Papular mucinosis
Synonym(s)
Lichen myxedematosus; myxedematosus; scleromyxedema
Definition
Spectrum of disease characterized by generalized, densely grouped, dome-shaped papules with increased mucin deposition in the dermis, sometimes associated with a monoclonal gammopathy
Pathogenesis
May be a fibroblast disorder, causing increased mucin deposition in the skin
Clinical manifestation
Papular mucinosis (lichen myxedematosus) variant: dome-shaped and flesh-colored or erythematous papules, often in a pattern of parallel ridges, sometimes coalescing into grouped lichenoid papules, on dorsal hands, face, or extensor surfaces of the arms and legs; with extensive involvement, leonine faces and difficulty opening the mouth
Scleromyxedema variant: widespread, erythematous, indurated skin resembling scleroderma, with diffuse tightness and decreased range of motion; systemic manifestations include restrictive and obstructive pulmonary dysfunction, cardiovascular abnormalities, and polyarthritis; gastrointestinal...
References
- Rongioletti F, Rebora A (2001) Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. Journal of the American Academy of Dermatology 44(2):273–281PubMedCrossRefGoogle Scholar