Erdheim-Chester disease, A fatal xanthelasma-like lesion

Authors

  • Patteera Patrapaisarnsin DIVISION OF DERMATOLOGY, DEPARTMENT OF MEDICINE, PHRAMONGKUTKLAO HOSPITAL, BANGKOK, THAILAND.
  • Kobkul Aunhachoke DIVISION OF DERMATOLOGY, DEPARTMENT OF MEDICINE, PHRAMONGKUTKLAO HOSPITAL, BANGKOK, THAILAND.

Keywords:

Erdheim-Chester disease, Histiocytosis, Xanthelasma-like lesion

Abstract

Erdheim-Chester disease is a rare, non-Langerhans histiocytosis characterized by chronic histiocytic infiltration of multiple organs. There are fewer than five hundred cases reported. Our case presented with headache, diplopia and knee pain. He had a year of progressively periorbital xanthelasma-like lesion as a presenting dermatologic manifestation.

References

Diamond EL, Dagna L, Hyman DM, et al. Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood 2014; 124: 483-92.

Volpicelli ER, Doyle L, Annes JP, et al. Erdheim-Chester disease presenting with cutaneous involvement: a case report and literature review. J Cutan Pathol 2011; 38: 280–85.

Allen TC, Chevez-Barrios P, Shetlar DJ, Cagle PT. Pulmonary and ophthalmic involvement with Erdheim-Chester disease: a case report and review of the literature. Arch Pathol Lab Med 2004; 128: 1428–31.

Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, et al. Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases. Medicine (Baltimore) 1996; 75: 157–69.

Baykal C, Polat Ekinci A, Yazganoglu KD, Buyukbabani N. The clinical spectrum of xanthomatous lesions of the eyelids. Int J Dermatol 2017; 56: 981-92.

Sanchez JE, Mora C, Macia M, Navarro JF. Erdheim-Chester disease as cause of end-stage renal failure: a case report and review of the literature. Int Urol Nephrol 2010; 42: 1107–12.

Arnaud L, Hervier B, Neel A, et al. CNS involvement and treatment with interferon-alpha are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients. Blood 2011; 117: 2778–82.

Haroche J, Cohen-Aubart F, Emile JF, et al. Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation. Blood 2013; 121: 1495–500.

Hervier B, Arnaud L, Charlotte F, et al. Treatment of Erdheim-Chester disease with long-term high-dose interferon-alpha. Semin Arthritis Rheum 2012; 41: 907–13.

Downloads

Published

2019-10-01

How to Cite

Patrapaisarnsin, P., & Aunhachoke, K. (2019). Erdheim-Chester disease, A fatal xanthelasma-like lesion. Thai Journal of Dermatology, 35(3), 128–132. Retrieved from https://he02.tci-thaijo.org/index.php/TJD/article/view/165562