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Dermatology > ENVIRONMENTAL
Pernio
Article Last Updated: Dec 15, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 10  
Author: Michele S Maroon, MD, Program Director, Department of Dermatology, Geisinger Medical Center
Michele S Maroon is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa
Coauthor(s):
David Hensley, MD, Mullanax Dermatology Associates, Arlington Memorial Hospital
Editors: Craig A Elmets, MD, Director of Dermatology, Departments of Dermatology, Pathology, and Environmental Health Sciences; Professor, The Kirklin Clinic, University of Alabama at Birmingham; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Lester F Libow, MD, Dermatopathologist, South Texas Dermatopathology Laboratory; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
Author and Editor Disclosure
Synonyms and related keywords:
chilblains, perniosis
Background
Pernio is an inflammatory skin condition presenting after exposure to cold as pruritic and/or painful erythematous to violaceous acral lesions. Pernio may be idiopathic or secondary to an underlying disease.
Pathophysiology
Pernio is due to an abnormal vascular response to cold exposure and is most frequent when damp or humid conditions coincide. Minor trauma also may predispose the acral parts to symptomatic lesions in otherwise appropriate weather conditions. The response to vasodilator drugs varies. Keeping acral areas warm and dry best prevents pernio.
Frequency
United States
The true incidence is unknown, as pernio frequently is unrecognized or misdiagnosed.
International
Rates of pernio vary with climate. England, with its cool damp climate, has an annual incidence of 10%.
Mortality/Morbidity
Most cases resolve without any adverse reactions.
Sex
Women are affected more frequently than men.
Age
Pernio is most frequent in young and middle-aged women and in children.
History
Most patients present with a history of recurrent painful and/or pruritic, erythematous, violaceous papules or nodules on the fingers and/or toes. Most cases resolve within 2-3 weeks. Elicit a history of cold exposure or repeated episodes of cold exposure.
Physical
Pertinent findings are limited to the skin. Cutaneous lesions present 12-24 hours after cold exposure as red or violaceous macules, papules, nodules, or plaques, which may form vesicles or ulcerate. They occur on acral areas, are associated with burning or pruritus, and last 1-3 weeks.
Causes
The direct cause of pernio is cold exposure. Chronic pernio may be secondary to various systemic diseases as follows:
- Chronic myelomonocytic leukemia
- Anorexia nervosa
- Dysproteinemias
- Macroglobulinemia
- Cryoglobulinemia, cryofibrinogenemia, cold agglutinins
- Antiphospholipid antibody syndrome
- Raynaud disease
- Variants
- Kibes (equestrian cold panniculitis): Erythrocyanotic plaques occur on the upper lateral thighs of women who ride horses. Histology is characterized by an intense perivascular infiltrate extending into subcutaneous fat.
- Chilblain lupus erythematosus: Violaceous "pernio" plaques appear prominent over dorsal interphalangeal joints, often with positive antinuclear antibody (ANA) or rheumatoid factor (RF). Histologic and immunofluorescent evidence of lupus is present in the skin lesions. Half of the patients have associated facial discoid lupus lesions, and 15% develop systemic lupus.
- Drug induced: Sulindac induced cases have been reported.
Erythema Multiforme
Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
Sarcoidosis
Other Problems to be Considered
Acrocyanosis
Emboli (septic or cholesterol)
Erythromelalgia
Ischemia
Polycythemia vera
Purple toe syndrome secondary to coumarin
Raynaud phenomenon
Trauma
Lab Studies
- CBC count and sedimentation rate should be obtained to rule out associated leukemia.
- Antiphospholipid antibody panel: Review of patients presenting with pernio shows an increased incidence of antiphospholipid antibody syndrome.
- Cryoglobulins, cryofibrinogen, and cold agglutinins generally are absent but should be considered as part of the laboratory evaluation in a patient with chronic pernio. Because of occasional false-negative cryoprecipitate screening results, consider hepatitis C antibody screening or even RF as a marker for cryoglobulinemia in select cases.
- ANA: Pernio lesions can occur in the setting of lupus erythematosus.
- Serum protein electrophoresis (SPEP) and quantitative immunoglobulins: Dysproteinemias and macroglobulinemia, causing increased serum viscosity, may be associated with pernio.
Histologic Findings
A clinical diagnosis can often be made. Biopsy may be indicated to rule out other inflammatory processes in difficult chronic cases. Punch biopsy is adequate. There is variable epidermal spongiosis or necrosis. Intense papillary dermal edema is present. A superficial and deep perivascular lymphocytic infiltrate is seen, with the described "fluffy edema" of vessel walls. Lymphocytic vasculitis may be present.
Medical Care
- Prophylactic warming of acral areas, achieved by heat and appropriate clothing, best prevents pernio.
- Ultraviolet light, given at the beginning of the cold, damp season, has been touted as preventing outbreaks of pernio in prone individuals. Pathogenesis was loosely based on damaging the minute vessels and minimizing their ability to vasoconstrict with subsequent cold exposure. However, in at least one double-blind study, ultraviolet therapy was of no value in prophylaxis of pernio.
Consultations
- Consult a dermatologist for diagnosis and evaluation of associated disease.
Diet
- Thin body habitus may be associated with heightened cutaneous vasoreactivity; the health care provider needs to be aware of this population at risk and its possible need for nutritional intervention.
Activity
- Prophylactic warming of acral areas with minimization of cold exposure may prevent disease recurrence.
The use of topical and systemic steroids, vasodilators, IV calcium followed by IM vitamin K, and ultraviolet B radiation has been anecdotally reported in the literature. In most cases, the value of these agents is questionable and often disputed.
Drug Category: Calcium channel blockers
Peripheral arterial vasodilators may be effective in the treatment and prevention of pernio.
| Drug Name | Nifedipine (Procardia) |
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| Description | Relaxes coronary smooth muscle and produces coronary vasodilation, which in turn improves myocardial oxygen delivery. Small studies have shown this drug to be effective in reducing symptoms associated with severe recurrent pernio. Currently is considered DOC. |
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| Adult Dose | 10-20 mg PO tid |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; excessive hypotension, CHF, and coronary artery disease |
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| Interactions | Caution with coadministration of any agent that can lower BP, including beta-blockers and opioids; digoxin, coumarin, cimetidine, ranitidine, and quinidine also may affect effects of nifedipine |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | May cause lower extremity edema; allergic hepatitis is rare; hypotension, peripheral edema secondary to arterial vasodilation, rare exacerbation of angina, or acute MI (especially in patients with severe obstructive coronary artery disease) may occur |
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Deterrence/Prevention
- Avoid exposure to cold.
- Cease smoking.
Complications
- Pernio lesions that blister may become secondarily infected.
Prognosis
- Prognosis is good.
- Recurrences may be observed annually with onset of cold weather.
- Long-term follow-up of patients with chronic recurrent pernio is advised because this may reveal connective-tissue disease (lupus erythematosus).
Patient Education
- Avoid exposure to cold.
- Keep extremities warm and dry.
- Cease smoking.
| Media file 1:
Erythematous macules on distal toes of a 6-month-old girl with pernio. |
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Media type: Photo
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| Media file 2:
Close-up of erythematous macules and plaques on distal plantar toes. |
 | View Full Size Image | |
Media type: Photo
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| Media file 3:
A 63-year-old man with pernio presenting as acral violaceous plaques with bullae. |
 | View Full Size Image | |
Media type: Photo
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Pernio excerpt Article Last Updated: Dec 15, 2006
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