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A Case of Dermatitis Herpetiformis With Fibrillar Immunoglobulin A Deposition

A Rare Pattern Not to Be Missed

Lilo, Mohammed T. MD*; Yan, Shaofeng MD, PhD*; Chapman, Michael S. MD; Linos, Konstantinos MD*

The American Journal of Dermatopathology: July 2019 - Volume 41 - Issue 7 - p 511–513
doi: 10.1097/DAD.0000000000001380
Extraordinary Case Report
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Abstract: Dermatitis herpetiformis is a rare, chronic autoimmune disorder characterized by intense pruritic papules and vesicles, which can be associated with celiac disease and other autoimmune disorders. Its histologic characteristic is the accumulation of neutrophils within the papillary dermis with granular deposition of immunoglobulin A (IgA) observed under direct immunofluorescence. Herein, we report a 58-year-old woman who presented with a vesicular rash on the buttocks. The patient reported a recent history of genital herpes, Entamoeba histolytica colitis, recurrent hives, and eczema. A representative biopsy demonstrated features of spongiotic dermatitis and focal papillary dermal neutrophilic aggregates. Direct immunofluorescence revealed fibrillary IgA deposition in the papillary dermis, granular C3 deposition at the dermal–epidermal junction, and dermal papillae. The overall clinical, histologic, and DIF findings were consistent with those of dermatitis herpetiformis. The fibrillar IgA pattern is rare and easily overlooked by the unwary. Pathologists should be aware of this rare pattern, especially when the histologic findings are not classic.

*Department of Pathology and Laboratory Medicine, Geisel School of Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH; and

Section of Dermatology, Department of Surgery, Geisel School of Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH.

Correspondence: Konstantinos Linos, MD, Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, 1 Medical Center Dr, Lebanon, NH 03756 (e-mail: Konstantinos.linos@hitchcock.org).

The authors declare no conflicts of interest.

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