Callot V, Bagot M
Service de dermatologie, hôpital Henri-Mondor, Créteil.
Rev Prat. 1994 Jan 1;44(1):81-5.
Pemphigus is a rare autoimmune bullous disease of the skin and mucosae. It has predisposing genetic factors and, in the case of pemphigus foliaceus, environmental factors; some therapeutic drugs may trigger off pemphigus. The disease often begins with painful buccal erosions, followed by flaccid bullae on healthy skin. Some lesions are erythematous plaques involving the seborrheic areas. Cytology shows acantholysis and histology, intraepidermal cleavage. Direct immunofluorescence confirms the diagnosis by showing a network deposit of immunoglobulins and complement. The titres of circulating antibodies to the intracellular substance vary with the course of the disease. The loss of cohesion between keratinocytes is induced by autoantibodies directed against antigens of the epidermal cell junction zone; the molecular weight of these antibodies is 130 and 85 kD for pemphigus vulgaris and 160 and 85 for superficial pemphigus. Paraneoplastic pemphigus is a recently individualized entity. Treatment consists of systematic corticosteroid therapy, sometimes associated with immunosuppressants; it has improved the prognosis of this once lethal disease.
天疱疮是一种罕见的皮肤和黏膜自身免疫性大疱性疾病。它有易感的遗传因素，对于落叶型天疱疮而言，还有环境因素；一些治疗药物可能引发天疱疮。该病常始于疼痛的颊部糜烂，随后在健康皮肤上出现松弛性水疱。一些损害为累及脂溢性区域的红斑性斑块。细胞学检查显示棘层松解，组织学检查显示表皮内裂隙。直接免疫荧光通过显示免疫球蛋白和补体的网状沉积来确诊。针对细胞内物质的循环抗体滴度随疾病进程而变化。角质形成细胞间黏附力的丧失是由针对表皮细胞连接区抗原的自身抗体诱导的；寻常型天疱疮这些抗体的分子量为130和85kD，浅表型天疱疮为160和85kD。副肿瘤性天疱疮是一种最近才被明确的疾病实体。治疗包括系统性皮质类固醇治疗，有时联合免疫抑制剂；这改善了这种曾是致命疾病的预后。
