Significance of IL36RN mutation analyses in the management of impetigo herpetiformis: A case report and review of published cases

Abstract

Impetigo herpetiformis (IH) is a rare variant of generalized pustular psoriasis (GPP), which develops during pregnancy. GPP is associated with mutations of IL36RN, but it is still unclear whether the same is true of IH. A 20-year-old Japanese woman developed erythema and pustules on her trunk during the 27th week of her first pregnancy.

Pustular psoriasis of pregnancy: Clinical and genetic characteristics in a series of eight patients and review of the literature

“…Two of these patients had a homozygous mutation c.80 T > C (p.L27P) in IL36RN gene. This was similar to findings of Suguira and Yoshikuwa, 17,30 who reported recurrence of GPP outside of pregnancy of two patients having a mutation in IL36RN gene.…”

“…The literature search identified 83 articles, only 35 were ultimately selected 5–39 . The corresponding results are summarized in Table 2.…”

“…The mean of the gestational age at the onset was 26 ± 5.2 GW in the present cohort similar to the literature. In fact, PPP often occurs in the last trimester of pregnancy [6][7][8][9][14][15][16]20,21,[24][25][26][27]30,32,35,37 but occurrence in the second trimester is not rare. [10][11][12]15,[17][18][19]23,28,29,31,33,34,36,39 Early onset at the first trimester of pregnancy is unusual and was reported only in three patients with no history of GPP.…”

Pustular Psoriasis: From Pathophysiology to Treatment

“…IL36RN variants have been detected also in IH, and, in particular, East Asian founder mutations might be implicated in IH pathogenesis [ 105 ]. Curiously, identical IL36RN mutations led to both isolated IH and IH with a preceding history of GPP and/or PV.…”

Recurrence of impetigo herpetiformis carrying compound heterozygous mutations in IL36RN after remission with secukinumab

“…2 Recently, mutations in IL36RN have been implicated as the underlying cause in some patients with IH who often experience postpartum flare-ups and require biologic therapy. 1,2 So far, the clinical course after delivery has been reported in four patients with IL36RN mutations, 1,3-5 and one patient was reported to have long-term remission after discontinuing a total of six doses of secukinumab. 3 Following this report, in our case, secukinumab was discontinued after a total of six doses; however, the skin symptoms recurred approximately 1 year after the secukinumab withdrawal.…”