Spieth Konstanze, Grundmann-Kollmann Marcella, Runne Ulf, Staib Gyde, Fellbaum Christian, Wolter Manfred, Kaufmann Roland, Gille Jens
Zentrum der Dermatologie, Klinikum der J W Goethe-Universität, Frankfurt am Main, Deutschland.
Dermatology. 2002;205(3):239-44. doi: 10.1159/000065862.
The etiopathology of chronic eczematous lesions of the palms and/or soles remains elusive in a considerable proportion of patients. Accumulating evidence suggests that a rare variant of mycosis fungoides (MF)-type cutaneous T cell lymphoma (CTCL) restricted to the palms and/or soles may mimic common palmoplantar dermatoses.
In the present study, we analyzed the clinical and histological characteristics of 3 adult patients with preexisting nonclassified chronic palmoplantar eczema poorly responding to standard therapies. Palmar and/or plantar MF was eventually diagnosed.
The course of the disease, response to previous therapies and dermatological features are described, results of histochemical and immunohistochemical analyses are reported, including T cell receptor gamma gene rearrangement where obtainable.
Onset of cutaneous lesions with broad clinical variation was experienced 2-10 years prior to diagnosis; conventional therapies led to short-time or partial remission only; except for 1 patient, the epidermotropic infiltrate was predominantly composed of CD4-positive cells; topical photochemotherapy seems to result in more durable responses.
As therapeutic strategies for this disease variant differ from symptomatic standard treatment regimens, awareness of MF-type CTCL as a relevant differential diagnosis of palmoplantar eczema should be expanded to prevent delay in diagnosis and adequate therapy.
相当一部分患者手掌和/或足底慢性湿疹样皮损的病因病理仍不清楚。越来越多的证据表明,一种罕见的蕈样肉芽肿(MF)型皮肤T细胞淋巴瘤(CTCL)局限于手掌和/或足底,可能会模仿常见的掌跖部皮肤病。
在本研究中,我们分析了3例成年患者的临床和组织学特征,这些患者先前患有未分类的慢性掌跖部湿疹,对标准治疗反应不佳,最终被诊断为掌跖部MF。
描述疾病病程、对先前治疗的反应和皮肤病学特征,报告组织化学和免疫组织化学分析结果,包括可获得的T细胞受体γ基因重排结果。
皮肤病变在诊断前2 - 10年出现,临床变化广泛;传统治疗仅导致短期或部分缓解;除1例患者外,亲表皮浸润主要由CD4阳性细胞组成;局部光化学疗法似乎能产生更持久的反应。
由于这种疾病变体的治疗策略与对症标准治疗方案不同,应提高对MF型CTCL作为掌跖部湿疹相关鉴别诊断的认识,以防止诊断和适当治疗的延误。