Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038362 (stomatitis)
 8,852 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pemphigus vegetans is a rare variant of pemphigus which is mainly localized in the intertriginous areas (Neumann type, Hallopeau type) or primarily involves the mucous membranes (pyostomatitis vegetans). A 18-year-old patient with erosive stomatitis developed a vegetating plaque with papillomatous and verruciform features in her left axilla. Histopathological examination of the axilla revealed papillomatosis and acanthosis as well as suprabasal clefting with acantholytic cells. By direct immunofluorescent examination, deposits of immunoglobulin IgG and complement (C3) were found in the intercellular space of the epidermis. Serological examination by indirect immunofluorescent techniques was indicative of pemphigus autoantibodies at a titer of 1:40. This case report demonstrates that the classical differentiation of pemphigus vegetans in two types, Neumann type and Hallopeau type, is mainly of historical importance, because both entities may represent variants of the same disease. In addition, other disorders such as IgA pemphigus may also present with vegetating plaques in intertriginous sites.
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PMID:[Pemphigus vegetans. A historical perspective]. 1142 79

Autoimmune bullous skin diseases are characterized by autoantibodies against adhesion molecules of the skin. Pemphigus is a disorder with an intraepidermal loss of adhesion and is characterized by fragile blisters and erosions. Pemphigus vulgaris often shows extensive lesions of the oral mucosa, while pemphigus foliaceus is commonly restricted to cutaneous involvement with puff pastry-like scale formation. Paraneoplastic pemphigus is obligatorily associated with malignancies and often presents as hemorrhagic stomatitis with multiforme-like exanthems. IgA pemphigus typically presents with pustules and annular plaques but not with mucosal involvement. The clinical spectrum of the pemphigoids includes tense blisters, urticarial plaques, and prurigo- like eczematous lesions. Pemphigoid gestationis mostly occurs during the last trimester of pregnancy and mucous membrane pemphigoid primarily involves the oral mucosa and conjunctivae and leads to scarring. Linear IgA bullous dermatosis manifests with tense blisters in a "cluster of jewels"-like pattern in childhood and is more heterogeneous in adulthood. Classical epidermolysis bullosa acquisita shows extensive skin fragility. Dermatitis herpetiformis is associated with gluten-sensitive enteropathy and manifests clinically with severe itching and papulovesicles on the extensor surfaces of the extremities and the lumbosacral area. The intention of the review is to demonstrate the heterogeneous clinical spectrum of autoimmune bullous disorders.
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PMID:Autoimmune bullous skin diseases. Part 1: Clinical manifestations. 2195 78