Carew Benjamin, Wagner Godfrey
Queensland Institute of Dermatology, Denman St, Greenslopes, Queensland, Australia.
Australas J Dermatol. 2014 Nov;55(4):292-5. doi: 10.1111/ajd.12154.
A 79 year-old male patient presented clinically as a typical case of pemphigus foliaceus. He displayed a seborrhoeic pattern of crusting and erosions with an absence of oral involvement. Surprisingly, a biopsy showed suprabasilar acantholysis and direct immunofluorescence consistent with pemphigus vulgaris. Indirect pemphigus antibodies were positive for desmoglein 3 (DSG-3) but negative for DSG-1 antibodies. Most cases of cutaneous pemphigus are reported to have both DSG-1 and DSG-3 antibodies. A rare cutaneous subtype of pemphigus vulgaris is reported in the literature that may present clinically as seen in our patient. Our patient is clinically similar to these previous reported cases but with a negative DSG-1 antibody titre. The extended DSG compensation theory explains the differences of clinical expression in pemphigus based on the variable pathogenicity of DSG antibodies as well as the distribution of DSG-1 and DSG-3. We discuss this case, highlighting the theories of the pathogenesis of pemphigus and, in particular, the cutaneous pemphigus subtype.
一名79岁男性患者临床表现为典型的落叶型天疱疮病例。他表现出脂溢性结痂和糜烂，无口腔受累。令人惊讶的是，活检显示基底细胞上层棘层松解，直接免疫荧光检查结果与寻常型天疱疮一致。间接天疱疮抗体桥粒芯糖蛋白3（DSG - 3）呈阳性，但桥粒芯糖蛋白1（DSG - 1）抗体呈阴性。据报道，大多数皮肤型天疱疮病例同时存在DSG - 1和DSG - 3抗体。文献中报道了一种罕见的寻常型天疱疮皮肤亚型，其临床表现可能与我们的患者所见相似。我们的患者在临床上与这些先前报道的病例相似，但DSG - 1抗体滴度为阴性。扩展的DSG补偿理论基于DSG抗体的可变致病性以及DSG - 1和DSG - 3的分布解释了天疱疮临床表型的差异。我们讨论此病例，重点阐述天疱疮的发病机制理论，特别是皮肤型天疱疮亚型。
