Hervez Bachelez
DOI: 10.2340/00015555-3388

Pustular psoriasis is a clinically heterogeneous entity of different, orphan disease subtypes, among which the most clearly defined are generalized pustular psoriasis, palmoplantar psoriasis, and acrodermatitis continua of Hallopeau. Although phenotypically and genetically distinct from psoriasis vulgaris, these subtypes may be associated with plaque psoriasis lesions, establishing the rationale for their inclusion in the psoriasis spectrum. Unlike psoriasis, however, their genetic background is thought to be mainly monogenic, as shown by the recent identification of mutations in 3 different genes of the skin innate immune system; IL36RN, CARD14 and AP1S3. These major advances in the understanding of the disease pathogenesis have led to the design and ongoing development of tailored therapeutic approaches, which are highly necessary given the refractory nature of pustular psoriasis in response to most available antipsoriatic drugs.

Pustular psoriasis defines a heterogeneous group of skin inflammatory diseases, which have in common the presence of aseptic pustules. Genetically distinct from psoriasis vulgaris, they have been shown to be related to mutations in any of 3 genes of the skin immune system, respectively called IL36RN, CARD14 and AP1S3. These recent advances have initiated the design of biological drugs specifically targeting key actors of inflammation in pustular psoriasis, with interleukin-36 inhibitors as the most advanced example of therapeutic development.