Merlet S, Dandurand M, Elmajaoui S, Joujoux J-M, Ligeron C, Michel B, Stoebner P-E, Meunier L
Service de Dermatologie, Groupe Hospitalo-Universitaire Carémeau, Nîmes.
Ann Dermatol Venereol. 2006 Feb;133(2):149-52. doi: 10.1016/s0151-9638(06)70866-3.
Pilotropic mycosis fongoides is a particular form of the disease, because of its clinical and histological aspects, its poor prognosis and its resistance to treatment. We report a case of pilotropic mycosis fongoides without mucinosis, immediately tumoral, the fatal progression of which was marked by the occurrence of pustular erythroderma.
In 1998 a 69 year-old man presented with infiltrated erythro-squamatous plaques and nodules on the limbs associated with follicular lesions predominating on the cervical-cephalic area. Histological explorations revealed a pilotropic infiltrate with atypical CD4+ CD8 CD30 T-cells, without epidermotrophism or mucinosis. Study of genetic rearrangements found a clone lymphocyte T-cell in the skin. Diagnosis of pilotropic mycosis fongoides at the tumoral stage was made and, despite various treatments, the disease developed towards fatal pustular erythroderma.
At the onset of its progression, pilotropic mycosis fongoides is sometimes difficult to distinguish from classical mycosis fongoides, during which follicular involvement is often seen. However it is important to differentiate these entities because of the poor prognosis of pilotropic mycosis fongoides. Development of tissue micro dissection techniques and lymphocyte T-cell clones from human skin would help to separate these cutaneous T-cell lymphoma sub-groups. Our case report is original because of the absence of dermal mucinosis combined with an immediately tumoral form and the progression towards generalized pustulosis. It also underlines the poor prognosis and resistance to treatment of pilotropic mycosis fongoides.
亲毛囊性蕈样霉菌病是该疾病的一种特殊形式，因其临床和组织学表现、预后不良以及对治疗的抵抗性。我们报告一例无黏蛋白沉积的亲毛囊性蕈样霉菌病病例，该病例呈即刻肿瘤性，其致命进展以脓疱性红皮病的出现为特征。
1998年，一名69岁男性患者四肢出现浸润性红斑鳞屑性斑块和结节，伴有以头颈区域为主的毛囊性损害。组织学检查显示为亲毛囊浸润，伴有非典型CD4 + CD8 CD30 T细胞，无表皮趋向性或黏蛋白沉积。基因重排研究在皮肤中发现了一个克隆性淋巴细胞T细胞。诊断为肿瘤期亲毛囊性蕈样霉菌病，尽管进行了各种治疗，疾病仍发展为致命的脓疱性红皮病。
在亲毛囊性蕈样霉菌病进展初期，有时难以与经典蕈样霉菌病区分开来，后者常可见毛囊受累。然而，由于亲毛囊性蕈样霉菌病预后不良，区分这些实体很重要。组织显微切割技术和从人皮肤中分离淋巴细胞T细胞克隆的发展将有助于区分这些皮肤T细胞淋巴瘤亚组。我们的病例报告具有独特性，因为不存在真皮黏蛋白沉积，伴有即刻肿瘤性形式，并进展为全身性脓疱病。它还强调了亲毛囊性蕈样霉菌病预后不良和对治疗的抵抗性。
