Stretching scleromxyoedema treatment with IVIG

Scleromyxoedema is a rare, cutaneous mucinosis disorder with associated systemic manifestations that can lead to significant morbidity and mortality. It is characterised by a diffuse, flesh-coloured papular eruption and skin thickening predominantly of the face and hands. In recent years, intravenous immunoglobulin (IVIG) has emerged as a mainstay of treatment.
We report the case of a 33-year-old male who presented with a four week history of rapid progression of widespread skin tightening and erythema particularly affecting the supraorbital ridges and nasal dorsum of his face, as well as his hands, trunk and thighs. This was associated with sheets of waxy, lichenoid dermal papules with overlying erythema across the posterior upper arms and shoulder girdle. Seronegative inflammatory arthritis, immunoglobulin G lambda monoclonal gammopathy and upper dermal mucin on skin biopsy were also present.
Treatment with monthly IVIG, Flebogamma at a dosage of 1.3 g/kg, was commenced. A complete clinical response was observed following six cycles of therapy with an overall improvement in skin elasticity and joint function. The patient remains in remission but under close monitoring with a low threshold to reintroduce IVIG if required. As a rare but debilitating disease, it is important to recognise and treat scleromyxoedema, with IVIg representing a promising treatment modality.

Dr. Anna Schauer

Princess Margaret Hospital for Children, Subiaco, Perth, WA