Paraneoplastic Pemphigus: Recent Advances
نویسنده
چکیده
These confirmatory reports have verified all the essential features of the disease that was first described. Mucosal ulceration in the form of intractable stomatitis is the most constant clinical feature of the disease. In no case to-date has this been absent. Cutaneous manifestations including blisters, lichenoid eruption or erythema multiforme like lesions are highly variable. These can change in an individual patient according to the stage of the disease. Histopathology also varies in line with the clinical picture: suprabasal acantholysis in blistering areas, vacuolar interface changes and individual keratinocyte necrosis in non-blistering areas.
منابع مشابه
Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy
Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several di...
متن کاملParaneoplastic pemphigus regression after thymoma resection
BACKGROUND Among human neoplasms thymomas are associated with highest frequency with paraneoplastic autoimmune diseases. CASE PRESENTATION A case of a 42-year-old woman with paraneoplastic pemphigus as the first manifestation of thymoma is reported. Transsternal complete thymoma resection achieved pemphigus regression. The clinical correlations between pemphigus and thymoma are presented. C...
متن کاملNon-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus*
The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. Th...
متن کاملParaneoplastic pemphigus associated with Castleman tumor: a commonly reported subtype of paraneoplastic pemphigus in China.
BACKGROUND Castleman tumor, a rare lymphoproliferative disorder, is one of the associated tumors in paraneoplastic pemphigus. We analyzed the characteristics of a group of patients with Castleman tumor to clearly understand and to improve the prognosis of the disease. OBSERVATIONS Ten cases of paraneoplastic pemphigus associated with Castleman tumor treated in the Department of Dermatology, P...
متن کاملAtypical laboratory presentation of paraneoplastic pemphigus associated with Castleman disease
ELISA: enzyme-linked immunosorbent assay IIF: indirect Immunofluorescence PNP: paraneoplastic pemphigus PV: pemphigus vulgaris INTRODUCTION Because of variability in clinical and histopathologic findings, the diagnosis of paraneoplastic pemphigus (PNP) frequently relies on the combination of direct immunofluorescence and indirect immunofluorescence (IIF). Here we present a unique case of PNP as...
متن کامل