Early onset chilblain lupus erythematosus with atypical presentation in an Indian girl

Neerja Puri, Anjugam Alagappan, Balvinder Kaur Brar


Chilblain lupus erythematosus (Chilblain LE) is a rare form of lupus erythematosus with only 70 cases reported worldwide with most of the patients in middle age and very few cases in adolescent age group. Herein, we are reporting a 16 years old girl presented with an early age of onset at 10 years with atypical presentation who fulfilled Mayo clinic diagnostic criteria for Chilblain LE.


Chilblain lupus, Atypical, Early onset

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Zajac N, Markiewicz A, Chuchla-Szczupacka K, Owczarczyk-Saczonek A, Placek W. Chilblain lupus erythematosus- a case report. Dermatology Review/Przeglad Dermatologiczny. 2015;102:14 -8.

Hutchinson J. Harveian lectures on lupus, lecture III: on the various forms of lupus vulgaris and erythematosus. Brit Med J. 1888; 1:113-8.

Gunther C, Meurer M, Stein A, Viehweg A, Lee-Kirsch MA. Familial chilblain lupus-A monogenic form of cutaneous lupus erythematous due to a heterozygous mutation in TREX1. Dermatology 2009;219:162-6.

Su WP, Perniciaro C, Rogers RS, White JW. Chilblain lupus erythematosus (lupus pernio): clinical review of the Mayo Clinic experience and proposal of diagnostic criteria. Cutis. 1994;2013:395-9.

Hedrich CM, Fiebig B, Hauck FH, Sallmann S, Hahn G, Pfeiffer C, et al. Chilblain lupus erythematosus-a review of literature. Clin Rheumatol. 2008;2013:949-54.

Boehm I. Chilblain lupus erythematosus Hutchinson: successful treatment with mycophenolate mofetil. Arch Dermatol. 2001;137:235-6.

Al-Sudany NK. Treatment of primary perniosis with oral pentoxifylline (a double-blind placebo-controlled randomized therapeutic trial). Dermatol Ther. 2016;29:263.

Viguier M, Pinquier L, Cavelier-Balloy B, De la Salmoniere 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.