Familial cold urticaria. Investigation of a family and response to stanozolol.
BACKGROUND
Familial cold urticaria is a rare cutaneous and systemic reaction to cold with autosomal dominant inheritance, distinctive clinical features, and unknown pathogenesis. Release of a chymotrypsinlike substance has been postulated. To date, no effective treatment has been reported.


METHODS
Eight cases from a large family pedigree are described. Three members showed a very favorable response in their cold urticaria, when treated with stanozolol; the response was reproducible. Histologic examination of an early lesion in one case revealed evidence of mast cell degranulation.


CONCLUSIONS
The biochemical observations are probably secondary epiphenomena. Correction of a deficiency of an inhibitory protein is a possible mechanism of action of stanozolol as in hereditary angioedema.
Ormerod
A D
AD
Department of Dermatology, University of Aberdeen, United Kingdom.
Smart
L
L
Reid
T M
TM
Milford-Ward
A
A
D000328:Adult; D003080:Cold Temperature / Q000009:adverse effects*; D004950:Esterases / Q000037:antagonists & inhibitors; D005260:Female; D006801:Humans; D008875:Middle Aged; D010375:Pedigree; D013197:Stanozolol / Q000627:therapeutic use*; D014581:Urticaria / Q000097:blood / Q000188:drug therapy* / Q000209:etiology* / Q000235:genetics
D013197:Stanozolol; D004950:Esterases