Chilblain Lupus erythematosus: Report of 15 Cases

Abstract

In this retrospective study, the authors describe the clinical, histologic and laboratory features of 15 cases of chilblain or perniotic lupus. In winter, the patients (14 women, 1 man) develop chilblain-like lesions, chiefly in the toes (8 times) and fingers (11 times). Histologic features are identical to those of discoid lupus erythematosus. The damaged skin gives a positive fluorescent band test. Usually, these lesions occur in association with discoid lupus of the face. However, in 8 patients, they were the only cutaneous sign of lupus. This form of lupus can evolve to a systemic form, as was the case with 3 patients.

Familial Chilblain Lupus – A Monogenic Form of Cutaneous Lupus Erythematosus due to a Heterozygous Mutation in <i>TREX1</i>

“…Thus, a low body mass index as well as pathological capillary changes observed in this patient may have played a role during exacerbation of chilblain lupus [20]. In fact, precipitation of chilblain lupus by anorexia in an adolescent male has recently been reported [21].…”

“…The therapy of chilblain lupus primarily includes protection from cold [25] and improvement of the peripheral blood flow with ASA or nifedipine [20, 26]. Topical steroids may be of benefit in some patients [27].…”

Kutaner Lupus erythematodes

“…Be vor zugt wer den die dor sa len und mar gi na len Berei che der Fin ger be fal len, aber auch die un te ren Ex tre mi tä ten, ins be son de re die Ze henkup pen und Fer sen so wie die Oh ren und die Nase kön nen be trof fen sein [14,66]. Be sonders in der feucht kal ten Jah res zeit oder nach ei nem Tem pe ra tu r ab fall kommt es zu symme tri schen, druck do len ten, li vi dro ten Schwel lun gen, die in große, um schrie be ne, pols terar ti ge Kno ten über ge hen und zent ral ero siv wer den und ul ze rie ren kön nen (.…”

“…In be fal le ner Haut fin den sich meistens Ab la ge run gen von Im mun glo bu li nen und Komple ment ent lang der Ba sal mem bran zo ne und auch pe ri vas ku lär; der Lu pus band test in unbe fal le ner, licht ge schütz ter Haut ist hin ge gen in der Re gel ne ga tiv [14,66].…”

Adult perniosis and cryoglobulinemia: A retrospective study and review of the literature

“…A review of the literature noted an additional 65 cases of perniosis (idiopathic and CLE) in which serum cyroglobulins were studied. [1][2][3][4] Only two of 79 (2.5%) patients were found to have cryoglobulinemia. 1 Limitations to our study are that different laboratories were used to determine cryoglobulin levels and that our study is retrospective in nature.…”