama

A Rare Disorder A girl was first seen at five years of age with a complaint of recurrent vesicles, bullae and pustules in the same circumscribed .areas of the abdomen and right inner thigh.. The fluid-filled lesions arose from a persisting base of discrete and confluent flesh-colored papules that had been present for 3 years. Moderately severe pruritus was present during acute episodes, which occurred about 4 times per year. Previously, treatment with systemic antibiotics had benefited the acute stage of the lesions but had not affected the papular stage. There was no family history of similar or other skin diseases. On physical examination, the papules and vesicles were found to be about 6 in number, covering only a small portion of the total area of circumscribed lesions on the abdomen and thigh. Each vesicle contained a cloudy fluid and was surrounded by a slight erythema. The lesions on the thigh were arranged in a linear manner. KOH preparations and fungal cultures were negative. Fluid from the vesicles contained numerous grampositive cocci which when cultured on blood agar proved to be Staphylococcus aureus, sensitive to many antibiotics, including benzylpenicillin. A biopsy from a vesicle showed hyperkeratosis and parakeratosis. There was an intraepidermal vesicle containing eosinophilic debris and mononuclear cells. The lower edge of the cleft was suprabasilar. The base of the vesicle had a villous appearance. In the upper stratum malphighii were cells having large, round, homogenous, basophilic nuclei and a hyalinized eosinophilic cytoplasm. These

cells were generally larger than normal squamous cells. In the horny layer there were smaller cells with prominent elongated grain-shaped nuclei.

Fig. 1

0011-9059-78-01 00-0065-0050 @ International Society of Tropical Dermatology

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INTERNATIONAL JOURNAL OF DERMATOLOGY

1 . Which of the following conditions

one may want t o consider as a possible diagnosis in this patient is not usually characterized by vesiculation within the epidermis? A. Familial benign chronic pemphigus B. Pemphigus foliaceaus C . Keratosis follicularis D. Polydysplastic epidermolysis bullosa dystrophica

On the basis of the clinical appearance of the patient, her history, and the histopathologic picture of the biopsy specimen, a diagnosis of Darier’s disease (keratosis follicularis) was entertained and various modalities of treatment were considered.

Answers and Explanations

January/February 1978

Vol. 17

2. Which of the following given in therapeutic doses would least likely provide a beneficial effect on this patient? A. Oral Vitamin A B. Intramuscular Vitamin A C. Topical alpha-tocopherol (Vitamin E) D. lntralesional Vitamin A E. Topical retinoic acid (Vitamin A acid)

3. Which of the following conditions

microscopically most resembles keratosis follicularis? A. Warty dyskeratoma B. Inverted follicular keratosis C. Acrokeratosis verruciformis. D. Epidermodysplasia verruciforrnis

to Self-Assessment Mini-Program

1 . The answer is D. The bullae are always found in a subepidermal location in the polydisplastic type of epidermolysis bullosa dystrophica. The lacunae of keratosis follicularis are intraepidermal, as are the vesicles of pemphigus foliaceous and familial benign chronic pemphigus. 2. The answer is C. Topical alpha tocopherol has not been reported to be efficacious. The other 4 preparations are‘ helpful and represent various forms of Vitamin A or its acid. Perhaps the least risky method yielding satisfactory results is topical retinoic acid. 3. The answer is A, warty dyskeratoma, a solitary benign tumor of the skin. This lesion occurs primarily in older adults and appear as elevated nodules with a central “pore.” A single follicle is involved in contrast to multiple areas in Darier’s disease. Graham, Johnson, and Helwig4 believe this lesion to be an

isolated form of keratosis follicularis. However, others could not find any case of warty dyskeratoma having any other manifestation of Darier’s disease. Invested follicular keratosis is also a solitary lesion, appearing usually o n the face. It fails to exhibit epidermal lacunae. Both acrokeratosis,verruciformis and epidermodysplasia verruciformis appear as multiple lesions on extremities. Neither shows clinical vesiculation or microscopic lacunae. References 1 . Kellum, R. E., and Haserick, J. R.: Localized linear keratosis follicularis. Arch. Dermatol. 86:450, 1962. 2. Mehregan, A. H.: Inverted follicular keratosis. Arch. Dermatol. 89:229, 1964. 3. Lever, W. F.: Histopathology of the Skin. 3rd edition. Philadelphia, J. 8. Lippincott Co., 1961, pp. 423, 99- 58, 61, 56, 63. 4. Graham, J. H., Johnson, W. C., and Helwig, E. B.: Dermal Pathology. Hagerstown, MD, Harper & Row Publishers, 1972, p. 547.