- Papular mucinosis
Papular mucinosis 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.
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
Papular mucinosis is chronic and may be progressive. Patients have their dermal layer of the skin breaks 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.
Erythemaand 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 viscerais involved, the disease will be fatal.
There is no effective treatment for this condition. It has been reported that clearance of lesions can be done with
melphalanand cyclophosphamidealone or in combination with prednisone. Both isotretinoinand etretinatehave 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, PUVAphotochemotherapy, electron-beam therapy, IVIg, and dermabrasion. However, the overall prognosis for the disease is poor.
* [http://www.thestar.com.my/news/story.asp?file=/2006/12/20/nation/16369803&sec=nation What is Papular Mucinosis] , The Star, December 20, 2006.
* [http://dermatology.cdlib.org/DOJvol7num2/nyu2/3/3.html Papular Mucinosis] , Dermatology Online Journal Vol. 7 No. 2.
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