Papular mucinosis

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Papular mucinosis
SpecialtyDermatology Edit this on Wikidata

Papular mucinosis (also known as scleromyxedema,[1][2] "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

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.

viscera is involved, the disease will be fatal. The dermatoneuro syndrome
is a rare neurological complication of the disease presenting with fever, seizures and altered mental status.

Diagnosis

Classification

Papular mucinosis may be divided into several types or variants:[1]

Treatment

There is no effective treatment for this condition. It has been reported that clearance of

IVIg and dermabrasion. However, the overall prognosis for the disease is poor. There are reported instances of remission of the disease when treated with a combination of Revlimid and Dexamethasone over a 24-month period.[citation needed
]

Incidence

Papular mucinosis affects adults of both sexes equally and appears between ages 30 and 80. Recently,[

paraproteinemias such as IgG lambda paraproteinemia.[citation needed
]

See also

  • List of cutaneous conditions

Notes

  1. ^
    ISBN 978-1-4160-2999-1
    .
  2. PMID 24919716. Archived from the original
    on December 29, 2012. Retrieved August 18, 2014.

References

External links


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