A 39-year-old man presents with a 3-year history of lesions on his fingers and toes that appear following exposure to cold temperatures. He complains of discomfort when wearing shoes, and light palpation is painful. The patient denies a history of smoking, systemic disease, or illicit drug use. Examination reveals edematous, erythematous papules of the affected areas.
Submit your diagnosis to see full explanation.
Pernio, otherwise known as perniosis and chilblains, is a benign, vasospastic, cutaneous eruption of the fingers and toes. It appears following exposure to cold temperatures and dampness. The condition is classified into primary and secondary forms. The primary form is idiopathic and not associated with underlying disease. Secondary forms accompany underlying connective tissue disorders or other pathologic processes such as cryoglobulinemia, lupus erythematosus, chronic myelomonocytic leukemia, and monoclonal gammopathy.1
Pernio presents as painful, red-to-purple edematous papules on the acral surfaces of the fingers and toes. Symptoms typically begin in early winter and resolve by spring. Patients may develop recurrences during subsequent winters or exposure to cold temperatures. Although a benign condition, pernio’s resemblance to other vascular diseases such as peripheral thromboemboli and vasculitis can lead to an extensive and unnecessary workup.2 Females are predominantly affected with an increased risk in those with a low body mass index. Vasospasm is thought to play a role in pathogenesis.3 Histopathology of both primary and secondary pernio reveals lymphocytic and perivascular infiltrates with dermal edema.4
Pernio is best prevented by protecting the hands and feet from cold exposure. Dihydropyridine calcium channel blockers such as nifedipine can be used to prevent recurrence and help promote faster healing.4
Lauren Ax, MSPAS, PA-C, is a physician assistant on staff at the DermDox Center for Dermatology in Hazleton, Pennsylvania,Lawrence Schiffman, DO, is the director of Miami Skin Dr in Doral, Florida, and Stephen Schleicher, MD, is director of the DermDox Center for Dermatology, as well as an associate professor of medicine at Commonwealth Medical College and a clinical instructor of dermatology at Arcadia University and Kings College.
1. Gordon R, Arikian AM, Pakula AS., Chilblains in Southern California: two case reports and a review of the literature. J Med Case Rep. 2014;8:381.
2. Prakash S, Weisman MH. Idiopathic chilblains. Am J Med. 2009;122(12):1152-1155.
3. Shahi V, Wetter DA, Cappel JA, Davis MDP, Spittell PC. Vasospasm is a consistent finding in pernio (chilblains) and a possible clue to pathogenesis. Dermatology. 2015;231(3):274-279.
4. Almahameed A, Pinto DS. Pernio (chilblains). Curr Treat Options Cardiovasc Med. 2008;10(2):128-135.