BENEFICIAL EFFECT OF GRANULOCYTE-COLONY-STIMULATING FACTOR IN SCLEROMYXEDEMA ASSOCIATED WITH SEVERE IDIOPATHIC NEUTROPENIA

The treatment of scleromyxoedema is notoriously difficult, We present a patient with long-standing diffuse scleromyxoedema associated with f unctional impairment who developed chronic idiopathic neutropenia comp licated by recurrent life-threatening infections, Treatment with recom binant granulocyte-colony stimulating factor led to normalization of t he neutrophil count, prevented further systemic infections, and unexpe ctedly was associated with a striking clinical improvement of her skin disorder and decrease in mucin deposition in the dermis.