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NF03.0
1.3.2 Pernio
Grading & Level of Importance: A
ICD-11
Synonyms
Dermatitis congelatio escharotica; Chilblains; perniosis
Epidemiology
Congelatio quite frequent in winter sport activities, mountain tracking; perniones mostly appear in spring and autumn around 0ºC degree. Often in adolescents with acrocyanosis (horse riding, long time cycling, inappropriate shoes, wet socks).
Definition
Congelatio: 1st to 3rd degree with maximum of irreversible necrosis or death. Perniones: Cold-induced blue-red indurated papules or nodules, usually on legs or feet. F > M.
Aetiology & Pathogenesis
Congelatio dependent on temperature grade and exposure time. Persistent exposure to cold, damp conditions predisposes to development of pernio over days to weeks.
Signs & Symptoms
In congelatio in grade 1 erythematous lesions, in grade 2 bullous lesions and grade 3 necrosis with irreversible sequelae. In perniones blue-red oedematous papules or nodules, sometimes quite tender or painful.
Localisation
Typically occur at acral sites and in areas which are easily cooled (especially the shins, fingers, toes and other thighs in equestrians). Dampness is also important and some professions are at increased risk, such as butchers and farmers. In congelatio depending if local or full body cold exposure.
Classification
Congelatio: Grade 1 – 3.
Laboratory & other workups
None.
Dermatopathology
Congelatio: depending of degree vessel narrowing, blistering, apoptosis and necrosis.
Perniones: severe courses show blistering, edema, hyperemic vessels and lymphocytic vasculitis.
Course
Congelatio: depending on length of injury in part reversible or irreversible.
Perniones: Recurrent, more common in spring and autumn (cool, moist climatic conditions).
Complications
Congelatio: longterm downcooling death.
Perniones: Rarely ulceration.
Diagnosis
History, localization, clinical features.
Differential diagnosis
Lupus pernio (sarcoidosis), chilblain lupus (lupus erythematosus).
Prevention & Therapy
Congelatio: slow warming up of central temperature, no direct warming of involved lesions. Hyperbaric oxygenation to avoid amputation, prostaglandin infusions.
Perniones: warm, water-resistant protective clothing, topical or oral vasodilators (e.g. calcium channel blockers).
Special
Severe congelatio to special centers and intensive care.
Differential Diagnosis
Podcasts
Tests
- Which dermatosis is a differential diagnosis to pernio?
- What is needed to diagnose pernio?
- Which therapeutic measures are appropriate in pernio?
- The most important therapeutic measure for pernio is avoiding cold and moisture
- Chilblains or pernio are
- Which of these are part of the differential diagnostic considerations in pernio?
- Which are needed to diagnose pernio?
- Which therapeutic measures are appropriate in pernio?
- Statement 1 Pernio or chilblains are acral lesions found in cutaneous sarcoidosis
- Statement 1 The most important therapeutic measure for pernio is avoiding cold and moisture
- Which treatment is appropriate for pernio?
- Which location is unusual for pernio?
- Causes of pernio or chilblains include:
- Chilblains or pernio are
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