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Lichen myxedematosus
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Lichen myxedematosus (LM) is a rare, chronic disease characterized by infiltration of the skin with mucin-producing fibroblasts. Typical examination findings include firm, indurated, shiny, skin-colored to erythematous papules that can coalesce into nodules and plaques. The face, trunk, and/or extremities may be affected. Extensive disease can cause widespread firm, thickened skin, including leonine facies. Typical histopathologic features include diffuse dermal mucin deposition and fibroblast proliferation. LM, typically scleromyxedema, can be associated with systemic sequelae including dysphagia due to esophageal dysmotility, proximal muscle weakness, arthralgia, arthritis, joint contractures, obstructive or restrictive lung disease, cardiac ischemia and cardiomyopathy, macular edema, peripheral neuropathy and dermatoneuro syndrome with encephalopathy, convulsions, coma, and/or psychosis. Thus, it is crucial to evaluate patients for systemic manifestations to distinguish those with a pure cutaneous form of LM from those with a severe form with systemic involvement. Localized forms typically do not have these associations and include acral persistent papular mucinosis, self-healing papular mucinosis, discrete LM, nodular LM, and cutaneous mucinosis of infancy.
Management Strategy
The treatment of LM remains a challenge. The absence of controlled studies makes comparison of different drugs or drug regimens difficult.
Localized forms may be observed or treated with topical medications ( topical corticosteroids or calcineurin inhibitors ) or destructive therapies such as cryotherapy , dermabrasion , or hyaluronidase .
The systemic form, scleromyxedema, is treated more aggressively and typically requires systemic treatment. Intravenous immunoglobulin (IVIG) is the first treatment of choice for patients with scleromyxedema, especially if there is rapid spread of skin disease or deterioration in systemic organ function. The standard dose is 2 g/kg divided, for 4 or 5 days. This is administered every 4 weeks, and once stability is achieved the frequency can be increased to 6 weeks. If there is no improvement after 6 months, then IVIG should be discontinued and an alternate treatment initiated. Given the association between scleromyxedema and monoclonal gammopathy, some therapies for systemic LM parallel the treatment of multiple myeloma. Melphalan has shown beneficial results when used alone or in combination with other therapies, including systemic corticosteroids, or autologous stem cell transplant. Melphalan use is severely limited by adverse effects, including malignancy, sepsis, and death.
Specific Investigations
Lichen myxedematosus: diagnostic criteria, classification, and severity grading
Nofal A, Amer H, Alakad R, et al. Int J Dermatol 2017; 56: 284–90.
This group suggests an alternative approach to categorizing LM based on extent of symptoms and cutaneous signs of disease: (1) mild, which has limited or localized cutaneous lesions without systemic symptoms; (2) moderate, which has generalized cutaneous lesions without systemic symptoms; and (3) severe, which includes people with localized or generalized cutaneous lesions and the presence of systemic symptoms. This helps to account for reported cases with localized skin changes and the presence of systemic symptoms as well as extensive skin involvement and the absence of systemic symptoms.
Scleromyxedema
Hummers LK. Curr Opin Rheumatol 2014; 26: 658–62.
An abnormal paraprotein is found in most patients (>80%) with scleromyxedema. The monoclonal gammopathy is usually IgG with a predominance of lambda light chains over kappa light chains, while IgM-kappa, IgA-kappa, or IgA-lambda are less frequent. Esophageal dysmotility can affect about 50% and myopathy about 10%–50%. The cause of the disease is unknown, and it must be distinguished from thyroid-related mucinoses. Associations with HIV and hepatitis C virus infections are rare.
Scleromyxedema secondary to hepatitis C virus and successfully treated with antiviral therapy
Smith JA, Kalimullah FA, Erickson CP, et al. Dermatol Online J 2015; 21: 9.
Small studies have associated scleromyxedema with hepatitis C infection. This appears more common in Japanese patients. A handful of cases outside Japan have been reported, including this patient who improved with ribavirin and interferon-α 2b .
Scleromyxedema and lichen myxedematosus: is it associated with viral hepatitis?
Chen K, Tzeng I, Lee Y, et al. J Dermatology 2019; 46: 879–85.
A retrospective review of patients with scleromyxedema and LM that analyzed 28 patients. The analysis showed the rate of hepatitis B to be similar among patients and the general population, whereas for hepatitis C the rate was found to be increased 6.5-times relative to the general population.
Scleromyxedema or LM with Systemic Manifestations
First-Line Therapies
Clinical and molecular phenotyping in scleromyxedema pre- and post-treatment with intravenous immunoglobulin
Mecoli CA, Talbot CC Jr, Fava A, et al. Arthritis Care Res 2019 Apr 22. doi:10.1002/acr.23908.
Fifteen patients with scleromyxedema were treated with IVIG 2 g/kg over a 2–5 day period every 4 weeks. The infusion interval is increased to every 6–12 weeks once improvement in skin flexibility and softening plateaus. Tc17 cells were elevated in the skin and correlated with extent of skin involvement and decreased with IVIG treatment.
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