Perniotic Lupus

A 9-year-old African American female was referred to dermatology for a history of recurring painful, red-purple bumps and blisters on her hands, feet and nose in the winter. She tried topical hydrocortisone 1% cream without improvement, though noted that the previous year she had similar lesions that resolved in the summertime. Family history was notable for lupus and Sjogren’s disease on her maternal side. On the nose and fingertips there were erythematous-to-violaceous papules and plaques. There was fissuring and scaling of the lips and some toes. All toenails and some fingernails were dystrophic with transverse band-like depressions and ragged cuticles. Punch biopsy from a red-purple papule on the right 5th finger revealed perivascular and periadnexal lymphocytic infiltrates within the superficial and deep dermis, papillary dermal edema, and vacuolar lichenoid infiltrate, consistent with perniotic lupus. Perniotic lupus is an erythrocyanotic form of chronic cutaneous lupus erythematosus in which patients have characteristic clinical findings in addition to other features of cutaneous or systemic lupus. The latter differentiates this condition from pernio (i.e. Chillblains). It is important to note that perniotic lupus is also distinct from lupus pernio, which is a form of cutaneous sarcoidosis. Typical lesions of perniotic lupus are edematous red-purple papules, plaques, or nodules that develop in response to cold exposure. They occur most commonly on the dorsal digits however the nose, ears, legs, buttocks, and plantar surfaces can also be affected. Patients often experience pruritus, pain, or a burning sensation and may also develop overlying blisters or ulceration. Perniotic lupus tends to affect young and middle-aged women, however it can also occur in children and men. The pathophysiology is thought to involve an aberrant inflammatory response to cold, triggered by vasoconstriction-induced hypoxemia. Autoantibody-mediated endothelial cell damage and hyperviscosity are also likely contributing factors. The condition is often recurrent with onset in the winter and improvement in the spring and summer months. Treatment therefore primarily focuses on limiting exposure of affected areas to moist, cold environments. Patients should be encouraged to wear well-insulated clothing, gloves, and socks. They may also benefit from medium-to-high potency topical steroids, prednisone, anti-malarials such as Hydroxychloroquine, calcium-channel blockers such as Nifedipine, and Nitroglycerin paste.https://scholarlycommons.henryford.com/merf2019caserpt/1019/thumbnail.jp