Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With Rituximab

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Rituximab for patients with refractory mucous membrane pemphigoid.

BACKGROUND Mucous membrane pemphigoid (MMP) still represents a potentially life- and sight-threatening disease. In a subset of patients with severe MMP, conventional immunosuppressants are ineffective or contraindicated. OBSERVATIONS Twenty-five patients with severe refractory MMP, including 5 with mucous membrane-dominant epidermolysis bullosa acquisita, received 1 or 2 cycles of rituximab (...

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Unilateral Oral Mucous Membrane Pemphigoid: Refractory Atypical Presentation Successfully Treated with Intravenous Immunoglobulins

A 57-year-old male presented with a 6-month history of blisters and painful erosions on the right buccal mucosa. No skin or other mucosal involvement was seen. The findings of histopathological and direct immunofluorescence examinations were sufficient for the diagnosis of oral mucous membrane pemphigoid in the context of adequate clinical correlation. No response was seen after topical therapi...

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Mucous membrane pemphigoid with severe esophageal stricture.

Mucous membrane pemphigoid (MMP) is a rare nosological entity. MMP consists of a clinical phenotype in which several autoimmune subepidermal bullous diseases are classified. It occurs predominantly in the mucous membranes and usually results in scarring. Esophageal involvement in MMP is rare and is generally seen in patients in whom lesions are widespread. The most common alterations are multip...

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Paraneoplastic mucous membrane pemphigoid with ocular and laryngeal involvement

A 73-year-old woman was treated 8 years previously for synchronous breast and uterine neoplasms. She presented with a severe sore throat, odynophagia, dysphonia, dyspnoea, ocular irritation and weight loss over the last 3 months. Physical examination revealed ulcerations in the oral cavity, posterior pharyngeal wall and supraglottic larynx, nasal crusting, bilateral conjunctivitis and three cut...

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Refractory Sweet's syndrome successfully treated with rituximab

IL: interleukin PG: pyoderma gangrenosum RA: rheumatoid arthritis SS: Sweet’s syndrome TNF: tumor necrosis factor INTRODUCTION Sweet’s syndrome (SS) is a neutrophilic dermatosis characterized by an abrupt onset of painful erythematous cutaneous lesions that have a neutrophilic infiltrate typically located in the papillary dermis. Fever and an elevated neutrophil count can accompany the cutaneou...

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ژورنال

عنوان ژورنال: Frontiers in Medicine

سال: 2019

ISSN: 2296-858X

DOI: 10.3389/fmed.2019.00008