Perniones T69.1

Authors: Prof. Dr. med. Peter Altmeyer, Prof. Dr. med. Martina Bacharach-Buhles

Last updated on: 19.03.2023

Synonym(s)

Chilblains; Frostbite; Frost-nip; Perniosis

Definition
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Frequent abnormal tissue reaction occurring in persons with the appropriate disposition, with reversible and very changeable inflammatory skin changes, which can occur even with moderate exposure to cold.

Etiopathogenesis
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Mostly vegetative disturbed vascular function with lack of adaptation to external temperature conditions (external temperatures little above 0 °C, especially in wet cold climate, also in cold damp work, e.g. in butchery professions), but also in local or systemic infections (tuberculosis patients). Of note is the clustering of frostbite-like lesions in COVID-19 infections (Sachdeva M et al. 2021). Such reactions may occur after COVID vaccinations (see also under Chilblain lupus).

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Manifestation
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Occurring mainly in female adolescents or adults with acrocyanosis. According to J.Darier, one is "probably predisposed to chilblains at the age of 5-15 years". Often there is general obesity or pasty habitus. Astonishing is the seasonal accumulation especially in spring and autumn. Occurrence in connection with occupational exposure to cold (butchers, cold storage workers, soldiers) or in homeless people is not rare. Increasingly, frostbite occurs in alpinists.

Localization
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Dorsal sides of the fingers and toes, lower leg, knee area.

Clinical features
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In the interval little conspicuous, laminar, livid redness, which changes to blue-reddish, possibly blue or reddish-brown (purple component), pasty, sometimes painless but also clearly painful plaques or nodules when the temperature changes. The duration of the changes varies, ranging from hours to several days to weeks. Itching or burning may occur when the skin warms up. Blistering and ulceration are possible. S.a. Perniosis follicularis, spring perniosis, autumn perniosis.

Notice! An apparently healthy red complexion can be an expression of chronic cold damage.

Histology
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Capillary dilatation exists in the corium. Edema is detectable in the upper corium. Larger vessels are wall thickened and filled with thrombi. Lymphohistiocytic infiltrates are perivascular.

Differential diagnosis
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Chilblain-Lupus: permanent condition; there is no direct relation to a cold trauma.

Erythema nodosum: highly painful; no relation to an acute cold trauma. Mostly associated with infections.

Sarcoidosis: not related to an acute cold trauma

Erythema exsudativum multiforme: exanthema with shooting-disc-like plaques

Complication(s)
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Secondary infections, ulcerations, scars.

External therapy
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In the acute phase supply of heat and/or hyperemic ointments. In case of strong inflammatory symptoms, short-term potent glucocorticoid extracts such as 0.05% clobetasol cream (e.g. R054, dermoxin), 0.1% mometasone cream (e.g. Ecural), if necessary under occlusion.

Internal therapy
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Systemic therapy can be considered in the case of very pronounced clinical symptoms, e.g. with interval therapy with iloprost (0.5-2.0ng/kgKG/min.
Furthermore, a trial with pentoxifylline (e.g. Trental) 2 times/day 400 mg p.o. can be initiated.

Progression/forecast
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Very variable course; recurrences with exposure to cold. Improvement at a higher age is possible.

Prophylaxis
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Especially in the transitional seasons, protection from cold and wet by wearing suitable clothing, warm footwear! Use of pocket ovens! Active vascular training through sauna, Kneipp cures, underwater massages, alternating warm foot baths, sports.

Note(s)
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Chronic cold damage caused by recurrent pernions may fall under BK number 5101 of the BVK plant.

Literature
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Ferrara G et al (2016) Cold-Associated Perniosis of the Thighs ("Equestrian-Type" Chilblain): A Reappraisal Based on a Clinicopathologic and Immunohistochemical Study of 6 Cases Am J Dermatopathol 38):726-731.
Gomes MM et al (2014) Perniosis. BMJ Case Rep doi: 10.1136/bcr-2014-203732.
Johnson-Arbor K et al (2014) Digital frostbite. N Engl J Med doi: 10.1056/NEJMicm1310126.
Kemper TC et al (2014) Frostbite of both first digits of the foot treated with delayed hyperbaric oxygen:a case report and review of literature. Undersea Hyperb Med 41:65-70
Müller M et al (2007) Recurrent perniosis on the floor of a congenital acrocyanosis. Dermatology in Occupation and Environment 55: 28-34.
Ohatee MA et al (2014) "Salt ice dare": a previously un-described mechanism of rapid frostbite injury. J Plast Reconstr Aesthet Surg 67:e248-249
Sachdeva M et al (2021) A Review of COVID-19 Chilblains-like Lesions and their Differential Diagnosis. Adv Skin Wound Care doi: 10.1097/01.ASW.0000737860.47789.3c.