NOVEMBER 2000

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A.B.

Histopathology:

Fig A: Hyperkeratotic epidermis with acanthosis and some suprabasal lacunae formation

Fig B: Large collections of eosinophils within lacunae and follicles

DIF: Showed interspinous IgG deposition

Discussion:

The pemphigus diseases are autoimmune intraepidermal blistering diseases of the skin and mucous membranes. The pemphigus vulgaris antigen is desmoglein-3, a keratinocyte cell surface antigen. Pemphigus vegetans is an uncommon variant of pemphigus vulgaris. It occurs in 1-2% of pemphigus vulgaris cases. The median age of onset is 40-50 years. Two clinical subtypes of pemphigus vegetans exist, characterized initially by flaccid bullae and erosions (Neumann) or pustules (Hallopeau). Both subtypes subsequently develop into hyperpigmented vegetative plaques with pustules and hypertrophic granulation tissue at the periphery. Lesions are typically located at intertriginous areas and the oral mucosa. Oral involvement is present in nearly all pemphigus vegetans cases. A characteristic feature of pemphigus vegetans is the cerebriform tongue, characterized by a pattern of sulci and gyri on the dorsum of the tongue.

Histologically, early lesions of pemphigus vulgaris and pemphigus vegetans show suprabasal acantholysis. Pemphigus vegetans also exhibits epidermal hyperplasia, papillomatosis, and intraepidermal eosinophilic abscesses as lesions age. Immunofluorescence findings in pemphigus vegetans are indistinguishable from those of pemphigus vulgaris. DIF demonstrates deposition of IgG and C3 on the cell surface of keratinocytes. Indirect immunofluorescence reveals circulating antiepithelial cell surface IgG.

Systemic glucocorticoids are the mainstay of treatment. Addition of immunosuppressive agents, such as cyclophosphamide, azathioprine, or intramuscular gold, may improve remission rates and allow for a steroid-sparing effect. Hallopeau patients have few if any relapses, while Neumann patients have relapses and remissions similar to pemphigus vulgaris patients.

References:

I .Fitzpatrick's Dermatology in General Medicine, 5 th ed. (Freedberg IM, Eisen AZ, Wolff K, AustenKF, Glodsmith LA, Katz SI eds.). New York: McGraw-Hill, 1999. pp 654-66.

2. Ahmed AR and Blose DA. Pemphigus vegetans. International Journal of Dermatology 23(2):135-41,1984.

3. Becker BA and Gaspari AA. Pemphigus vulgaris and vegetans. Dermatologic Clinics 11 (3):429-52,1993.

4. Hurt WC. Observation on pemphigus vegetans. Oral Surgery, Oral Medicine, & Oral Pathology 20(4):481-7, 1965.

5. Korman N. Pemphigus. Journal of the American Academy of Dermatology 18(6):1219 38, 1988.

I would like to thank Drs. Helen Kim, John Streidl , Christina Shimek , and Michael Heffernan for their assistance in preparing this case.