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Immunoglobulin Type Gamma 4‐Related Disease Presenting with Cutaneous Plaques

We read with interest the recent article ‘IgG4-related disease with cutaneous manifestations treated with rituximab: Case report and literature review ’ in Australian Journal of Dermatology and would like to report a case of extensive immunoglobulin type gamma 4 (IgG4)-related skin disease which responded extremely well to prednisolone and azathioprine. A 61-year-old woman presented at our clinic with a 3-year history of a large erythematous, nodular plaque on the left cheek (Fig. 1a). Six months previously, similar lesions had developed on her forehead. Eight years prior, she had undergone a left parotidectomy and the histology revealed a non-specific lymphocytic infiltrate. A skin biopsy of the left cheek plaque demonstrated an inflammatory infiltrate of CD20-positive B-cells and CD4predominant T-cells, with a large number of IgG4 positive plasma cells. The parotid gland was re-examined, and histology demonstrated marked lymphoid hyperplasia with prominent germinal centres, polyclonal plasma cells and focal granulomas. Her serum IgG4 level was elevated at 2.24 g/L (0.03–2.01 g/L). A full body CT scan showed no systemic involvement. She was commenced on prednisolone and azathioprine with significant amelioration of the plaques (Fig. 1b). IgG4-related disease is a newly described systemic, autoimmune, fibro-inflammatory condition that can present with cutaneous features, with recent reports demonstrating the effectiveness of multiple agents including oral steroids, mycophenolate mofetil, azathioprine, methotrexate, thalidomide and rituximab. Our case complements that of Jalilian and colleagues and again demonstrates a late diagnosis after a previous parotidectomy, possibly because the condition was not well described when the patient initially presented.