Clinical, biological and histological characteristics of bullous pemphigoid associated with anti‐PD‐1/PD‐L1 therapy: A national retrospective study

Abstract

Dear Editor, Costello syndrome is a rare genetic syndrome. The mode of inheritance is autosomal dominant; however, most cases are sporadic. Mutations in the HRAS gene lead to a dysregulation of the Ras/mitogen-activated protein kinase (MAPK) pathway. 1 These genetic diseases are known as RASopathies and include Costello syndrome, LEOPARD syndrome, Noonan syndrome, cardiofaciocutaneous syndrome, neurofibromatosis type 1 and Legius syndrome. Patients with RASopathies often suffer from mental retardation, cardiovascular disease, short stature, macrocephaly

European recommendations for management of immune checkpoint inhibitors‐derived dermatologic adverse events. The EADV task force ‘Dermatology for cancer patients’ position statement

“…5a-c) have been also described in association with ICI. 65,66 They are mostly linked to anti PD-1/PD-L1 agents. 59,67 BP is the predominant phenotype.…”

“…1,66 The eruption develops from weeks to several months after treatment initiation and manifests with tense bullae and vesicles involving the trunk and extremities. 59,65,67,71,72 A pre-bullous phase, characterised by intractable pruritic and non-specific maculopapular or urticarial plaques may precede bullae formation. 69,73 Mucosal involvement is more common as compared to classic BP, with an incidence close to 40% in the most recent series.…”

“…12,69 Direct immunofluorescence (DIF and salt-split DIF), demonstrating linear deposits of IgG and C3 to the epidermal part of the clefting and ELISA studies, mostly detecting BP180 and less often BP230 antibodies, are of high diagnosticvalueand shouldbeincludedin the workup. 59,65,73 Bullous pemphigoid has a profound impact on ICI therapy, resulting in permanent discontinuation in 20%-50% of patients. 59,65,67,69 Early diagnosis and appropriate intervention seem to facilitate adequate control of BP, limiting treatment withdrawals.…”

“…69,73 Mucosal involvement is more common as compared to classic BP, with an incidence close to 40% in the most recent series. 65,73 Histopathology is characterised by subepidermal clefting containing fibrin and eosinophils and dermal infiltrates composed of lymphocytes, eosinophils and often neutrophils. 12,69 Direct immunofluorescence (DIF and salt-split DIF), demonstrating linear deposits of IgG and C3 to the epidermal part of the clefting and ELISA studies, mostly detecting BP180 and less often BP230 antibodies, are of high diagnosticvalueand shouldbeincludedin the workup.…”

Quoi de neuf en dermatologie clinique ?

“…Dans ce cas, elle atteint des hommes dans 80 % des cas, au bout d’un délai médian de 6 mois, avec atteinte muqueuse plus fréquente (38 %). Elle serait associée à une meilleure réponse antitumorale et n’impose pas l’arrêt de l’immunothérapie chez les patients traités par dermocorticoïdes [ 75 ].…”