Linear IgA bullous dermatosis in the setting of angioimmunoblastic T‐cell lymphoma. (12th September 2022)

• Record Type: Journal Article
• Authors: Andriano, Tyler M.; Tannenbaum, Rachel; Xu, Haoming; Magro, Cynthia M.
• Is Part Of: Journal of cutaneous pathology. Volume 50:Number 1(2023)
• Page Start: 43
• Page End: 46
• Publication Date: 2022-09-12
• Subjects: angioimmunoblastic T‐cell lymphoma -- autoimmune vesiculobullous disorder -- linear IgA bullous dermatosis -- paraneoplastic; Skin -- Diseases -- Periodicals; Dermatology -- Periodicals; 616
• DOI: 10.1111/cup.14309
• Languages: English
• ISSNs: 0303-6987

Abstract

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.