Lichen Myxedematosus

Lichen Myxedematosus

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The terms lichen myxedematosus, papular mucinosis, and scleromyxedema are used interchangeably to describe the same disorder. A spectrum of disease appears to exist, with the more localized, less severe forms, which are generally called lichen myxedematosus or papular mucinosis, and the more sclerotic, diffuse form, which is referred to as scleromyxedema. Lichen myxedematosus is a rare skin disorder characterized by fibroblast proliferation and mucin deposition in the dermis, in the absence of thyroid disease. Sclerotic features, systemic involvement, and monoclonal gammopathy are absent in localized lichen myxedematosus.

The etiology is unknown; however, the disease is commonly associated with plasma cell dyscrasia. The basic defect is hypothesized to be a fibroblast disorder, which causes the increased mucin deposition in the skin. The cytokines interleukin (IL)–1, tumor necrosis factor (TNF)–alpha, and TNF-beta may play a role. Most patients have a monoclonal paraprotein band, usually of the immunoglobulin G (IgG) type. The association between this paraprotein and the mucin deposition is not clear, and the protein does not directly stimulate fibroblast proliferation. Paraprotein levels have not been shown to correlate with disease severity, response to therapy, or disease progression.

Persons of any race can be affected.

No sex-related predilection is reported.

Most individuals with lichen myxedematosus are aged 30-70 years.

For lichen myxedematosus, the prognosis is a chronic course with little tendency for spontaneous resolution. Although most patients have a monoclonal paraproteinemia, they rarely have associated multiple myeloma. However, when myeloma is present, the patient generally has a poor prognosis.

Scleromyxedema usually has a poorer prognosis than that of the other forms. Typical causes of death include complications of systemic therapeutic agents such as melphalan or systemic disease such as cardiovascular involvement.

Patients with cardiac or pulmonary involvement also have a poor prognosis.

Patients with lichen myxedematosus have a long-term, disfiguring, possibly disabling disorder. The risks and benefits of systemic therapy must be weighed carefully.

Marshall K, Klepeiss SA, Ioffreda MD, Helm KF. Scleromyxedema presenting with neurologic symptoms: a case report and review of the literature. Cutis. 2010 Mar. 85(3):137-40. [Medline].

Serdar ZA, Altunay IK, Yasar SP, Erfan GT, Gunes P. Generalized papular and sclerodermoid eruption: scleromyxedema. Indian J Dermatol Venereol Leprol. 2010 Sep-Oct. 76(5):592. [Medline].

Montgomery H, Underwood LJ. Lichen myxedematosus; differentiation from cutaneous myxedemas or mucoid states. J Invest Dermatol. 1953 Mar. 20(3):213-36. [Medline].

Yaron M, Yaron I, Yust I, Brenner S. Lichen myxedematosus (scleromyxedema) serum stimulates hyaluronic acid and prostaglandin E production by human fibroblasts. J Rheumatol. 1985 Feb. 12(1):171-5. [Medline].

Brunet-Possenti F, Hermine O, Marinho E, Crickx B, Descamps V. Combination of intravenous immunoglobulins and lenalidomide in the treatment of scleromyxedema. J Am Acad Dermatol. 2013 Aug. 69(2):319-20. [Medline].

Hill TG, Crawford JN, Rogers CC. Successful management of lichen myxedematosus. Report of a case. Arch Dermatol. 1976 Jan. 112(1):67-9. [Medline].

Kaymen AH, Nasr A, Grekin RC. The use of carbon dioxide laser in lichen myxedematosus. J Dermatol Surg Oncol. 1989 Aug. 15(8):862-5. [Medline].

Jacob SE, Fien S, Kerdel FA. Scleromyxedema, a positive effect with thalidomide. Dermatology. 2006. 213(2):150-2. [Medline].

Caudill L, Howell E. Scleromyxedema: a case clinically and histologically responsive to intravenous immunoglobulin. J Clin Aesthet Dermatol. 2014 May. 7(5):45-7. [Medline].

Cao XX, Wang T, Liu YH, Zhou DB, Li J. Successful treatment of scleromyxedema with melphalan and dexamethasone followed by thalidomide maintenance therapy. Leuk Lymphoma. 2016 Apr 27. 1-3. [Medline].

Chockalingam R, Duvic M. Scleromyxedema: long-term follow-up after high-dose melphalan with autologous stem cell transplantation. Int J Dermatol. 2016 May 21. [Medline].

Rongioletti F, Zaccaria E, Cozzani E, Parodi A. Treatment of localized lichen myxedematosus of discrete type with tacrolimus ointment. J Am Acad Dermatol. 2008 Mar. 58(3):530-2. [Medline].

Sansbury JC, Cocuroccia B, Jorizzo JL, Gubinelli E, Gisondi P, Girolomoni G. Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients. J Am Acad Dermatol. 2004 Jul. 51(1):126-31. [Medline].

Horn KB, Horn MA, Swan J, Singhal S, Guitart J. A complete and durable clinical response to high-dose dexamethasone in a patient with scleromyxedema. J Am Acad Dermatol. 2004 Aug. 51(2 Suppl):S120-3. [Medline].

Howsden SM, Herndon JH Jr, Freeman RG. Lichen myxedematosus. A dermal infiltrative disorder responsive to cyclophosphamide therapy. Arch Dermatol. 1975 Oct. 111(10):1325-30. [Medline].

Elizabeth A Liotta, MD Chief Dermatologist and Sole Proprietor, Integrated Skin Care Centers

Elizabeth A Liotta, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, Association of Military Dermatologists, Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Mark W Cobb, MD Consulting Staff, WNC Dermatological Associates

Mark W Cobb, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology

Disclosure: Nothing to disclose.

Lichen Myxedematosus

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