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Tristan Manalac, 25 Sep 2019
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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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Most Read Articles
Tristan Manalac, 25 Sep 2019
Aqueous cream moisturizers that do not contain sodium lauryl sulphate (SLS) are less irritating to the skin than SLS-containing ones, while achieving similar levels of hydration and skin barrier integrity, according to a recent Singapore study.
Tristan Manalac, 16 Jul 2019
Caffeinated drinks, such as coffee and black tea, may protect against nonmelanoma skin cancer (NMSC) among Chinese in Singapore, according to a recent study.
24 Sep 2019
Generalized and localized dry skin is prevalent among middle-aged and elderly adults, a recent study has found.
5 days ago
New drug applications approved by US FDA as of 01 - 15 October 2019 which includes New Molecular Entities (NMEs) and new biologics. It does not include Tentative Approvals. Supplemental approvals may have occurred since the original approval date.