Physical Address

304 North Cardinal St.
Dorchester Center, MA 02124

Pernio (Chilblains)


Pernio , commonly known as chilblains , is a cold-induced localized inflammatory condition presenting as skin lesions predominantly on unprotected acral areas. Typically there is swelling of the dorsa of the proximal phalanges of fingers and toes ( Fig. 49.1 ). Pernio is a Latin term meaning “frostbite.” Chilblains is an Anglo-Saxon term used in older literature and means “cold sore.” The tissue and vascular damage is less severe in pernio than in frostbite, in which the skin is actually frozen. The numerous names that were used to describe this syndrome created much confusion and misunderstanding of this entity ( Box 49.1 ). In the mid-1800s, there were attempts to better classify the disease and in 1894, Corlett was the first to describe the clinical characteristics of pernio, which he called dermatitis hiemalis .

Fig. 49.1
Typical changes of pernio on dorsal portion (A) and pads of toes (B). Distribution around nail beds (A) and swollen toes with brownish yellow and red lesions (B) are characteristic of pernio. At this stage, affected extremities often itch and burn.

Box 49.1
Different Names Used in the Literature to Describe Pernio or Pernio-Like Conditions

  • Pernio

  • Chilblains

  • Nodular vasculitis

  • Erythrocyanosis

  • Erythrocyanose sur malléolair

  • Erythema induratum

  • Lupus pernio

  • Kibes

  • Perniosis

  • Dermatitis hiemalis

  • Frostschaden

  • Erythrocyanosis frigida

  • Erythrocyanosis crurum puellaris

  • Bazin disease

  • L’engelune

  • Cold panniculitis

Epidemiology

The first epidemiological study to explicate the prevalence of chilblains and its impact on productivity in servicewomen was carried out in 1942 by the US Medical Department of the War Office. The study concluded that at least 50% of questionnaire participants had chilblains by age 40 during World War II (1939-1943). Although pernio is most common in young women, it has also been reported in all ages and both sexes. The number of reported cases of pernio is higher during times of wet near-freezing weather, and less common in dry freezing weather or in a bitterly cold climate. Pernio is most commonly encountered in the northern and western parts of the United States; isolated cases have been reported in warmer climates in times of cooler damp weather.

As shown in a cross-sectional study conducted by the US Army, the yearly rate of cold weather injuries declined from 38.2/100,000 in 1985 to 0.2/100,000 in 1999. This and other observations from clinical practice suggest that the disease is becoming less common with higher standards of home and workplace heating and greater use of appropriate clothing during the cold winter months.

Pathophysiology

The first response to cold exposure is vasoconstriction in the dermis and subcutaneous tissue. Heat loss is minimized by shutting down distal capillary beds and diminishing blood supply to the acral portions of the extremities to maintain central body temperature. Stasis and shunting of blood flow away from the superficial vessels occurs secondary to arteriolar constriction, venular relaxation, and cold-associated increased blood viscosity. The result of these changes is superficial tissue anoxia and ischemia. The arteriolar vasoconstriction described in pernio has been demonstrated in pathological and radiographic studies. Female predominance may be related to increased responsiveness of their cutaneous circulation to cold. Indeed, there is a higher frequency of vasomotor instability, cold hands and feet, and Raynaud phenomenon in women.

Humidity has an important role in the pathophysiology of pernio because it enhances air conductivity, promoting heat loss from the skin. Most individuals tolerate exposure to nonfreezing damp cold, but others may experience pernio, Raynaud phenomenon, acrocyanosis, or cold urticaria. The clinical manifestations of cold injuries are related to duration, severity, and dampness of cold exposure as well as the individual’s underlying predisposition to cold injury and the stage at which medical attention was sought. The exposed skin of affected subjects remains cool longer and warms slower than that of controls, further highlighting the importance of individual susceptibility for development of pernio after cold exposure. The increased incidence of pernio among relatives of affected patients suggests the possibility of genetic or familial predisposition. Several other conditions have been proposed to promote vulnerability to the disease ( Box 49.2 ).

Box 49.2
Categories of Diseases Associated with Pernio

Defective Cutaneous Vasomotor Reactivity

  • Raynaud phenomenon

  • Acrocyanosis

  • Complex regional pain syndrome (reflex sympathetic dystrophy [RSD])

  • Anterior poliomyelitis

  • Syringomyelia

  • Livedo reticularis

  • Erythromelalgia

You're Reading a Preview

Become a Clinical Tree membership for Full access and enjoy Unlimited articles

Become membership

If you are a member. Log in here

Related Posts