Awareness * Advocacy * Research

Familial Atypical Cold Urticaria

Familial Atypical Cold Urticaria (ACU) is usually a self-limited, sporadic, cutaneous disease diagnosed based on history and a positive cold stimulation time test (CSTT) result.

Three unrelated families with lifelong atypical cold urticaria distinguished from ACU and familial cold autoinflammatory syndrome displayed an autosomal dominant pattern of inheritance. All tested subjects had negative CSTT results. Revealed lifelong symptoms beginning in early childhood with pruritus, erythema, and urticaria after cold exposure. Angioedema and syncope, near syncope, were also present.

Triggers included cold atmosphere, aquatic activities, handling cold objects, and ingestion of cold foods or beverages. Skin biopsy specimens demonstrated a mast cell infiltrate with the appearance of degranulation after cold challenge.

FACU is a new cold-induced inherited disease that is different than ACU in its natural history, atmospheric cold elicitation, severity of systemic reactions, and CSTT results. FACU differs from familial cold auto-inflammatory syndrome in symptom timing and the absence of fever, chills, and joint pain. The cause is suspected to be mast cell related. Treatment of reactions is similar to that for ACU. Further evaluation of pathogenesis and genetics is warranted.

FACU is a new cold-induced inherited disease that is different than ACU in its natural history, atmospheric cold elicitation, severity of systemic reactions and Cold Stimulation Timed Test results. FACU differs from FCAS in symptom-timing and the absence of fever, chills and joint pain. The etiology is suspected to be mast cell-related. Treatment of reactions is similar to ACU. Further evaluation of pathogenesis and genetics is warranted.

Links to external websites for further research:
http://www.ncbi.nlm.nih.gov/pubmed/19910034
ainotes.wikispaces.com/file/view/FACU.pdf

 

 

Nuggets of Wisdom

Yes, there is such a thing as having an allergic type reaction to temperatures. This is defined as a physical urticaria. Other physical urticarias include Solar, Aquagenic, Pressure, Vibration and Exercise.

Most reactions are pseudo-allergic. By definition, an allergy involves inhaling or consuming an allergen. Physical urticarias have no known allergen. Despite the terminology and medical definitions, systemic reactions can be life threatening.

Cold has an arbitrary definition based on an individual feeling. For a person with a cold urticaria, cold can be defined as any temperature cooler than their own body temperature.

You do not have to be cold to have a reaction to the cold; contact with cold can trigger a reaction.

You can have an allergic type reaction to both cold and heat simultaneously.

Most reactions considered anaphylactic are really anaphylactoid by definition.

Moving to a warmer climate as a treatment for Cold Urticaria is a myth. Warmer climates present their own issues for those with Cold Urticaria.

Translate »