Acrodermatitis continua of Hallopeau (ACH) is a rare, chronic, sterile, pustular eruption that predominantly affects the fingertips with nail involvement. While some consider ACH a distinct entity, many believe it to be a variant of pustular psoriasis, especially as cases of ACH progressing to generalized pustular psoriasis (GPP) have been reported. Recently, recessively inherited mutations in the IL36RN gene, which encodes interleukin-36 receptor antagonist (IL-36Ra), have been demonstrated to be the cause of familial GPP, a condition termed DITRA (deficiency of IL-36Ra). Here, we identified a homozygous missense mutation c.338C>T (p.Ser113Leu) in the IL36RN gene in a male patient with ACH, as well as in his sister who had a history of GPP.

Hallopeau MH: Sur une asphyxie locale des extrémités avec polydactylite suppurative chronique et poussées éphémères de dermatite pustuleuse disséminée et symétrique. Bull Soc Fr Dermatol Syphiligr 1890;1:39-45.
Kirkup ME, Lovell CR: Acquired syndactyly secondary to acrodermatitis continua of Hallopeau. Br J Dermatol 2005;152:1083-1084.
Brill TJ, Elshorst-Schmidt T, Valesky EM, et al: Successful treatment of acrodermatitis continua of Hallopeau with sequential combination of calcipotriol and tacrolimus ointments. Dermatology 2005;211:351-355.
Baker H, Ryan TJ: Generalized pustular psoriasis. A clinical and epidemiological study of 104 cases. Br J Dermatol 1968;80:771-793.
Yerushalmi J, Grunwald MH, Hallel-Halevy D, et al: Chronic pustular eruption of the thumbs. Diagnosis: acrodermatitis continua of Hallopeau (ACH). Arch Dermatol 2000;136:925-930.
O'Keefe E, Braverman IM, Cohen I: Annulus migrans. Identical lesions in pustular psoriasis, Reiter's syndrome, and geographic tongue. Arch Dermatol 1973;107:240-244.
Onoufriadis A, Simpson MA, Pink AE, Di Meglio P, Smith CH, Pullabhatla V, Knight J, Spain SL, Nestle FO, Burden AD, Capon F, Trembath RC, Barker JN: Mutations in IL36RN/IL1F5 are associated with the severe episodic inflammatory skin disease known as generalized pustular psoriasis. Am J Hum Genet 2011;89:432-437.
Marrakchi S, Guigue P, Renshaw BR, Puel A, Pei XY, Fraitag S, Zribi J, Bal E, Cluzeau C, Chrabieh M, Towne JE, Douangpanya J, Pons C, Mansour S, Serre V, Makni H, Mahfoudh N, Fakhfakh F, Bodemer C, Feingold J, Hadj-Rabia S, Favre M, Genin E, Sahbatou M, Munnich A, Casanova JL, Sims JE, Turki H, Bachelez H, Smahi A: Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011;365:620-628.
Farooq M, Nakai H, Fujimoto A, Fujikawa H, Matsuyama A, Kariya N, Aizawa A, Fujiwara H, Ito M, Shimomura Y: Mutation analysis of the IL36RN gene in 14 Japanese patients with generalized pustular psoriasis. Hum Mutat 2013;34:176-183.
Lutz V, Lipsker D: Acitretin and tumor necrosis factor inhibitor-resistant acrodermatitis continua of Hallopeau responsive to the interleukin-1 receptor antagonist anakinra. Arch Dermatol 2012;148:297-299.
Viguier M, Guigue P, Pagès C, Smahi A, Bachelez H: Successful treatment of generalized pustular psoriasis with the interleukin-1-receptor antagonist anakinra: lack of correlation with IL1RN mutations. Ann Intern Med 2010;153:66-67.
Blumberg H, Dinh H, Trueblood ES, Pretorius J, Kugler D, Weng N, Kanaly ST, Towne JE, Willis CR, Kuechle MK, Sims JE, Peschon JJ: Opposing activities of two novel members of the IL-1 ligand family regulate skin inflammation. J Exp Med 2007;204:2603-2614.
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