Chilblains in immune-mediated inflammatory diseases: a review.

Chilblains were first described over a hundred years ago as cutaneous inflammatory lesions, typically on the digits, occurring on cold exposure. Chilblains can be primary, or secondary to a number of conditions such as infections, including COVID-19, and immune-mediated inflammatory disorders (IMIDs) with SLE being the commonest. Chilblain lupus erythematosus (CHLE) was first described in 1888 as cold-induced erythematous lesions before the terms 'chilblains' or 'perniosis' were coined. Diagnostic criteria exist for both chilblains and CHLE. Histopathologically, CHLE lesions show interface dermatitis with perivascular lymphocytic infiltrate. Immunofluorescence demonstrates linear deposits of immunoglobulins and complement in the dermo-epidermal junction. This narrative review focuses on chilblains secondary to immune-mediated inflammatory disorders, primarily the epidemiology, pathogenesis and treatment of CHLE.

Chilblain Lupus with Nail Involvement: A Case Report and a Brief Overview.

Chilblain lupus erythematosus is a rare, chronic variant of cutaneous lupus erythematosus that occurs during cold or damp periods on the hands, fingers, or feet. It is often associated with other forms of cutaneous lupus and about 20% of patients develop systemic lupus erythematosus. Various medications have been put forward, including steroids, mycophenolate mofetil, calcium channel blockers, and hydroxychloroquine, but the symptoms do not remit completely.

JAK inhibitor ruxolitinib inhibits the expression of cytokines characteristic of cutaneous lupus erythematosus.

This study was stimulated by the clinical observation of a rapid response of a chilblain lupus patient to treatment with JAK1/2-kinase inhibitor ruxolitinib. We investigated the in vivo expression of phospho-JAK2 in CLE skin samples as well as the immunomodulatory in vitro effect of ruxolitinib in cultured immortalized keratinocytes and in a 3D human epidermis model (epiCS). Our results demonstrate that ruxolitinib significantly decreases the production of CLE-typical cytokines (CXCL10, CXCL9, MxA) and might be a promising drug for future clinical studies in patients with CLE and related autoimmune skin diseases.

Chilblain lupus erythematosus in an adolescent girl.

Chilblain Lupus Erythematosus (CHLE) is a rare form of cutaneous lupus erythematosus (LE), more frequently seen in middle aged females. It is characterized by erythematous to violaceous plaques over the acral areas induced by exposure to cold or drop in temperature unlike lesions of lupus erythematosus that worsen with sun exposure. Here, we present a case of chilblain lupus erythematosus in an adolescent girl with few unique features not previously reported.

Chilblain Lupus Erythematosus: A Case Report and Review of Literature / 대한피부과학회지

Chilblain lupus erythematosus (LE) is a rare, chronic form of cutaneous LE (CLE), which presents mostly in women as erythematous to violaceous plaques on the acral areas and face, precipitated by cold and damp climates. It may be accompanied by discoid LE (DLE) lesions or other forms of CLE. Up to 20% of patients develop systemic LE (SLE). Although two missense mutations in TREX1, encoding the 3'-5' repair exonuclease 1, were described in familial chilblain LE, the pathogenesis of sporadic chilblain LE remains unknown. To our knowledge, there are a few reports of chilblain LE in the Korean dermatologic literature. Herein, we present a rare and interesting case of sporadic chilblain LE in 71-year-old man and review the Korean literatures.

A Case of Chilblain Lupus Erythematosus / 대한피부과학회지

Chilblain lupus erythematosus is a special, relatively rare subtype of chronic cutaneous lupus erythematosus. Usually, discoid lesions precede or occur concurrently with perniotic lesions, and about 20% of the patients later develop systemic lupus erythematosus. Herein, we present a 26-year-old female patient who developed erythematous to purplish scaly and crusted papules on the palms, fingers and toes. She also had erythematous hyperkeratotic plaques on the ears and scalp with scarring alopecia that was consistent with discoid lupus erythematosus. Histopathologic finding revealed perivascular and periappendigeal lymphocytic infiltration and fibrin deposition in dermal blood vessels. Therefore, this represents a case of chilblain lupus erythematosus which is a rare subtype and shows typical clinical and histopathologic findings.

A Case of Chilblain Lupus Erythematosus Associated with Antibodies to SSA/Ro / 대한피부과학회지

Chilblain lupus erythematosus (CLE) is a subtype of lupus erythematosus. It is characterized by cutaneous lesions located on the fingers, toes, nose, ears, elbows, heels and knees and is induced or aggravated by exposure to a cold or damp climate. Various laboratory alterations including antinuclear antibody (ANA), anti-dsDNA antibody, anti-SSA/SSB antibody, rheumatoid factor, and cryoglobulin have been reported in CLE patients. Especially, SSA/Ro antibodies may be especially associated with CLE. Approximately 20% of patients presenting with CLE later develop systemic lupus erythematosus (SLE). A 28-year-old man diagnosed with SLE presented with a 2-year history of pruritic erythematous plaques on the ears and dorsa of his hands and feet. The lesions developed or were aggravated the cold weather. In the summer, they were reported to improve, but they did not heal. ANA anti- SSA/SSB antibodies, and anti-dsDNA antibodies were found to be present. He was treated with a topical steroid and advised to avoid the cold.

A Case of Chilblain Lupus Erythematosus / 대한피부과학회지

Chilblain lupus erythematosus (LE) is a chronic unremitting form of LE seen predominantly in women. Chronic facial discoid LE frequently appears before the chilblain form. It may progress to systemic LE more frequently than other forms of chronic cutaneous LE do. We reported a case of chilblain LE in a 28-year-old male. He has had 1-5mm sized scaly erythematous macules on the ear, face, finger and toe tips for 10 years and these lesions have been aggravated in winter. Histopathologic findings were consistent with chilblain LE. The laboratory findings showed antinuclear antibody and anti-ds DNA antibody were positive but cryoglobulin and cold agglutinin were negative. One year later, lupus nephritis developed with leukopenia and decreased C3, C4 levels. He was diagnosed as systemic LE.

A Case of Chilblain Lupus Erythematosus / 대한피부과학회지

Chilblain lupus erythematsus is a rare special subtype of chronic cutaneous lupus erythematosus characterized erythematous acral skin lesion induced by cold, damp climate. Approximately, only 20% of the patients presenting with chilblain lupus erythematosus later develop systemic lupus erythematosus(SLE). We present here a case of chilblain lupus erythematosus, associated with a development of SLE later. A 29-year-old female patient has had violaceous and erythematous papules on the ear, dorsal hands and finger tips for 10 years and these lesions have been aggravated by winter. She has been diagnosed as a suspicious SLE for 3 years and then was treated with oral prednisolone and hydroxychloroqine.

A Case of Chilblain Lupus Erythematosus / 대한피부과학회지

Chilblain lupus erythematosus(CLE) is a chronic unremitting from of LE seen predomin antly in women, The chilblain lesion occurs commonly on the digits, calves and heels. Chronic facial discoid LE usually appears before the chilblain form. A 23-year-old female had relatively well defined, erythematous discoid plaques on her both cheeks and scattered erythema multiforme-like ring lesions on her right hand and right wrist. There were also chilblain lesions showing multiple, purple colored macules on her knees, lower legs and periungual areas of fingers and toes. The skin lesions developed at November, 1982 and then the skin lesions remitted during the next summer. The skin lesions recurred at December, 1983.