IgA multiple myeloma presenting as an acquired bullous disorder

• Published in: Australasian journal of dermatology Vol. 40; no. 1; pp. 31 - 34
• Main Authors: Wong, Deanna A, Hunt, Michelle J, Stapleton, Karen
• Format: Journal Article
• Language: English
• Published: Melbourne, Australia Blackwell Science Pty 01.02.1999; Blackwell
• Subjects: Antineoplastic Combined Chemotherapy Protocols - administration & dosage; Biological and medical sciences; Biopsy; cutaneous paraneoplastic conditions; dermatitis herpetiformis; Diagnosis, Differential; Disease Progression; Fatal Outcome; Fluorescent Antibody Technique, Indirect; Humans; Immunodeficiencies. Immunoglobulinopathies; Immunoglobulin A - analysis; Immunoglobulinopathies; Immunopathology; linear IgA disease; Male; Medical sciences; Middle Aged; Multiple Myeloma - diagnosis; Multiple Myeloma - drug therapy; Multiple Myeloma - immunology; Multiple Myeloma - pathology; Skin - pathology; Skin Diseases, Vesiculobullous - diagnosis; Skin Diseases, Vesiculobullous - pathology; subepidermal blistering disorders; Bullous dermatosis; Case study; Human; Immunopathology; Skin disease; IgA; Immunoglobulinopathy; Lymphoproliferative syndrome; Malignant hemopathy; Myeloma; Inaugural sign
• ISSN: 0004-8380; 1440-0960
• 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 IgAκ 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 microabscesses. 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.