Papular mucinosis (also known as scleromyxedema, "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. Presentation
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 waxy substance of mucus, the bodys 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. Redness and scleroderma-like induration occurs on the skin. In addition, the mobility of the lips, hands, arms, and legs is reduced.
Aquagenic pruritus is a skin condition characterized by the development of severe, intense, prickling-like epidermal itching without observable skin lesions and evoked by contact with water. Presentation
Presentation varies from person to person. Some people have discrete attacks, which can last between 10 and 120 minutes while others are symptomatic almost constantly due to atmospheric humidity levels and/or sweating. Itching most frequently occurs on the legs, arms, chest, back, and abdomen, though it can also occur elsewhere.Itching on contact with water that also includes hives is known as aquagenic urticaria. Pathogenesis
The exact mechanism of the condition is unknown. Some studies have suggested the itching occurs in response to increased fibrinolytic activity in the skin, inappropriate activation of the sympathetic nervous system, increased activity of acetylcholinesterase, or an increase in mast cell degranulation that releases histamine and other chemicals into the body. Diagnosis
No definitive medical test is known for aquagenic pruritus. Rather, diagnosis is made by excluding all other possible causes of the patients itching, including polycythemia vera. Since pruritus is a symptom of many serious diseases, it is important to rule out other causes before making a final diagnosis. Treatment
Beta-Alanine, a nonessential amino acid and freely available as a nutritional supplement in many countries, has been found to suppress or significantly reduce the symptoms in many cases. Anecdotal evidence indicates that it is commonly consumed in doses of 750 mg to 2 grams before water contact.