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 HIV
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. 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.
There is no effective treatment for this condition. It has been reported that clearance of lesions can be done with melphalan
alone or in combination with prednisone
. Both isotretinoin
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
photochemotherapy, electron-beam therapy, IVIg
, and dermabrasion
. However, the overall prognosis for the disease is poor.