Martin J C, Lacombe D, Lefebvre D, Bonafé J L, Taïeb A, Maleville J
Unité de Dermatologie Pédiatrique, Hôpital Pellegrin-Enfants, Bordeaux.
Ann Dermatol Venereol. 1994;121(4):309-14.
Erythromelalgia is an acrosyndrome characterized by paroxysmal manifestations associating erythema, local sensations of warmth and pain which improve with exposure to cold. Childhood forms, exceptional and usually primary, are quite severe and particularly resistant to treatment. The search for a causal agent is most often negative and the pathogenesis remains to be determined. We report a case of erythromelalgia observed in a 4-year-old girl, her father and her younger sister. This case was particular due to an association with mercury poisoning. The symptomatology was improved after different therapeutic attempts including Clomipramide and, particularly effective, rerigerating socks (D(r) Comet, CNES, Toulouse). In the literature we were unable to find any case of erythromelalgia related to mercury poisoning. The cases of familial erythromelalgia reported suggest X-linked dominant transmission. Finally, this case demonstrated the difficulties in diagnosing and treating erythromelalgia, especially in the child.
红斑性肢痛症是一种肢端综合征，其特征为阵发性发作，伴有红斑、局部温热感和疼痛，遇冷症状改善。儿童型红斑性肢痛症较为罕见，通常为原发性，病情相当严重且对治疗特别抗拒。寻找病因的结果大多为阴性，发病机制仍有待确定。我们报告一例在一名4岁女孩、其父亲和妹妹身上观察到的红斑性肢痛症病例。该病例较为特殊，因为它与汞中毒有关。经过包括氯米帕明在内的不同治疗尝试后症状有所改善，尤其是使用制冷袜（D(r) Comet，法国国家空间研究中心，图卢兹）效果显著。在文献中，我们未能找到任何与汞中毒相关的红斑性肢痛症病例。所报道的家族性红斑性肢痛症病例提示X连锁显性遗传。最后，该病例表明了红斑性肢痛症诊断和治疗的困难，尤其是在儿童患者中。
