Oral pemphigus vulgaris. A report of ten cases

Abstract

The conditions of ten patients were diagnosed as pemphigus vulgaris limited to the oral cavity. In eight patients the histopathologic findings were consistent with oral pemphigus. Direct immunofluorescence demonstrated intercellular substance (ICS) deposition of C3 either alone or in combination with IgG, IgA, IgM, C4, C1q, properdin, and factor B in all nine patients tested. Circulating ICS antibodies were detected initially in eight of the ten patients, nine of whom had positive titers at a later date, without subsequent development of cutaneous lesions. Physicians should be alerted to a diagnosis of pemphigus in patients who have chronic erosive disease confined to the oral cavity. Prompt diagnosis by means of routine histopathologic and immunofluorescence studies followed by early initiation of treatment are warranted.

Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris

“…Patients with PF, however, do not develop blisters or erosions of the mucous membranes. Two-thirds of patients with PV develop mucous membrane blisters early in the course of their disease, and as the disease progresses, they develop skin blisters as well (3). Histologically, PV blisters occur deep in the epidermis between the basal and most immediate suprabasal keratinocytes, as well as between the basal cells themselves.…”

Localisation anale d’un pemphigus vulgaire. Une nouvelle observation

“…Keywords Pemphigus vulgaris · Anal involvement · Treatment · Listering disease Introduction Le pemphigus vulgaire (PV) est une pathologie auto-immune chronique qui atteint la peau et les muqueuses dans deux tiers des cas, une localisation muqueuse débutant souvent au niveau de la cavité orale [1][2][3]. La localisation anale reste très rare avec moins de 20 cas qui ont été rapportés dans la littérature [1].…”

Pemphigus Vulgaris: Update on Etiopathogenesis, Oral Manifestations, and Management

“…However, the prevalence of oral involvement varies: One recent multicenter study in several countries showed that Bulgarian patients less frequently had oral mucous membrane lesions (66%) compared with Italian (83%) and Israeli (92%) patients (Brenner et al, 2001). Initially vesiculobullous, the oral lesions readily rupture, new bullae developing as the older ones rupture and ulcerate (Sciubba, 1996), and thus erosions and ulcers are the main features and are seen primarily in the buccal mucosa, palate, and lips (Pisanty et al, 1974;Meurer et al, 1977;Zegarelli and Zegarelli, 1977;Orlowski et al, 1983;Shah and Bilimoria, 1983;Sklavounou and Laskaris, 1983;Lamey et al, 1992;Kanwar and Dhar, 1995;Weinberg and Abitbol, 1995;Scully et al, 1999;Davenport et al, 2001). Ulcers heal slowly, but scarring is rare (Zegarelli and Zegarelli, 1977;Shklar et al, 1978).…”