J Rheum Dis.  2015 Aug;22(4):269-270. 10.4078/jrd.2015.22.4.269.

Childhood Chilblain Lupus Erythematosus

Affiliations
  • 1Department of Dermatology, Hanyang University College of Medicine, Seoul, Korea. drko0303@hanyang.ac.kr
  • 2Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea.

Abstract

No abstract available.


MeSH Terms

Chilblains*

Figure

  • Figure 1. (A) Multiple erythematosus scaly plaques were observed on dorsal aspects of patient's hands. (B) Similarily, his father showed erytheamtous skin lesions on dorsum of hands.

  • Figure 2. Erythematous to pur-puric swollen scaly patches were noted on the ear of the patient (A, B) and his father (C, D).

  • Figure 3. (A) Histopathologic examination revealed interface dermatitis and prominent periap-pendigeal, perivascular and peri-neural lymphohistiocytic infiltrates throughout the entire dermis (H&E, ×40). (B) Lesional direct immunofluorescence results showed granular deposits of immunoglobulin M in the basement membrane zone (×200).


Reference

1. Hedrich CM, Fiebig B, Hauck FH, Sallmann S, Hahn G, Pfeiffer C, et al. Chilblain lupus erythematosus: a review of literature. Clin Rheumatol. 2008; 27:949–54.
2. Viguier M, Pinquier L, Cavelier-Balloy B, de la Salmonière P, Cordoliani F, Flageul B, et al. Clinical and histopathologic features and immunologic variables in patients with severe chilblains. A study of the relationship to lupus erythematosus. Medicine (Baltimore). 2001; 80:180–8.
3. Tüngler V, Silver RM, Walkenhorst H, Günther C, Lee-Kirsch MA. Inherited or de novo mutation affecting aspartate 18 of TREX1 results in either familial chilblain lupus or Aicardi-Goutières syndrome. Br J Dermatol. 2012; 167:212–4.
Article
4. Abe J, Izawa K, Nishikomori R, Awaya T, Kawai T, Yasumi T, et al. Heterozygous TREX1 p. Asp18Asn mutation can cause variable neurological symptoms in a family with Aicardi-Goutieres syndrome/familial chilblain lupus. Rheumatology (Oxford). 2013; 52:406–8.
5. Günther C, Hillebrand M, Brunk J, Lee-Kirsch MA. Systemic involvement in TREX1-associated familial chilblain lupus. J Am Acad Dermatol. 2013; 69:e179–81.
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