Linear IgA bullous dermatosis in the setting of angioimmunoblastic T‐cell lymphoma

Abstract

We report an 80‐year‐old male developing linear IgA bullous dermatosis (LAD) in the setting of angioimmunoblastic T‐cell lymphoma (AITL). This phenomenon is rare, as only three cases have been described in the literature. The pathophysiologic process can be attributed to dysregulation in somatic hypermutation and the expression of chemokine receptor 5 in AITL, contributing to increased IgA. Immunoglobulin production resulting from clonal plasma cell expansion may be because of the B‐cell promotional effect by neoplastic follicular helper T‐cells. Beyond providing a pathophysiologic platform for AITL‐associated LAD, we also briefly summarized prior cases. This report demonstrates the importance of considering LAD in the differential diagnosis for patients with a bullous eruption in the setting of AITL.

Linear IgA Bullous Dermatosis in Korea Using the Nationwide Health Insurance Database

“…Among malignancies, the association between hematologic malignancies and LABD is more established than that with solid organ tumors. Andriano et al reported a case of LABD in the setting of angioimmunoblastic T-cell lymphoma (AITL) and explained the pathophysiological process of dysregulation of somatic hypermutation and expression of chemokine receptor 5 in AITL, which leads to increased IgA [33]. Colmant et al reported that a case of LABD associated with cutaneous involvement of an angioimmunoblastic T-cell lymphoma showed deep cutaneous involvement of the lymphoma with sub-epidermal blistering and direct immunofluorescence of heavy IgA linear deposits at the dermal-epidermal junction [34].…”