Wong D A, Hunt M J, Stapleton K
Department of Dermatology, Westmead Hospital, Sydney, New South Wales, Australia.
Australas J Dermatol. 1999 Feb;40(1):31-4. doi: 10.1046/j.1440-0960.1999.00312.x.
A 63-year-old man presented with an intensely pruritic vesiculo-bullous eruption on the limbs and was subsequently found to have an IgA kappa multiple myeloma. The eruption clinically and histologically was suggestive of linear IgA disease (LAD), dermatitis herpetiformis (DH), epidermolysis bullosa acquisita (EBA), or bullous lupus erythematosus (LE), with the skin biopsy revealing subepidermal bullae and dermal papillary micro-abscesses. However, direct immunofluorescence showed a unique pattern of diffuse dermal IgA staining. Although chemotherapy produced a dramatic resolution of the lesions, which paralleled the fall in serum IgA paraprotein level, the myeloma later became progressive and the resulting paraprotein increase was accompanied by recurrence of the eruption. We propose that this patient's rash was the presenting manifestation of his multiple myeloma, and was a consequence of transudation of IgA paraprotein into the dermis.
一名63岁男性患者四肢出现剧烈瘙痒的水疱大疱性皮疹,随后被诊断为IgA κ型多发性骨髓瘤。该皮疹在临床和组织学上提示为线状IgA病(LAD)、疱疹样皮炎(DH)、获得性大疱性表皮松解症(EBA)或大疱性红斑狼疮(LE),皮肤活检显示表皮下水疱和真皮乳头微脓肿。然而,直接免疫荧光显示真皮弥漫性IgA染色的独特模式。尽管化疗使病变显著消退,这与血清IgA副蛋白水平下降相平行,但骨髓瘤后来进展,副蛋白增加伴随皮疹复发。我们认为该患者的皮疹是其多发性骨髓瘤的首发表现,是IgA副蛋白渗入真皮的结果。