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持久性豆状角化过度症(弗莱格尔病)的一种罕见的全身性形式。

An unusual generalized form of hyperkeratosis lenticularis perstans (Flegel's disease).

作者信息

Miljković Jovan

机构信息

Department of Dermatology, Maribor Teaching Hospital, Maribor, Slovenia.

出版信息

Wien Klin Wochenschr. 2004;116 Suppl 2:78-80.

Abstract

An 82-year-old female presented with an 11-year history of multiple, symmetrically disseminated hyperkeratotic red-brown papules 3-5 mm in diameter on the dorsal aspects of the upper and lower extremities. The eruption first appeared on the dorsa of the feet and on the lower parts of the legs. During the last few years, the lesions extended gradually to the upper part of the legs and thighs, also disseminating over the trunk. There was no family case history of Flegel's disease. Histopathological examination revealed a hyperkeratosis with a focal parakeratosis overlying the thinned epidermis. In the papillary dermis there was a dense band-like infiltrate composed of lymphocytes and some histiocytes. Electron microscopy showed a normal number of membrane-coated granules (Odland bodies). The clinical and histopathological findings in the patient corresponded with the diagnosis of hyperkeratosis lenticularis perstans. The patient was treated with PUVA (psoralen plus ultraviolet A)-bath therapy, retinoid plus PUVA and calcipotriol. However, all of these treatments were unsuccessful.

摘要

一名82岁女性,上肢和下肢背部出现直径3 - 5毫米的多发性、对称性分布的角化过度性红棕色丘疹,病程11年。皮疹最初出现在足部背侧和小腿下部。在过去几年中,病变逐渐扩展至大腿和小腿上部,也散布于躯干。家族中无匐行性穿通性弹力纤维病病例史。组织病理学检查显示角化过度,变薄的表皮上有灶性角化不全。乳头真皮层有由淋巴细胞和一些组织细胞组成的致密带状浸润。电子显微镜检查显示膜包颗粒(奥尔德兰小体)数量正常。该患者的临床和组织病理学表现符合匐行性持久性角化过度症的诊断。患者接受了补骨脂素加紫外线A(PUVA)浴疗法、维甲酸加PUVA和卡泊三醇治疗。然而,所有这些治疗均未成功。

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