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Papular mucinosis (also known as "Scleromyxedema,"^[1]^[2] "Generalized lichen myxedematosus," and "Sclerodermoid lichen myxedematosus") is a rare skin disease. Localized and disseminated cases are called papular mucinosis or lichen myxedematosus while generalized, confluent papular forms with sclerosis are called scleromyxedema. Frequently, all three forms are regarded as papular mucinosis. However, some authors restrict it to only mild cases. Another form, acral persistent papular mucinosis is regarded as a separate entity.

Classification

Papular mucinosis may be divided into several types or variants:^[1]

Acral persistent papular mucinosis
Self-healing papular mucinosis
Papular mucinosis of infancy
Cutaneous mucinosis of infancy
Nodular lichen myxedematosus
Localized lichen myxedematosus
Discrete papular lichen myxedematosus

Incidence

Papular mucinosis affects adults of both sexes equally and appears between ages 30 and 80. Recently, it has been reported in patients infected with the HIV/AIDS virus.

Characteristic

Papular mucinosis is chronic and may be progressive. The dermal layer of the skin breaks out into small and solid bumps, usually conical in shape and measured from 2 to 4 mm or sometimes flat-topped papules. Unlike pustules, these bumps do not contain pus. Instead they contain mucin, a substance of mucus, the body's natural and protective lubricant found in saliva and epithelial cells in lungs and the sensitive part of the nose. They usually come in clusters such as linear arrays. Less frequently, urticarial, nodular, or sometimes annular lesions may be appreciated. The dorsal aspect of the hands, face, elbows, and extensor portions of the extremities are most frequently affected. Mucosal lesions are absent. The coalescence of papules on the face, particularly on the glabella, results in longitudinal folding and gives the appearance of a leonine facies.

In scleromyxedema, symptoms can occur on larger part of the body. Erythema and scleroderma-like induration occurs on the skin. In addition, the mobility of the lips, hands, arms, and legs is reduced. Proximal myopathy, inflammatory polyarthritis, central nervous system symptoms, esophageal aperistalsis, and hoarseness are among the notable systemic symptoms. If viscera is involved, the disease will be fatal. The dermatoneuro syndrome is a rare neurological complication of the disease presenting with fever, seizures and altered mental status.

Treatment

There is no effective treatment for this condition. It has been reported that clearance of lesions can be done with melphalan and cyclophosphamide alone or in combination with prednisone. Both isotretinoin and etretinate have also been shown to improve the conditions. All medications listed can cause adverse symptoms, with isotretinoin and etretinate particularly dangerous since they are both teratogens. Other attempted treatments include interferon-alpha, cyclosporine, PUVA photochemotherapy, electron-beam therapy, IVIg, and dermabrasion. However, the overall prognosis for the disease is poor. There are reported instances of remission of the disease when treated with a combination of Revlimid and Dexamethasone over a 24-month period.

Notes

^ ^a ^b Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
^ Applebaum DS, Daulat S, Duvic M (August 2014). "Clinicopathological Challenge: Multiple Skin-Colored Papules With Diffuse Sclerosis". JAMA Dermatol. 150 (8): 893–894. doi:10.1001/jamadermatol.2014.14.

References

What is Papular Mucinosis, The Star, December 20, 2006.
Papular Mucinosis, Dermatology Online Journal Vol. 7 No. 2.

UpToDate Contents

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English Journal

Scleromyxoedema with disseminated subcutaneous nodules: rare presentation of an uncommon dermatosis.

Singh S, Kanwar AJ, Saikia UN.SourceDepartment of Dermatology and Venereology Department of Pathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
Clinical and experimental dermatology.Clin Exp Dermatol.2013 Jan;38(1):36-9. doi: 10.1111/j.1365-2230.2012.04387.x. Epub 2012 May 21.
Scleromyxoedema, also known as generalized lichen myxoedematosus, is a cutaneous mucinosis characterized by a generalized papular and sclerodermoid eruption, mucin deposition, increased fibroblast proliferation and fibrosis. It is often associated with underlying monoclonal gammopathy, and it respon
PMID 22607617

Successful treatment of scleromyxedema with oral thalidomide.

Guarenti I, Sebastiani V, Pinto G, de Souza PR, de Almeida H Jr.SourceFrom the Department of Dermatology Federal and Catholic University of Pelotas Santa Casa de Porto Alegre Brazil E-mail: hiramalmeidajr@hotmail.com.
International journal of dermatology.Int J Dermatol.2012 Dec 11. doi: 10.1111/j.1365-4632.2011.05030.x. [Epub ahead of print]
PMID 23231733

Japanese Journal

Scleromyxedema

Internal Medicine 53(21), 2561-2562, 2014
NAID 130004704968

手背から前腕に病変が多発したDiscrete Papular Lichen Myxoedematosus (特集代謝異常症)

皮膚科の臨床 55(13), 1871-1874, 2013-12
NAID 40019921745

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