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获得性反应性穿孔胶原病,一种罕见部位的罕见疾病。

Acquired reactive perforating collagenosis, a rare entity at uncommon site.

机构信息

Department of Dermatology, MGM Medical College, Kamothe, Navi Mumbai, Maharashtra, India.

出版信息

Indian J Pathol Microbiol. 2022 Oct-Dec;65(4):895-897. doi: 10.4103/ijpm.ijpm_333_21.

DOI:10.4103/ijpm.ijpm_333_21
PMID:36308201
Abstract

Acquired reactive perforating collagenosis (ARPC), rare disorder characterized by transepidermal elimination (TEE) of collagen fibers, is seen in adult diabetics. Genetic predisposition, familial aggregation, trauma, bites and scratching are implicated. Diabetics develop microvascular diseases leading to intense pruritus causing repeated micro trauma leading to necrosis of connective tissue of dermis, causing TEE. Isolated papules, plaques and nodules with central keratotic plugs, are mostly seen on extensor surfaces of limbs but trunk and face may be involved. Histopathology shows extrusion of abnormal collagen fibers through epidermis. Multiple treatment modalities show variable response. A 52 year old diabetic female had multiple, itchy, well defined, erythematous papules and plaques with central adherent crusting on lower back since 1 month. Histopathology showed cup shaped epidermal depression filled with plug of altered collagen, acanthotic epidermis with hyperkeratosis and parakeratosis. Underlying epidermis was thin with fine slits through which vertically oriented basophilic collagen fibers were extruded.

摘要

获得性反应性穿孔性胶原病 (ARPC) 是一种罕见的疾病,其特征为跨表皮胶原纤维消除 (TEE),见于成年糖尿病患者。遗传易感性、家族聚集、创伤、咬伤和搔抓均与之相关。糖尿病患者会发生微血管疾病,导致强烈瘙痒,从而反复造成微创伤,导致真皮结缔组织坏死,导致 TEE。孤立的丘疹、斑块和结节,中央有角化塞,大多见于四肢的伸展面,但也可能累及躯干和面部。组织病理学显示异常胶原纤维通过表皮挤出。多种治疗方法显示出不同的反应。一位 52 岁的糖尿病女性,1 个月前背部出现多个瘙痒、边界清楚的红斑性丘疹和斑块,中央有粘连性结痂。组织病理学显示杯状表皮凹陷,充满改变的胶原塞,棘层肥厚伴过度角化和角化不良。表皮下层薄,有细缝,垂直排列的嗜碱性胶原纤维从中挤出。

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