Shakeri Aria, Kindley Kimball Jade, Noland Mary Margaret, Gru Alejandro Ariel
University of Virginia School of Medicine, Charlottesville, VA.
Department of Dermatology, University of Virginia School of Medicine, Charlottesville, VA.
Am J Dermatopathol. 2019 Sep;41(9):675-679. doi: 10.1097/DAD.0000000000001401.
Immunoglobulin gamma (Ig) type 4-related disease (IgG4-RD) is a chronic immunologic systemic disorder that has gained worldwide recognition in the past decade. This entity can affect almost every organ system, and its characteristic lesions have been found in a variety of organs such as lacrimal and salivary glands, pancreas, liver, bile ducts, lungs, kidneys, retroperitoneum, breast, aorta, thyroid, and prostate. This case reports a very rare case of IgG4-RD presenting with a cutaneous lesion in a young female patient mimicking a lymphoproliferative disorder. IgG4-RD affecting the skin is a rare entity and has mostly been reported in Japanese men of middle to older age. IgG4-RD with cutaneous involvement should be in the differential of non-neoplastic, lymphomas, autoimmune, and infectious disorders of the skin.
免疫球蛋白γ（Ig）4相关疾病（IgG4-RD）是一种慢性免疫性全身性疾病，在过去十年中已获得全球认可。该疾病几乎可累及每个器官系统，其特征性病变已在多种器官中被发现，如泪腺和唾液腺、胰腺、肝脏、胆管、肺、肾脏、腹膜后、乳腺、主动脉、甲状腺和前列腺。本病例报告了一名年轻女性患者出现皮肤病变，酷似淋巴增殖性疾病，这是一例非常罕见的IgG4-RD病例。IgG4-RD累及皮肤是一种罕见情况，大多报道于中老年日本男性。皮肤受累的IgG4-RD应与皮肤的非肿瘤性、淋巴瘤、自身免疫性和感染性疾病相鉴别。
