The localized papular form has four variants: a discrete papular form, an acral persistent form, a nodular form, and a papular mucinosis of infancy (2).
Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease.
Other differential diagnoses include self-healing juvenile cutaneous mucinosis and acral persistent
papular mucinosis. Self-healing juvenile cutaneous mucinosis is less likely given the lesions persisted in our patients.
Papular mucinosis (discrete papular lichen myxedematosus)
Papular mucinosis (PM) or lichen myxedematosus, is a primary cutaneous mucinosis which is characterized by lichenoid papules, nodules or plaques due to dermal mucin deposition and fibroblast proliferation in absence of thyroid disorder.2 The most accepted classification for papular mucinosis is by Rongioletti.3 Papular mucinosis is classified into generalized and localized forms.4,5,6
Adults of both sexes are affected equally by papular mucinosis. The disease appears between 30-80 years of age.
Interestingly, in a case of self-healing papular mucinosis, which is a milder form of sclerodermoid lichen myxedematosus, CD34+ or FXIII-A-positive DCs have been described next to mast cells, suggesting that these cells together could contribute to the dermal mucin deposition of the lesions [67].
Muto, "Adult variant of self-healing papular mucinosis in a patient with rheumatoid arthritis: Predominant proliferation of dermal dendritic cells expressing CD34 or factor XIIIa in association with dermal deposition of mucin," Journal of Dermatology, vol.
Scleromyxedema (also called
papular mucinosis) is a chronic connective tissue disorder in which mucin deposits cause the skin to become red and raised, and make the movement of affected areas difficult.
(12.) Rongioletti F: Lichen myxedematosus (
papular mucinosis): new concepts and perspectives for an old disease.
Based on the histopathology alone, the differential diagnosis would include cutaneous mucinosis of infancy,
papular mucinosis, and localized myxedema.
Eruptive syringoma may clinically resemble lichen planus, verruca plana, xanthoma disseminatum, granuloma annulare,
papular mucinosis and mastocytosis.7
