1. Autoimmune bullous diseases with acantholysis : p. vulgaris, p. foliaceus, p. erythematosus, or p. vegetans. 2. A nonspecific term for blistering skin diseases. [G. pemphix, a blister]
- benign familial chronic p. [MIM*169600] recurrent eruption of vesicles and bullae that become scaling and crusted lesions with vesicular borders, predominantly of the neck, groin, and axillary regions; autosomal dominant inheritance, presenting in late adolescence or early adult life. SYN: Hailey-Hailey disease.
- Brazilian p. SYN: fogo selvagem.
- p. erythematosus an eruption involving sun-exposed skin, especially the face; the lesions are scaling erythematous macules and blebs, combining the clinical features of both lupus erythematosus and p. vulgaris; bullae are subcorneal; probably a variant of p. foliaceus, occasionally penicillamine-induced. SYN: Senear-Usher disease, Senear-Usher syndrome.
- p. foliaceus a generally chronic form of p., rarely affecting mucosal surfaces, in which extensive exfoliative dermatitis, with no perceptible blistering, may be present in addition to the bullae; serum autoantibodies induce bullae and crusted acantholytic superficial epidermal lesions.
- paraneoplastic p. painful mucosal erosions and polymorphous skin eruptions with biopsy findings resembling p. vulgaris, associated with neoplasm and serum antibodies reactive with intercellular substance of all epithelia; usually rapidly fatal.
- p. vegetans 1. a rare, verrucous form of p. vulgaris in which vegetations develop on the eroded surfaces left by ruptured bullae; new bullae continue to form; SYN: Neumann disease. 2. a chronic benign vegetating form of p., with lesions commonly in the axillae and perineum; spontaneous remissions and occasionally permanent healing to occur. SYN: Hallopeau disease.
- p. vulgaris a serious form of p., occurring in middle age, in which cutaneous flaccid acantholytic suprabasal bullae and oral mucosal erosions may be localized a few months before becoming generalized; blisters break easily and are slow to heal; results from the action of autoimmune antibodies that localize to intercellular sites of stratified squamous epithelium.
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pem·phi·gus 'pem(p)-fi-gəs, pem-'fī-gəs n, pl -gus·es or -gi -.jī any of several autoimmune diseases characterized by the formation of successive eruptions of large blisters on apparently normal skin and mucous membranes often in association with sensations of itching or burning
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n.
a rare but serious autoimmune disease marked by successive outbreaks of blisters. The blisters are superficial and do not remain intact for long; the mouth and other mucous membranes, as well as the skin, are usually affected. A number of milder variants of the disease exist.
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pem·phi·gus (pemґfĭ-gəs) [Gr. pemphix blister] 1. any in a group of chronic, relapsing, sometimes fatal autoimmune diseases of the skin, characterized clinically by successive crops of vesicles and bullae, histologically by acantholysis, and immunologically by serum autoantibodies against antigens (see desmogleins) in the intracellular zones of the epidermis. Cf. pemphigoid. 2. p. vulgaris.Medical dictionary. 2011.