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March 1993

Familial Cold Urticaria: Investigation of a Family and Response to Stanozolol

Author Affiliations

From the Departments of Dermatology (Dr Ormerod), Pathology (Dr Smart), and Microbiology (Dr Reid), University of Aberdeen; and Protein Reference Laboratory, Department of Immunology, Royal Hallamshire Hospital, Sheffield (Dr Milford-Ward), United Kingdom.

Arch Dermatol. 1993;129(3):343-346. doi:10.1001/archderm.1993.01680240083012

• 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.

Observations.—  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.(Arch Dermatol. 1993;129:343-346)

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