Successful therapy with secukinumab in a patient with generalized pustular psoriasis carrying homozygous IL36RN p.His32Arg mutation

Excellent response to secukinumab in an infant with severe generalized pustular psoriasis

“…Following previous reports of GPP and DITRA treated successfully with IL-17 antagonists, secukinumab was started with an incredibly prompt response from the first dose and maintained efficacy at 12-month follow up that permitted a sparing of the injections to every 12 weeks. 1,[7][8][9] This dramatic response to IL-17 antagonists probably reflects a higher pathogenic role of IL-17 in these patients than the one expected by the interruption of the IL-36 pathway.…”

“…[3][4][5][6] The lack of response to treatments of classic forms of psoriasis is common in these patients, and new immunomodulators such as anti-interleukin (IL)-1, anti-IL-12/ 23 or anti-IL-17 drugs have been tried with variable outcomes. 1,2,[7][8][9] We report herein a case of severe GPP in a 2-month-old infant who carried a heterozygous pathogenic IL36RN variant and a heterozygous CARD14 variant. She was treated successfully with the IL-17 antagonist secukinumab.…”

“…Generalized pustular psoriasis (GPP) is an unusual form of psoriasis that can be life-threatening. 1 It presents as flares of sudden-onset extensive erythema covered with pinheaded pustules associated with systemic symptoms such as fever and/or malaise, neutrophilia, liver test abnormalities and elevated acute phase reactants. 2 In the last decade, some GPP patients have been related to (likely) pathogenic variants in the IL36RN, CARD14 and AP1S3 genes, pointing at an autoinflammatory nature with variable penetrance and clinical expressivity.…”

“…Generalized pustular psoriasis (GPP) is an unusual form of psoriasis that can be life‐threatening 1 . It presents as flares of sudden‐onset extensive erythema covered with pinheaded pustules associated with systemic symptoms such as fever and/or malaise, neutrophilia, liver test abnormalities and elevated acute phase reactants 2 .…”

“…In the last decade, some GPP patients have been related to (likely) pathogenic variants in the IL36RN , CARD14 and AP1S3 genes, pointing at an autoinflammatory nature with variable penetrance and clinical expressivity 3–6 . The lack of response to treatments of classic forms of psoriasis is common in these patients, and new immunomodulators such as anti‐interleukin (IL)‐1, anti‐IL‐12/23 or anti‐IL‐17 drugs have been tried with variable outcomes 1,2,7–9 …”