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Chilblain, pernio, cutaneous lupus erythematosus, raynaud’s phenomenon, CHLE
Chilblain lupus erythematosus (CHLE), also known as Hutchinson lupus, is rare form of chronic cutaneous lupus erythematosus. The diagnosis is made in patients with clinical findings of chilblains in conjunction with the clinical or laboratory features of cutaneous or systemic lupus erythematosus (SLE). Similar to idiopathic chilblains (or perniosis), CHLE presents with tender, reddish-blue papules, nodules, or plaques on the toes, fingers, nose, or ears which are precipitated by cold exposure. There is a variation in lab findings that become positive in these patients ranging from a positive Antinuclear antibody (ANA), Rheumatoid factor (RF), SSA/Ro autoantibodies. Anemia, hypocomplementemia, other autoantibodies and elevated ESR have also been described in numerous patients Sporadic disease commonly affects middle-aged women whilst familial disease manifests in childhood. We report herein, a case of CHLE in a young male with no family history of lupus and a negative ANA on initial evaluation. There is little in the published literature on CHLE. This case report serves to revisit the diagnosis of CHLE and review the existing literature. Clinicians should understand the importance of early diagnosis and prompt treatment initiation in order to reduce associated morbidity and possible disfigurement.
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