Motta Letícia Marra da, Soares Cleverson Teixeira, Nakandakari Sadamitsu, Silva Gardênia Viana da, Nigro Maria Helena Mazzi Freire, Brandão Leticia Stella Gardini
General Outpatient Dermatology Clinic at Instituto Lauro de Souza Lima (ILSL) - Bauru (SP), Brazil.
Pathology Service at Instituto Lauro de Souza Lima (ILSL) - Bauru (SP), Brazil.
An Bras Dermatol. 2017 Sep-Oct;92(5):694-697. doi: 10.1590/abd1806-4841.20175099.
We report a case of granulomatous slack skin, a rare and indolent subtype of mycosis fungoides. It affects mainly men between the third and fourth decades. It is characterized by hardened and erithematous plaques that mainly affect flexural areas and become pedunculated after some years. Histological examination shows a dense infiltrate of small atypical lymphocytes involving the dermis (and sometimes the subcutaneous tissue) associated with histiocytic and multinucleated giant cells containing lymphocytes and elastic fibers (lymphophagocytosis and elastophagocytosis, respectively). Patients affected by this entity can develop secondary lymphomas. There are several but little effective therapeutic modalities described. Despite the indolent behavior of granulomatous slack skin, its early recognition and continuous monitoring by a dermatologist becomes essential for its management and prevention of an unfavorable outcome.
我们报告一例肉芽肿性皮肤松弛症，这是蕈样肉芽肿的一种罕见且进展缓慢的亚型。它主要影响30至40岁之间的男性。其特征为硬化性红斑斑块，主要累及屈侧部位，数年后会形成带蒂肿物。组织学检查显示真皮（有时也累及皮下组织）有密集的小非典型淋巴细胞浸润，伴有含有淋巴细胞和弹性纤维的组织细胞及多核巨细胞（分别为噬淋巴细胞现象和噬弹性纤维现象）。患有这种疾病的患者可能会发展为继发性淋巴瘤。目前有多种治疗方式，但效果甚微。尽管肉芽肿性皮肤松弛症进展缓慢，但皮肤科医生对其早期识别和持续监测对于其管理及预防不良后果至关重要。
