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[Extensive primary cutaneous mucinosis associated with refractory anemia with excess of blast]
P Stoebner1, T Lavabre-Bertrand, N Raison-Peyron
1Service de Dermatologie-Allergologie-Photobiologie, Hôpital Saint-Eloi, 2, avenue Bertin-Sans, 34295 Montpellier Cedex 05, France.
Background:
We describe an original case of a primary cutaneous mucinosis, associated with a refractory anemia with excess of blast.
Case Report:
A 65 year-old female consulted for erythematous-purple blush reticulated and large plaques that progressively developed on the trunk and limbs. Biopsies showed abundant dermis mucin deposits with a perivascular lymphocytic infiltrate. The epidermis was normal. Laboratory evaluation showed refractory anemia with excess of blast, associated with cytogenetic abnormalities. After two years course, the progressive aggravation of the myelodysplastic syndrome was associated with a progressive extension of the cutaneous mucinosis.
Discussion:
This is the first report of an atypical primary diffuse and extensive cutaneous mucinosis. Primary cutaneous mucinosis may be associated with systemic diseases. To our knowledge, the association with refractory anemia with excess of blast has not been described previously. Mucine deposits may be considered as a consequence of neutrophilic dysfunction that are observed in myelodysplastic syndromes.