Pemphigoid nodularis - rare presentation of bullous pemphigoid: A case report and literature review

Vornicescu,Corina; Șenilă,Simona   Corina; Cosgarea,Rodica; Candrea,Elisabeta; Pop,Alexandra   Dana; Ungureanu,Loredana

doi:10.3892/etm.2018.7057

Pemphigoid nodularis - rare presentation of bullous pemphigoid: A case report and literature review

Authors:
- Corina Vornicescu
- Simona Corina Șenilă
- Rodica Cosgarea
- Elisabeta Candrea
- Alexandra Dana Pop
- Loredana Ungureanu
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Affiliations: Department of Dermatology, ‘Iuliu Hațieganu’ University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania, Department of Physiology, ‘Iuliu Hațieganu’ University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania
Published online on: December 5, 2018 https://doi.org/10.3892/etm.2018.7057
Pages: 1132-1138
Copyright: © Vornicescu et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

Pemphigoid nodularis (PN) is a rare clinical variant of bullous pemphigoid characterized by the presence of nodular prurigo-like lesions and pemphigoid blisters. The diagnosis is confirmed by direct immunofluorescence (DIF)/ indirect immunofluorescence (IIF) and immunoserology tests. For some patients, with long mean duration of symptoms, the correct diagnosis of PN is delayed because the disease is not recognized. We present a case and summarize the reported characteristics of PN. The search in MEDLINE database, after selection, resulted in 36 articles presenting 47 cases of PN. Between published cases a female predominance was noted (female to male ratio of 1.8:1), almost half of the reported patients were non-Caucasian, and the mean age at presentation was 66.2 years. The mean duration until the diagnosis was almost 2 years. Sixteen patients also had other autoimmune diseases. Twenty-two patients developed vesicles/bullae/urticarial plaques before or after the diagnosis. Peripheral eosinophilia and high levels of serum total IgE were reported in 10.6 and 27.2% of patients, respectively. ELISA for either BP180, BP230 or both were positive in all tested cases. DIF and IIF microscopy were positive overall in 100 and 92.3% of cases, respectively. Corticosteroids, either topical or systemic, were the most efficient therapeutic option, although many others were used. PN remains a diagnostic and therapeutic challenge in elderly patients with unexplained refractory chronic pruritus associated with papulo-nodular lesions.

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