Last Updated: 2020-06-24

Author(s): Anzengruber F., Navarini A.

ICD11: EB51.Y

Hutchinson 1888.

Chilblain lupus erythematosus.

Rare variant of cutaneous lupus erythematosus, whose skin changes are reminiscent of frostbite (= chilblain). Caution: Do not confuse with lupus pernio, which is a variant of sarcoidosis!

  • < 100 cases described.
  • So far, almost only with women.
  • Occurs at low temperatures.

The exact cause is unknown. There is at least one genetic component, since family forms of "chilblain lupus" are described.

Livid pressure umbels, partly hyperkeratotic, sometimes hyperesthetic, cushion-like swellings, plaques and knots. Central atrophy, erosion or ulcers can occur. 

  • S. Cutaneous lupus erythematosus.
  • Note:
    • Frequently increased: γ-Globulins, ANAs, ds-DNA-Ak, antiphospholipid-Ak, anti SSA/Ro, BSG, pos. rhesus factor.
    • Association with SLE in approx. 20%.

Areas exposed to cold.

Orthohyperkeratosis, atrophy of the surface epithelium, perivascular lymphocellular infiltrates, intraluminal fibrin deposits.

Transition to an SLE.

Often chronic.

  1. Millard LG, Rowell NR. Chilblain lupus erythematosus (Hutchinson) A CLINICAL AND LABORATORY STUDY OF 17 PATIENTS. Br J Dermatol 1978;98:497-506.
  2. Doutre MS, Beylot C, Beylot J, Pompougnac E, Royer P. Chilblain Lupus erythematosus: Report of 15 Cases. Dermatology 1992;184:26-8.
  3. Kanwar AJ, Ghosh S, Dhar S. Chilblain Lupus erythematosus and Lupus pernio &ndash; the same Entity? / Erratum. Dermatology 1992;185:160-.
  4. Franceschini F, Calzavara-Pinton P, Quinzanini M, et al. Chilblain lupus erythematosus is associated with antibodies to SSA/Ro. Lupus 1999;8:215-9.
  5. Lee-Kirsch MA, Gong M, Schulz H, et al. Familial Chilblain Lupus, a Monogenic Form of Cutaneous Lupus Erythematosus, Maps to Chromosome 3p. The American Journal of Human Genetics 2006;79:731-7.
  6. Rice G, Newman WG, Dean J, et al. Heterozygous Mutations in TREX1 Cause Familial Chilblain Lupus and Dominant Aicardi-Goutières Syndrome. The American Journal of Human Genetics 2007;80:811-5.