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Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid

Author
Kristin M Leiferman, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD

INTRODUCTION

Bullous pemphigoid and mucous membrane pemphigoid (MMP) are autoimmune blistering diseases that most commonly arise in older adults. These disorders are characterized by subepithelial blister formation and the deposition of immunoglobulins and complement within the epidermal and/or mucosal basement membrane zone.

Although both bullous pemphigoid and MMP may affect skin and mucosa, the classical clinical findings in bullous pemphigoid are tense, fluid-filled bullae on skin whereas the prevailing clinical feature in MMP is mucosal involvement. In MMP, inflamed and eroded mucosa is characteristic, involving any or all of the oral cavity, ocular conjunctiva, nose, pharynx, larynx, esophagus, anus, and genital mucous membranes.

The clinical features and diagnosis of bullous pemphigoid and MMP will be reviewed here. The epidemiology, pathogenesis, and treatment of these disorders as well as greater detail on the ocular form of MMP (ocular cicatricial pemphigoid) and other autoimmune blistering disorders are discussed separately. (See "Epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid" and "Management and prognosis of bullous pemphigoid" and "Management of mucous membrane pemphigoid" and "Ocular cicatricial pemphigoid" and "Dermatoses of pregnancy", section on 'Pemphigoid gestationis' and "Pathogenesis, clinical manifestations, and diagnosis of pemphigus".)

CLINICAL FEATURES

Bullous pemphigoid — A prodromal phase lasting weeks to months may precede the development of cutaneous bullae in patients with bullous pemphigoid [1,2]. The prodromal phase may present with pruritic eczematous, papular, or urticaria-like skin lesions (picture 1) [3]. Some patients with bullous pemphigoid never develop blistering.

When blisters develop, the classical lesion is a 1 to 3 cm tense bulla on an erythematous, urticarial, or noninflammatory base, and blisters may be numerous and widespread (picture 2A-C) [4]. Associated pruritus is common and can be severe [5,6]. The tense quality of the bullae differentiates the lesions of bullous pemphigoid from the flaccid bullae of pemphigus vulgaris (picture 3A) (see "Pathogenesis, clinical manifestations, and diagnosis of pemphigus") [2,7]. Eventually, the bullae rupture, leaving moist erosions and crusts that resolve without scarring. When mainly erosions are present, it may be difficult to clinically distinguish bullous pemphigoid and pemphigus (picture 3B).

                           

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Literature review current through: Nov 2016. | This topic last updated: Wed Oct 19 00:00:00 GMT 2016.
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