Arndt-Gottron scleromyxoedema: successful therapy with intravenous immunoglobulins.

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-k 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. In September 1999, a 48-year-old white female first noticed an erythematous induration with a lichenoid papular eruption on her forehead. Three months later the lesions became more widespread including her face (Fig. 1a), neck, shoulders, forearms (Fig. 2a) and legs. When the patient first presented in our department in June 2000, she had problems opening her mouth fully as well as clenching both hands or moving her wrist. The histological examination of the skin biopsy was highly characteristic of Arndt–Gottron scleromyxoedema. Full blood count, blood morphology, bone marrow biopsy, bone scintigraphy and thyroid function tests were normal. Serum immunoelectrophoresis revealed an IgG-k paraproteinaemia. Urinary Bence-Jones proteins were negative. No systemic involvement was disclosed. We initiated ECP therapy in August 2000, initially at 2-week intervals (later monthly) on two succeeding days. When there was no improvement after 3 months, we also administered cyclophosphamide (Endoxana ; Baxter Healthcare Ltd, Newbury, U.K.) at a daily dose of 100 mg with mesna 400 mg (Uromitexan ; Baxter) prophylaxis. The response to this therapy was rather moderate. In February 2003 the patient developed a change of personality and loss of orientation and was admitted to hospital. The extensive neurological, radiological and microbiological diagnostics were unremarkable at that time. A few hours later the patient had seizures and was put on artificial ventilation in an intensive care unit. The patient was comatose for several days. A repeated magnetic resonance imaging scan was still normal, but the cerebrospinal fluid tap showed a dysfunction of the blood–cerebrospinal fluid barrier. A bilateral loss of somatosensory evoked potentials was noticeable. The neurological symptoms were classified as a ‘dermatoneuro’ syndrome, a rare extracutaneous manifestation of scleromyxoedema. After initiation of treatment with methylprednisolone (Urbason ; Aventis, Frankfurt, Germany) the neurological situation normalized in the following 2 weeks. No further medical treatment was necessary. In April 2003 therapy options were re-evaluated and the patient was started and maintained on a 7-day course of melphalan 7.5 mg daily (Alkeran ; GlaxoSmithKline, Uxbridge, U.K.) in combination with prednisolone 40 mg daily (Decortin H ; Merck, Darmstadt, Germany) every 6 weeks. This treat(a)