pemphigus vulgaris

PEMPHIGUS VULGARIS

This adult male developed extensive blisters and erosions on several sites including the scalp. There is no family history of a similar problem. There was relative sparing of the intertriginous areas.

The clinical presentation and pathology are typical for pemphigus vulgaris. Immunofluorescent studies confirmed the diagnosis.

Although pemphigus is thought of as an acantholytic disease, there usually is not as much acantholysis of individual cells as is seen in Hailey-Hailey disease (benign chronic familial pemphigus). The epidermis in pemphigus vulgaris tends to slough off above the basal layer in a relatively intact fashion though there are individual acantholytic cells. Follicular involvement, as is seen in this case, is a very helpful diagnostic finding.

The scalp involvement is the most striking feature in this photo. The lip involvement does not show up very well. There are erosions on the neck. Most of the vesicles have ruptured.

Close-up view of the scalp. It is difficult to state whether the yellow, granular areas represent secondary impetiginization or verrucoid hyperplasia of pemphigus vegetans type since such foci were not biopsied.

Low power view. A suprabasal split involves the epidermis and a hair follicle. So-called villi are composed of a layer of basal cells over connective tissue cores. The epidermis is not seen in the biopsy as it has sloughed off.

Medium power view of above. Eosinophilic leukocytes are not seen in this case though they can be seen in other cases, particularly in the vegetans variant..

High power view of part of top picture. A chunk of epithelium plus a few acantholytic cells are found in the lumen of this hair follicle. There is no dyskeratosis of the type found in warty dyskeratomas.

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	The scalp involvement is the most striking feature in this photo. The lip involvement does not show up very well. There are erosions on the neck. Most of the vesicles have ruptured.
	Close-up view of the scalp. It is difficult to state whether the yellow, granular areas represent secondary impetiginization or verrucoid hyperplasia of pemphigus vegetans type since such foci were not biopsied.

	Low power view. A suprabasal split involves the epidermis and a hair follicle. So-called villi are composed of a layer of basal cells over connective tissue cores. The epidermis is not seen in the biopsy as it has sloughed off.
	Medium power view of above. Eosinophilic leukocytes are not seen in this case though they can be seen in other cases, particularly in the vegetans variant..
	High power view of part of top picture. A chunk of epithelium plus a few acantholytic cells are found in the lumen of this hair follicle. There is no dyskeratosis of the type found in warty dyskeratomas.