Lilo Mohammed T, Yan Shaofeng, Chapman Michael S, Linos Konstantinos
Department of Pathology and Laboratory Medicine, Geisel School of Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH.
Section of Dermatology, Department of Surgery, Geisel School of Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH.
Am J Dermatopathol. 2019 Jul;41(7):511-513. doi: 10.1097/DAD.0000000000001380.
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.
疱疹样皮炎是一种罕见的慢性自身免疫性疾病，其特征为剧烈瘙痒的丘疹和水疱，可与乳糜泻及其他自身免疫性疾病相关。其组织学特征是乳头真皮层内有中性粒细胞聚集，在直接免疫荧光下可见免疫球蛋白A（IgA）呈颗粒状沉积。在此，我们报告一名58岁女性，其臀部出现水疱性皮疹。患者报告近期有生殖器疱疹、溶组织内阿米巴结肠炎、复发性荨麻疹和湿疹病史。一份代表性活检显示有海绵状皮炎和局灶性乳头真皮层中性粒细胞聚集的特征。直接免疫荧光显示乳头真皮层有纤维状IgA沉积，真皮-表皮交界处和真皮乳头有颗粒状C3沉积。总体临床、组织学和直接免疫荧光检查结果与疱疹样皮炎相符。纤维状IgA模式罕见，粗心者容易忽略。病理学家应知晓这种罕见模式，尤其是当组织学表现不典型时。
