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Antienvoplakin antibodies, BO linked to death in HM-related paraneoplastic pemphigus

28 May 2019

There is a high rate of mortality among patients with haematologic malignancy (HM)-related paraneoplastic pemphigus (PNP), suggests a new study. The independent survival factors in HM-related PNP include toxic epidermal necrolysis–like and lung bronchiolitis obliterans (BO)-associated forms.

In 144 patients included in this systematic review, majority of the HMs were non-Hodgkin lymphoma (52.78 percent), followed by chronic lymphocytic leukaemia (22.92 percent), Castleman disease (18.60 percent) and other underlying haematologic malignancy (5.70 percent).

There was a 57-percent mortality rate, with most deaths occurring within the first year following the diagnosis of PNP. In multivariate analysis, the presence of antienvoplakin antibodies and BO significantly correlated with death, while a toxic epidermal necrolysis–like clinical pattern, bullous pemphigoid–like clinical pattern and BO were significantly associated with decreased survival.

In this study, the authors conducted a systematic review of previously described cases of PNP associated with HMs to determine the precise clinical and biologic features of HM-related PNP, as well as identify factors associated with mortality and survival. This was limited by including only case reports with sufficient mortality data.

PNP occurs more often in patients with HMs than in those with solid cancer, and lung BO is a severe complication of PNP, according to the authors.

A study by Yong and Tey described PNP as a distinct autoimmune blistering disease that affects several organs other than the skin. Its clinical presentation consists of painful, severe oral erosions which is sometime accompanied by a generalized cutaneous eruption and systemic involvement. [Australas J Dermatol 2013;54:241-250]

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