Oesophageal involvement in bullous pemphigoid

Departments of Gastroenterology and Hepatology Dermatology, Sackler Faculty of Medicine, Tel-Aviv Sourasky Medical Center, Tel-Aviv University, Tel Aviv, Israel.
Clinical and Experimental Dermatology (Impact Factor: 1.09). 04/2013; 38(3):274-5. DOI: 10.1111/j.1365-2230.2012.04408.x
Source: PubMed


Bullous pemphigoid (BP) is an acquired autoimmune disease that predominantly affects older people. Mucosal involvement is rare in BP. We report an unusual case of an elderly patient with BP with involvement of the oesophagus presenting as gastrointestinal (GI) bleeding. Although mucosal involvement is typically rare in BP, it should be considered in the differential diagnosis of GI bleeding in patients affected with the disease.

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    ABSTRACT: Objective. Bullous pemphigoid is well known for its cutaneous features; however in rare cases it may present with mucosal involvement. We report a case of bullous pemphigoid presenting with haemoptysis, initially presenting to the Ear, Nose and Throat Department for investigation. Methods. An 87-year-old lady was admitted with haemoptysis. She also complained of a spreading, pruritic, bullous rash, which first began three weeks previously. Initial investigations, which included nasendoscopy, revealed a normal nasal mucosa and a normal postnasal space. A large deroofed blister was observed on the soft palate. The presenting symptoms and signs raised the suspicion of an immunobullous disease including bullous pemphigoid. Conclusion. Bullous pemphigoid (BP) is a subepidermal immunobullous disease that typically manifests in elderly patient populations. Although rare, BP can present in a mucocutaneous fashion akin to its more aggressive variant, mucous membrane pemphigoid (MMP). Differentiation of the two is based on clinical grounds, with the prevailing feature for the latter being the predominance of mucosal involvement, which may be extensive. The mainstay of treatment for bullous pemphigoid is steroid therapy, which may be administered both topically and systemically. A deeper understanding into the pathophysiology of the various immunobullous diseases may assist in our understanding of how the various disease entities manifest themselves.
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    ABSTRACT: Context: Inflammatory lesions of the esophagus are a diverse group, often with nonspecific histologic findings. These benign changes can produce diagnostic difficulties for pathologists. Objective: To discuss the typical histologic findings of a variety of the most common causes of esophagitis (reflux, eosinophilic esophagitis, infections, medications) along with less common issues such as sloughing esophagitis and skin disorders involving the squamous mucosa. Data sources: The literature has been reviewed to discuss histologic definitions of current and developing issues in the area of esophagitis. Conclusions: Histologic features are not entirely sensitive and specific for inflammatory esophageal disorders. Awareness of these problems is essential; clinical and endoscopic information can be very useful in distinguishing among the various lesions.
    No preview · Article · Jun 2015 · Archives of pathology & laboratory medicine