PAPASH syndrome
Alerts and Notices
Synopsis
PAPASH should be distinguished from the following PG syndromes: PAPA (pyogenic arthritis, PG, and acne), PASH (PG, acne, and HS), and PsAPASH (psoriatic arthritis, PG, acne, and HS). These rare inflammatory syndromes are thought to share a common mechanism of interleukin (IL)‐1‐driven episodes of neutrophil‐predominated sterile inflammation.
Codes
ICD10CM:M04.9 – Autoinflammatory syndrome, unspecified
SNOMEDCT:
363138005 – Hereditary disorder of immune system
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Differential Diagnosis & Pitfalls
- PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome
- PASH (pyoderma gangrenosum, acne, and hidradenitis suppurativa) syndrome
- PsAPASH (psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa) syndrome
- Pyoderma gangrenosum, acne, and ulcerative colitis (PAC) syndrome
- SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome
- Hidradenitis suppurativa
- Folliculitis
- Furunculosis
- Epidermoid cysts
- Pyogenic gangrenosum
- Acne vulgaris
- Acne conglobata
- Pilonidal cleft
- Crohn disease
- Granuloma inguinale
- Septic arthritis
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Last Reviewed:06/11/2019
Last Updated:08/12/2019
Last Updated:08/12/2019