Remission of scleromyxoedema following treatment with extracorporeal photopheresis

Abstract

Scleromyxoedema, a disseminated papular and sclerotic variant of lichen myxoedematosus, is a rare disease with a chronic progressive course, and little tendency towards spontaneous remission. The treatment of scleromyxoedema has been largely ineffective. Aggressive chemotherapeutic agents have been used, often leading to therapy-related morbidity and mortality. We report a 41-year-old woman with scleromyxoedema, associated with a monoclonal gammopathy of IgG-kappa type, whose condition almost completely cleared with 12 monthly sessions of extracorporeal photopheresis. The patient had previously not responded to isotretinoin, and chlorambucil with prednisolone.

Successful treatment of lichen myxoedematosus with PUVA photochemotherapy

“…Photo-chemotherapy has been used with satisfactory results in patients with scleromyxoedema. One patient was treated with PUVA photochemotherapy (3) and two cases with extracorporeal photopheresis (6,7). The photopheresis was done on 2 consecutive days every month, and significantly or completely cleared the skin changes after 12 months of therapy in both patients (6,7).…”

Cutaneous and noncutaneous disorders treated with extracorporeal photopheresis

“…At the end of 12 treatments, joint mobility was improved and the skin lesions were greatly improved. After 5 months without ECP therapy, the patient's condition was still improved 89 …”

“…There have been two case reports of scleromyxedema treated with ECP 89,90 . In one report, a 41‐year‐old woman had a 6‐month history of scleromyxedematous lesions and a serum monoclonal IgG‐κ type gammopathy 89 . After 6 months of isotretinoin therapy with no success, chlorambucil and prednisone were administered for 18 months, again with little improvement.…”

Scleredema associated with paraproteinaemia treated by extracorporeal photopheresis

“…On the basis of two reports of successful treatment with ECP of scleromyxoedema, another cutaneous mucinosis associated with paraproteinaemia, the patient was referred to the Rotherham ECP service. 1 , 2 She embarked on a course of photopheresis, receiving one treatment on each of 2 consecutive days per month. Psoralen levels were monitored to achieve a buffy coat concentration of 50 ng mL −1 .…”