Dermatology Therapy

2004 Edition
| Editors: N. Levine, C. C. Levine

Papular mucinosis

Reference work entry
DOI: https://doi.org/10.1007/3-540-29668-9_2032

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...

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References

  1. 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

Copyright information

© Springer-Verlag 2004