Acrodermatitis Continua of Hallopeau Is a Clinical Phenotype of DITRA: Evidence that It Is a Variant of Pustular Psoriasis

Abstract

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.

Acrodermatitis Continua of Hallopeau Treated Successfully with Ustekinumab and Acitretin after Failure of Tumour Necrosis Factor Blockade and Anakinra

“…Moreover, anakinra was recently successfully used in a case of DITRA syndrome in an infant [10] as well as in 3 cases [11,12] of generalized pustular psoriasis, one of them carrying a mutation of the IL-36RN gene [12]. IL-36 RN mutations can be found in patients with generalized pustular psoriasis [10,11] and in rare patients with ACH [12,13,14,15]. It has been hypothesized that such mutations could be associated with the response of generalized pustular psoriasis [12] or ACH [9] to anakinra [13,14], but this has not been demonstrated [14,16].…”

Impetigo Herpetiformis during Pregnancy: A Case Report and Literature Review

“…It was found that the function loss of IL-36RN (previously known as IL-1F5) implicates that GPP is caused by innate immune dysregulation in this severe episodic inflammatory disease. Therefore, taking IL-1 as a potential target for therapeutic intervention might cure the disease [20,21]. In order to find a better treatment for IH, more work should be continuously done to find the gene mutation of IH.…”

Highly Resistant Acrodermatitis Continua of Hallopeau and Pustular Psoriasis

“…Physicians unfamiliar with the disease can misdiagnose it as a chronic infection through identification of commensals (such as M. fortuitum ) on culture specimens. Reports of a Lebanese family with a common IL-36RN mutation presenting as both ACH and GPP raises questions as to the epigenetic or environmental cues that may result in different phenotypes [2], progression from one to the other [5], or manifestations of both, as seen in our case. It is also unclear why, if the conditions are related, such contrasting responses to treatment are seen within the same individual and why long-term remission often remains elusive [6].…”

“…ACH is a chronic, recalcitrant disease with a predisposition to elderly females [1], is considered an acral variant of pustular psoriasis [2], and shares a common pathogenesis with DITRA (deficiency in interleukin-1 receptor antagonist) [2,3,4]. Physicians unfamiliar with the disease can misdiagnose it as a chronic infection through identification of commensals (such as M. fortuitum ) on culture specimens.…”