无黏蛋白沉积的肿瘤性及向毛发性蕈样肉芽肿:向脓疱型红皮病进展(法语)

无黏蛋白沉积的肿瘤性及向毛发性蕈样肉芽肿：向脓疱型红皮
病进展(法语)
Merlet S.;Dandurand M.;Elmajaoui S.;L.Meunier;潘敏
【期刊名称】《世界核心医学期刊文摘：皮肤病学分册》
【年(卷),期】2006(0)10
【摘要】Introduction.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.Observation.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 Tcell 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.Discussion.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.
【总页数】1页(P26-26)
【关键词】蕈样肉芽肿;脓疱型;红皮病;黏蛋白;肿瘤性;肿瘤期;基因重排;组织学检查;首例报道;组织病理学
【作者】Merlet S.;Dandurand M.;Elmajaoui S.;L.Meunier;潘敏
【作者单位】Service de Dermatologie,Groupe Hospitalo-Universitaire
Carémeau,Pl.du Professeur Robert Debre,30029 Nmes
【正文语种】中文
【中图分类】R739.5
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