Von zumbusch psoriasis : a rare autoinflammatory disease

Publication Date

2018

Document Type

Article

Abstract

Psoriasis vulgaris is a common skin disorder but it has many variants, which include the most severe and rare form: Acute Generalized Pustular Psoriasis (of Von Zumbusch). We report a case of RA, a 14-year-old female presenting with generalized erythematous pustular lesions with plaque formation and scaling which later on developed generalized desquamation and bullous lesions. Biopsy was done revealing epidermal acanthosis, parakeratosis with exocytosis of neutrophils and pustular formation ins stratum corneum, and perivascular inflammatory infiltrate in dermis consistent with Von Zumbusch Psoriasis. Acute Generalized Pustular Psoriasis of Von Zumbusch is an autoinflammatory condition; the most severe and rare subtype of psoriasis. It presents as widespread pustules on a background of red, tender skin. Ten (10%) precedes a previous history of psoriasis. This case report adds to literature of Acute Generalized Pustular Psoriasis of Von Zumbusch, in which there is no currently published data locally. This may offer possible studies for the incidence/prevalence of such condition in the Philippines.

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