Extensive primary cutaneous mucinosis associated with refractory anemia with excess of blast.

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 re ticulated and large plaques that progressively developed on the trunk and l imbs. Biopsies showed abundant dermis mucin deposits with a perivascular ly mphocytic infiltrate. The epidermis was normal. Laboratory evaluation showe d refractory anemia with excess of blast, associated with cytogenetic abnor malities. After two years course, the progressive aggravation of the myelod ysplastic syndrome was associated with a progressive extension of the cutan eous mucinosis. Discussion, This is the first report of an atypical primary diffuse and ext ensive cutaneous mucinosis. Primary cutaneous mucinosis may be associated w ith systemic diseases. To our knowledge, the association with refractory an emia with excess of blast has not been described previously. Mucine deposit s may be considered as a consequence of neutrophilic dysfunction that are o bserved in myelodysplastic syndromes.