Beneficial effect of granulocyte-colony stimulating factor in scleromyxoedema associated with severe idiopathic neutropenia.
The treatment of scleromyxoedema is notoriously difficult. We present a patient with long-standing diffuse scleromyxoedema associated with functional impairment who developed chronic idiopathic neutropenia complicated by recurrent life-threatening infections. Treatment with recombinant granulocyte--colony stimulating factor led to normalization of the neutrophil count, prevented further systemic infections, and unexpectedly was associated with a striking clinical improvement of her skin disorder and decrease in mucin deposition in the dermis.
Cohen
A M
AM
Hematology Unit, Golda Medical Center, Israel.
Hodak
E
E
David
M
M
Mittelman
M
M
Gal
R
R
Stern
R
R
D002908:Chronic Disease; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D008875:Middle Aged; D017520:Mucinoses / Q000473:pathology / Q000628:therapy*; D009503:Neutropenia / Q000628:therapy*; D011994:Recombinant Proteins / Q000627:therapeutic use; D012595:Scleroderma, Systemic / Q000473:pathology / Q000628:therapy*
D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor