Familial cold urticaria
| Cold urticaria | |
|---|---|
 |
|
| Allergic urticaria on leg in the form of hives induced by cold. | |
| Classification and external resources | |
| Specialty | dermatology |
| ICD-10 | L50.2 (ILDS L50.210) |
| ICD-9-CM | 708.2 |
| OMIM | 120100 |
| DiseasesDB | 4688 |
| Familial cold urticaria | |
|---|---|
| Classification and external resources | |
| Specialty | dermatology |
| ICD-10 | L50.2 (ILDS L50.220) |
| OMIM | 120100 |
| MeSH | D056587 |
Cold urticaria (essentially meaning "cold hives") is a disorder where hives (urticaria) or large red welts form on the skin after exposure to a cold stimulus. The welts are usually itchy and often the hands and feet will become itchy and swollen as well. Hives vary in size from about 7mm in diameter to as big as about 27mm diameter or larger. The disease is classified as chronic when hives appear for longer than 6 weeks; they can last for life, though their course is often unpredictable. This disorder, or perhaps two disorders with the same clinical manifestations, can be inherited (familial cold urticaria) or acquired (primary acquired cold urticaria). The acquired form is most likely to occur between ages 18–25, although it can occur as early as 5 years old in some cases.
Cold urticaria may be divided into the following types:
Primary cold contact urticaria is a cutaneous condition characterized by , and occurs in rainy, windy weather, and after swimming in cold water and after contact with cold objects, including ice cubes.
Secondary cold contact urticaria is a cutaneous condition characterized by , due to serum abnormalities such as cryoglobulinemia or cryofibrinogenemia are extremely rare, and are then associated with other manifestations such as Raynaud's phenomenon or purpura.
Reflex cold urticaria is a cutaneous condition in which generalized cooling of the body induces widespread .
Familial cold urticaria (also properly known as familial cold autoinflammatory syndrome, FCAS) is an autosomal dominant condition characterized by rash, conjunctivitis, fever/chills and arthralgias elicited by exposure to cold - sometimes temperatures below 22 °C (72 °F).
It has been mapped to CIAS1 and is a slightly milder member of the disease family including Muckle–Wells syndrome and NOMID. It is rare and is estimated as having a prevalence of 1 per million people and mainly affects Americans and Europeans.
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