A Unified Concept of Acne in the PAPA Spectrum Disorders

Dermatology. 2021;237(5):827-834. doi: 10.1159/000509874. Epub 2020 Sep 16.

Abstract

Background: Acne is a common condition. Sometimes acne or acne-like features are a part of autoinflammatory diseases such as PAPA spectrum disorders. Our objective wasto determine whether acne in PAPA spectrum disorders has specific phenotypic traits.

Summary: This is aliterature review on reported cases of patients with PAPA, PAMI, PASH, PAPASH, PsAPASH, PAC or PASS syndrome. Acne was classified into four categories: moderate, severe, nodulocystic, acne fulminans.One hundred patients were included. Age of onset of acne and topography were the same as in acne vulgaris. Acne's phenotype was at least severe (including acne fulminans, nodulocystic and severe) in 83% of cases. Genetic mutations or variants with potential pathological significance were reported in 49 patients. The addition of isotretinoin to biotherapies was required to control acne in several patients.

Keywords: Acne; Interleukin-1 inhibitors; Isotretinoin; PAPA syndrome; PSTPIP1 gene.

Publication types

  • Review

MeSH terms

  • Acne Vulgaris / complications
  • Acne Vulgaris / diagnosis*
  • Acne Vulgaris / therapy
  • Anti-Inflammatory Agents / therapeutic use
  • Arthritis, Infectious / complications
  • Arthritis, Infectious / diagnosis*
  • Arthritis, Infectious / therapy
  • Dermatologic Agents / therapeutic use
  • Diagnosis, Differential
  • Humans
  • Pyoderma Gangrenosum / complications
  • Pyoderma Gangrenosum / diagnosis*
  • Pyoderma Gangrenosum / therapy

Substances

  • Anti-Inflammatory Agents
  • Dermatologic Agents

Supplementary concepts

  • Pyogenic arthritis, pyoderma gangrenosum, and acne