(Last Updated On: October 30, 2018)

Authors: Lauren Camille B. Livelo, MD, Elisabeth Ryan, MD, Ricky H. Hipolito, MD, DPDS, Johannes F. Dayrit, MD,FPDS

Abstract

Introduction: Dermatitis Herpetiformis (DH) is a chronic, relapsing cutaneous disease with associated gluten sensitive enteropathy (GSE). DH is characterized by an symmetric, intensely pruritic papulovesicular herpetiform eruption involving extensor surfaces of the body. It is believed to be rare among Asians and AfricanAmericans. Diagnosis of DH relies on clinical presentation, histology, direct immunofluorescence, and serology. More rarely, seronegative DH occurs in 30% of patients with DH. Anti IgA anti-tissue transglutaminase and IgA antiendomysial antibodies are rarely expressed among the Japanese and may probably represent a variant of
DH different from Europeans and other Caucasian races.

Case Presentation: A 33 year-old Filipino female consulted because of a 3-week history of severely pruritic vesicles and crusts on the face, trunk and arms. Patient noted no gastrointestinal symptoms on consultation. Skin punch revealed subepidermal blisters with collection of neutrophils at the dermal papillae. Direct immunofluorescence showed strong (+2) granular deposits of IgA at the suprapapillary tips of the epidermis in perilesional skin thus validating the diagnosis. The patient’s serum was negative for IgA anti-tissue transglutaminase and IgA antiendomysial antibodies. Patient was treated with topical corticosteroids and Dapsone 50 mgs OD with remarkable improvement.

Conclusion: The unique features of “Japanese DH” are the ff: (i) high frequency of deposition of fibrillar collagen in the papillary dermis; (ii) the rare occurrence of gluten-sensitive enteropathy; (iii) absence of HLA DQ2 and HLA DQ8; and (iv) rare association of autoimmune disease or lymphomas. This case of seronegative DH in a Filipino may represent a variant similar to the Japanese with no anti-transgutaminase antibodies and no specific HLA genes despite IgA deposits in the dermis.

 

Citation

 

Keywords

Dermatitis Herpetiformis, autoimmune bullous diseases, anti-tissue transglutaminase, antiendomysial antibody, Japanese, Dapsone

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