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.
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.
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.(Arch Dermatol. 1993;129:343-346)
Ormerod AD, Smart L, Reid TMS, Milford-Ward A. Familial Cold Urticaria: Investigation of a Family and Response to Stanozolol. Arch Dermatol. 1993;129(3):343–346. doi:10.1001/archderm.1993.01680240083012
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