Flageul B, Hassan F, Pinquier L, Blanchet-Bardon C, Dubertret L
Service de Dermatologie, Hôpital Saint-Louis, Paris.
Ann Dermatol Venereol. 1999 Aug-Sep;126(8-9):604-7.
Lichen planus pemphigoides is a rare acquired auto-immune bullous dermatosis which usually affects adults. Only four cases have been reported in children. We describe a new case of lichen planus pemphigoides in a child unusual by its association with an evolutive hepatitis B and by the occurrence of a lichen planus relapse.
A 10-year-old African boy has been seen for a pruritic dermatosis with papular lichenoid lesions on the trunk and the limbs and blisters on the lower limbs, both arise on lichen planus lesions and normal skin. The diagnosis of lichen planus pemphigoides was confirmed by histology which showed the features of lichen planus on a papule and of a sub-epidermal split on a bulla and by direct and indirect immunofluorescent studies which revealed an IgG and C3 linear deposit at the dermo-epidermal junction and the presence of circulating IgG anti-basement membrane zone antibodies. Laboratory investigations showed an evolutive hepatitis B (HBsAg +). Healing was obtained by dapsone and topical steroid therapy. Eight months after withdrawal of treatment the patient presented a non-bullous relapse of lichen planus. The histology showed a typical aspect of lichen planus and the immunofluorescence studies were negative. The hepatitis B serology was unchanged. The lesions rapidly improved with topical steroid and coaltar. One year later the patient exhibited few slight lichen planus lesions on the limbs and the hepatitis B serology showed the onset of sero-conversion.
Lichen planus and lichen planus pemphigoides are probably variants of the same disease. Their successive occurrence in our case report favours this hypothesis as does the association with an hepatitis B.
扁平苔藓类天疱疮是一种罕见的获得性自身免疫性大疱性皮肤病，通常影响成年人。儿童病例仅报道过4例。我们描述了1例儿童扁平苔藓类天疱疮新病例，该病例不同寻常之处在于其与进行性乙型肝炎相关，且出现了扁平苔藓复发。
一名10岁非洲男孩因躯干和四肢出现丘疹样苔藓样病变伴瘙痒性皮肤病，下肢出现水疱前来就诊，这些病变均出现在扁平苔藓皮损和正常皮肤上。扁平苔藓类天疱疮的诊断通过组织学得以证实，组织学显示丘疹处有扁平苔藓的特征，水疱处有表皮下裂隙，直接和间接免疫荧光研究显示在真皮-表皮交界处有IgG和C3线状沉积，且存在循环IgG抗基底膜带抗体。实验室检查显示为进行性乙型肝炎（乙肝表面抗原阳性）。通过氨苯砜和外用类固醇治疗病情得以缓解。停药8个月后，患者出现了扁平苔藓的非大疱性复发。组织学显示为典型的扁平苔藓表现，免疫荧光研究为阴性。乙肝血清学检查结果未变。皮损经外用类固醇和煤焦油治疗后迅速改善。1年后，患者四肢仅出现少许轻微扁平苔藓皮损，乙肝血清学检查显示出现血清学转换。
扁平苔藓和扁平苔藓类天疱疮可能是同一种疾病的不同变体。在我们的病例报告中它们相继出现，以及与乙型肝炎的关联均支持这一假说。
