Le Brun V, Boulinguez S, Bouyssou-Gauthier M L, Roux J, Labrousse F, Bonnetblanc J M, Bedane C
Service de Dermatologie, Hôpital Dupuytren, CHRU, 87042 Limoges Cedex, France.
Ann Dermatol Venereol. 2000 Feb;127(2):184-7.
We report a case of primary non-autoimmune hypothyroidism causing pretibial epidermolysis bullosa.
A 70-year-old man with primary non-autoimmune hypothyroidism developed blisters of different ages on the lateral aspect of both legs. Pathology reported blisters with subepidermal cleavage. Direct immunofluorescence was negative. Electron microscope examination showed a variable cleavage level and diffuse infiltration of a granulous and amorphous microfibrillar substance. After hormone replacement therapy, euthyroidism was associated with a reduction in the number of bullae and finally complete remission. After 12 months follow-up, the patient has not experienced recurrence.
Recurrence-free clinical improvement after hormone replacement therapy suggests the diagnosis of hypothyroidism pretibial epidermolysis bullosae. Mochizuki et al. described a similar case which rapidly regressed after hormone therapy but where the electron microscope showed a different cleavage level. These bullae appear to result from a mechanical mechanism due to their localization in areas exposed to friction and also to the presence of bullae of different ages. This hypothesis is confirmed by the presence of a variable level of cleavage and a substance dense to electrons at electron microscopy as well as by the skin weakness. Our case confirms the reality of hypothyroidism pretibial epidermolysis bullosa. Thyroid hormones should be assayed in patients presenting pretibial bullae.
我们报告一例原发性非自身免疫性甲状腺功能减退症导致胫前大疱性表皮松解症的病例。
一名70岁原发性非自身免疫性甲状腺功能减退症男性患者双侧小腿外侧出现不同时期的水疱。病理报告显示水疱位于表皮下。直接免疫荧光检查为阴性。电子显微镜检查显示分裂水平各异，并有颗粒状和无定形微原纤维物质的弥漫性浸润。激素替代治疗后，甲状腺功能正常与水疱数量减少相关，最终完全缓解。经过12个月的随访，患者未复发。
激素替代治疗后临床症状改善且无复发提示为甲状腺功能减退性胫前大疱性表皮松解症。Mochizuki等人描述了一个类似病例，该病例在激素治疗后迅速消退，但电子显微镜显示分裂水平不同。这些水疱似乎是由机械机制引起的，因为它们位于易受摩擦的部位，而且存在不同时期的水疱。电子显微镜下分裂水平各异以及存在电子致密物质，再加上皮肤脆弱，证实了这一假设。我们的病例证实了甲状腺功能减退性胫前大疱性表皮松解症的存在。对于出现胫前水疱的患者应检测甲状腺激素。
