Generalized verrucosis in a patient with GATA2 deficiency. (May 2014)

• Record Type: Journal Article
• Authors: West, E.S.; Kingsbery, M.Y.; Mintz, E.M.; Hsu, A.P.; Holland, S.M.; Rady, P.L.; Tyring, S.K.; Grossman, M.E.
• Is Part Of: British journal of dermatology. Volume 170:Number 5(2014:May)
• Page Start: 1182
• Page End: 1186
• Publication Date: 2014-05
• Subjects: Dermatology -- Periodicals; Skin -- Diseases -- Periodicals; 616.5
• DOI: 10.1111/bjd.12794
• Languages: English
• ISSNs: 0007-0963

Abstract

<abstract abstract-type="main" id="bjd12794-abs-0001"> <title>Summary</title> <p>Generalized verrucosis is a characteristic of several genetic and immunodeficiency disorders including epidermodysplasia verruciformis; warts, hypogammaglobulinaemia, infections and myelokathexis (WHIM) syndrome; warts, immunodeficiency, lymphoedema and anogenital dysplasia (WILD) syndrome; severe combined immune deficiency and HIV, among others. In recent years, it has been consistently recognized in patients with <italic>GATA2</italic> deficiency, a novel immunodeficiency syndrome characterized by monocytopenia, B‐cell and natural killer‐cell lymphopenia, and a tendency to develop myeloid leukaemias and disseminated mycobacterial, human papillomavirus (HPV) and opportunistic fungal infections. Mutations in <italic>GATA2</italic> cause haploinsufficiency and track in families as an autosomal dominant immunodeficiency. GATA2 is a transcription factor involved in early haematopoietic differentiation and lymphatic and vascular development. We describe a case of generalized verrucosis with HPV type 57 presenting in a young man with <italic>GATA2</italic> deficiency. <italic>GATA2</italic> deficiency is a novel dominant immunodeficiency that is often recognized later in life and should be considered in the differential diagnosis of patients with generalized verrucosis.</p> </abstract>