2020SKIN The Journal of Cutaneous MedicineOpen access

A Case Report of Chilblain Lupus Erythematosus in a Young Male with Literature Review

Azib Shahid, Faiza Jan Iftikhar, Katherine Glaser, Anthony Fernández

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Abstract

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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What this paper is about

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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Available abstract

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.

Key concepts: Medicine, Anti-nuclear antibody, Dermatology, Lupus erythematosus, Systemic lupus erythematosus, Autoantibody, Rheumatoid factor, Family history

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