Bakar Ozgür, Seçkin Dilek, Demirkesen Cuyan, Baykal Can, Büyükbabani Nesimi
Department of Dermatology, School of Medicine, Acibadem University, Istanbul, Turkey.
Department of Dermatology, School of Medicine, Marmara University, Istanbul, Turkey.
Ann Dermatol. 2014 Jun;26(3):385-91. doi: 10.5021/ad.2014.26.3.385. Epub 2014 Jun 12.
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, and it rarely exhibits predilection for hair follicle and eccrine gland infiltration. Here, we present 2 similar cases that display folliculotropism with varying amounts of follicular mucinosis, with and without syringotropism. The features observed in both cases were cystic, comedo-like, acneiform lesions; generalized involvement with loss of body hair; pruritus; and hidradenitis suppurativa-like lesions. Hypohidrosis as well as nail and palmoplantar involvement with lichen planopilaris-like clinical features were unique characteristics of the first case. Despite the well-known aggressive behavior of follicular mycosis fungoides, the presented cases had a subtle, slowly progressive, but persistent, clinical course. Folliculotropic and syringotropic mycosis fungoides are variants of cutaneous T-cell lymphoma. Clinical presentations might be challenging, and multiple, deep biopsy specimens containing adnexal structures are required for this critical diagnosis. Aggressive treatment may not be necessary in cases having an indolent course, especially in those with syringotropism.
蕈样肉芽肿是皮肤T细胞淋巴瘤最常见的形式，很少表现出对毛囊和汗腺浸润的偏好。在此，我们展示2例相似病例，其显示不同程度毛囊黏蛋白沉积的向毛囊性，伴或不伴有向汗腺性。两例中观察到的特征为囊性、粉刺样、痤疮样损害；全身性受累伴体毛脱落；瘙痒；以及化脓性汗腺炎样损害。少汗以及伴有扁平苔藓样临床特征的甲和掌跖受累是第一例的独特特征。尽管毛囊性蕈样肉芽肿具有众所周知的侵袭性行为，但所展示病例具有隐匿、缓慢进展但持续存在的临床病程。向毛囊性和向汗腺性蕈样肉芽肿是皮肤T细胞淋巴瘤的变异型。临床表现可能具有挑战性，为此关键诊断需要多个包含附属器结构的深部活检标本。对于病程惰性的病例，尤其是伴有向汗腺性的病例，可能无需积极治疗。
