A 13-year-old boy presents to the dermatology clinic concerned about the development of red, itchy, and sometimes painful bumps on his fingers. He first noted seeing the lesions 2 months prior, in early December. Although the initial lesions have since resolved, new lesions appeared after a skiing trip the previous weekend. The patient is otherwise healthy, with no significant past medical history, and he does not take any medications. Physical examination reveals erythematous, tender papules on the lateral and dorsal aspects of the distal fingers.
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Perniosis, also known as pernio or chilblains, is an inflammatory skin condition characterized by cold-induced painful erythematous papules on the distal aspect of the fingers or toes. Perniosis may be idiopathic; less commonly, it can be secondary to a systemic disease, such as systemic lupus erythematosus. This is condition is different than, and should not be confused with, lupus pernio, which is a form of cutaneous sarcoidosis.
Perniosis presents as an exaggerated response to cold and wetness in predisposed individuals. It is seen most commonly in Northern Europe and the northern United States,1 particularly in areas with nonfreezing cool temperatures and increased humidity. Symptoms commonly begin in early winter and resolve by spring as cold exposure decreases. However, patients may develop recurrences during subsequent winters or may progress to chronic persistent disease with symptoms often lasting until the month of April. Although patients of any age may be affected, perniosis is most common in young to middle-aged women.2
Most patients presenting with perniosis will report a history of recurrent pruritic and painful bumps on the fingers and/or toes. In addition, many report that their fingertips often look blue or purple in cooler temperatures; some describe numbness and tingling in affected digits.
Clinically, perniosis presents with single or multiple erythematous, dusky papules or nodules that are often edematous and tender on palpation. In more severe cases, ulcerations or necrosis may be present. The disease typically affects the distal and dorsal aspect of phalanges of the fingers and toes. However, the lesions may also appear on heels, lower legs, thighs, nose, and ears. Chronic untreated disease can lead to scarring, depigmentation, and superinfection.3 The lesions typically appear 12 to 24 hours after a cold exposure and often spontaneously resolve within 2 to 3 weeks.
The direct cause of perniosis is cold exposure, including prolonged contact with the wet lining in boots. Factors contributing to disease expression include abnormal vasoconstriction, vasospasm, hyperviscosity, or autoimmunity. Chronic cases may be secondary to underlying disease. For this reason, it is important to rule out other conditions, such as cryoglobulinemia, antiphospholipid antibody syndrome, Raynaud disease, hemolytic anemia, chronic myelomonocytic leukemia, celiac disease, and systemic lupus erythematosus.
Patients with anorexia nervosa are more likely to develop perniosis due to low body mass index and impaired thermoregulation. Additionally, 40% of patients with the genetic disease Aicardi-Goutières syndrome will develop perniosis, with disease often appearing in infancy.4
Results of a skin biopsy of lesional skin help confirm diagnosis. Results of hematoxylin and eosin staining will reveal a T-cell papillary and deep infiltrate with perieccrine reinforcement, associated with dermal edema and necrotic keratinocytes; these are the hallmarks of chilblains of the hands.5
Persistent or atypical cases should undergo laboratory workup to rule out underlying cause. Appropriate tests include complete blood count, erythrocyte sedimentation rate, antinuclear antibody measurement, antiphospholipid antibody panel, and testing for levels of cold-insoluble proteins, including the cryoglobulins, cryofibrinogens, and cold agglutinins. It is important to note that for cold-insoluble protein testing, the blood sample must be kept at 37° C until centrifugation.
The best treatment approach is prevention. Patients should be educated to keep acral areas warm and dry with appropriate protective clothing, including wool socks and protective gloves. In addition, avoidance of nicotine is strongly encouraged.6
Lesions are usually self-limiting and last 2 to 3 weeks. Anecdotal reports suggest the benefit of topical corticosteroids to relieve symptoms. Severe cases may require the use of nifedipine, a calcium channel blocker, and peripheral artery vasodilator. Results of a recent small study showed a clear benefit for the use of pentoxyfilline,7 a xanthine derivative that reduces blood viscosity.
For the patient above, a 3-mm punch skin biopsy taken from lesion skin revealed histologic changes consistent with perniosis. Results of further blood testing to rule out underlying systemic conditions were negative. The patient and his parents were educated regarding the condition and the importance of avoiding exposure to cool and humid temperatures. A prescription for triamcinolone cream was given, to be applied up to 3 times as needed for symptomatic flares.
Esther Stern, NP, is a family nurse practitioner at Advanced Dermatology & Skin Surgery, P.C., in Lakewood, N.J.
- Paller AS, Mancini AJ. Collagen vascular disorders. In: Paller AS, Mancini AJ, eds. Hurwitz Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. 4th ed. Philadelphia, PA: Elsevier Saunders; 2011:497-527.
- Yang X, Perez OA, English JC 3rd. Adult perniosis and cryoglobulinemia: a retrospective study and review of the literature. J Am Acad Dermatol. 2010;62(6):e21-e22.
- Almahameed A, Pinto DS. Pernio (chilblains). Curr Treat Options Cardiovasc Med. 2008;10(2):128-135.
- Abdel-Salam GM, El-Kamah GY, Rice GI, et al. Chilblains as a diagnostic sign of Aicardi-Goutières syndrome. Neuropediatrics. 2010;41(1):18-23.
- Cribier B, Djeridi N, Peltre B, Grosshans E. A histologic and immunohistochemical study of chilblains. J Am Acad Dermatol. 2001; 45(6):924-929.
- James WD, Berger TG, Elston DM. Dermatoses resulting from physical factors. In: James WD, Berger TG, Elston DM, eds. Andrews’ Diseases of the Skin: Clinical Dermatology. 10th ed. Philadelphia, PA: Saunders-Elsevier; 2011:18-44.
- Al-Sudany NK. Treatment of primary perniosis with oral pentoxifylline (a double-blind placebo-controlled randomized therapeutic trial). Dermatol Ther. 2016;29:263-268.