5 articles
Acrodermatitis continua of Hallopeau
Acrodermatitis continua of Hallopeau (ACH), dermatitis repens, acropustulosis: rare chronic pustular psoriasis variant of the digits with sterile pustules, nail destruction, and osteolysis; IL36RN mutations in 20%; acitretin first-line, biologics second-line.
DITRA
DITRA (deficiency of interleukin-36 receptor antagonist; OMIM 614204) is a rare autosomal recessive autoinflammatory disease caused by IL36RN mutations, causing recurrent generalized pustular psoriasis flares with fever; treated with spesolimab.
Generalized pustular psoriasis
Generalized pustular psoriasis (GPP), also called von Zumbusch psoriasis, is a rare life-threatening neutrophilic skin disease with widespread sterile pustules and systemic symptoms, driven by IL-36 signaling and treated with spesolimab.
Pustular psoriasis
Pustular psoriasis, including generalized pustular psoriasis (von Zumbusch), palmoplantar pustulosis, and acrodermatitis continua of Hallopeau, is a psoriasis form with sterile neutrophilic pustules, IL36RN/CARD14/AP1S3 genetics, and spesolimab as the only FDA-approved GPP drug.
Pustulosis palmaris et plantaris
Pustulosis palmaris et plantaris (palmoplantar pustulosis, PPP), chronic relapsing sterile pustular dermatosis of palms and soles, female-predominant, smoking-linked, distinct from psoriasis, with pustulotic arthro-osteitis in 10-30%.