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Invited Commentary | Practice Gaps

Underrecognition of the Heterogeneous Clinical Spectrum of Bullous Pemphigoid

Michael Hertl, MD1; Thomas Schmidt, PhD1
[+] Author Affiliations
1Departments of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany
JAMA Dermatol. 2013;149(8):954-955. doi:10.1001/jamadermatol.2013.4250.
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Bullous pemphigoid (BP) is commonly characterized clinically by the presence of tense blisters that develop on erythematous or urticarial pruritic skin. To establish the diagnosis of BP, the presence of tissue-bound IgG autoantibodies against components of the dermoepidermal basement membrane (by direct immunofluorescence of perilesional skin) and of serum IgG autoantibodies (by indirect immunofluorescence or enzyme-linked immunosorbent assay [ELISA]) is mandatory according to newly published guidelines.1 Recently, it has become apparent that the clinical spectrum of BP is rather heterogeneous.1,2 Several atypical, nonbullous clinical variants have been acknowledged that have urticarial or erythematous plaques, eczema-like or prurigo-like lesions with pruritic papules or nodules, generalized multiforme-like exanthems, and pruritus sine materia. A common feature of all clinical pemphigoid variants is a pronounced pruritus, which recently has been included as a diagnostic criterion of pemphigoid.3

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