Arndt–Gottron scleromyxoedema: successful therapy with intravenous immunoglobulins

Conflicts of interest: none declared. Sir, Arndt–Gottron scleromyxoedema is a rare fibromucinous disorder regarded as a variant of the lichen myxoedematosus. The diagnostic criteria are a generalized papular and sclerodermoid eruption, a microscopic triad of mucin deposition, fibroblast proliferation and fibrosis, a monoclonal gammopathy (mostly IgG‐λ paraproteinaemia) and the absence of a thyroid disorder. This disease initially presents with sclerosis of the skin and clusters of small lichenoid papules with a predilection for the face, neck and the forearm. Progressively, the skin lesions can become more widespread and the induration of skin can result in a scleroderma‐like condition with sclerodactyly and microstomia, reduced mobility and disability. Systemic involvement is common, e.g. upper gastrointestinal dysmotility, proximal myopathy, joint contractures, neurological complications such as psychic disturbances and encephalopathy, obstructive/restrictive lung disease, as well as renal and cardiovascular involvement. Numerous treatment options have been described in the literature. These include corticosteroids, retinoids, thalidomide, extracorporeal photopheresis (ECP), psoralen plus ultraviolet A radiation, ciclosporin, cyclophosphamide, melphalan or autologous stem cell transplantation.