Lichen myxedematosus

Published on 19/03/2015 by admin

Filed under Dermatology

Last modified 19/03/2015

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Lichen myxedematosus

Jessica A. Kaffenberger and Joslyn S. Kirby

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

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Lichen myxedematosus (LM) is a rare, chronic disease characterized by infiltration of the skin with mucin-producing fibroblasts. Typical examination findings include shiny, flesh-colored to erythematous papules, nodules, and plaques. More extensive cutaneous disease can cause widespread thickening and hardening of the skin with large, raised folds. The disease favors the face and extremities. Localized and systemic subtypes of the disease are now recognized. The systemic form, scleromyxedema, is associated with a monoclonal IgG lambda gammopathy. Localized forms of the disease are limited to the skin, have a better prognosis and are not associated with paraproteinemia. Localized forms include acral persistent papular mucinosis, self-healing papular mucinosis, discrete LM, nodular LM, and cutaneous mucinosis of infancy. The cause of the disease is unknown.

Management strategy

The treatment of LM remains a challenge. The absence of any controlled studies makes comparison of different drugs or drug regimens difficult.

Localized forms may be observed or treated with topical medications such as topical calcineurin inhibitors or destructive therapies such as cryotherapy, dermabrasion, or hyaluronidase.

The systemic form, scleromyxedema, is treated more aggressively and patients may require treatment with multiple medications either serially or in combination before a successful therapy is found. Given the association between scleromyxedema and monoclonal gammopathy, some of the therapies for systemic LM are taken from the treatment of multiple myeloma. Melphalan is an alkylating agent generally prescribed as a pulse regimen of four times daily for 4 days every 4 to 6 weeks, or four times daily until symptoms resolve; however, its use is limited by secondary adverse effects including malignancy, sepsis, and death. Melphalan has also shown beneficial results when used in combination with other therapies including plasmapheresis, oral prednisone, or autologous stem cell transplant. Several other agents, including bortezomib, 2-chlorodeoxyadenosine (cladribine), cyclophosphamide, cyclosporine, methotrexate, and thalidomide, have demonstrated some efficacy.

Other alternatives to immunosuppressive agents include intravenous immunoglobulin (IVIG), isotretinoin, interferon-α2b, intralesional triamcinolone acetonide, psoralen with UVA (PUVA), and extracorporeal photochemotherapy.

Specific investigations

First-line therapies

imageMelphalan C
imageSystemic corticosteroids D
imagePlasmapheresis D
imageIntravenous immunoglobulin D

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