A case of myeloperoxidase-antineutrophil cytoplasmic antibody and anticardiolipin antibody-positive pyogenic arthritis, pyoderma gangrenosum, acne and hidradenitis suppurativa (PAPASH) syndrome with colitis.

• 发表时间: 07 2021
• 来源期刊: Mod Rheumatol Case Rep
• DOI: 10.1080/24725625.2021.1881216
• PMID: 33560194
• 文章类型: Case Reports
• 作者列表: Kawanishi K,Nishiwaki H,Oshiro T,Kajitani H,Amagasa M,Uehara N,Inoue Y,Nagahama M,Koiwa F

关键词: MPO-ANCA; PAPA syndrome; PAPASH syndrome; anticardiolipin antibody; inflammatory bowel disease

Mesh: Acne Vulgaris / diagnosis; Adult; Antibodies, Anticardiolipin / isolation & purification; Antibodies, Antineutrophil Cytoplasmic / isolation & purification; Arthritis, Infectious / diagnosis; Colitis / complications; Hidradenitis Suppurativa / diagnosis; Humans; Male; Peroxidase / immunology; Pyoderma Gangrenosum / diagnosis; Syndrome

Abstract

A previous case report of colitis and serine proteinase 3-antineutrophil cytoplasmic antibody positivity in pyogenic arthritis, pyoderma gangrenosum (PG), acne and hidradenitis suppurativa (PAPASH) syndrome with colitis has been published. Herein, we report a similar case of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity. A 26-year-old man presented with recurrent aseptic pyogenic arthritis, acne, hidradenitis suppurativa and PG. Lower gastrointestinal endoscopy was performed, and colitis was observed. No PSTPIP1 gene mutation was found in the gene-sequencing test. Based on these findings and prior case reports, we diagnosed the patient with PAPASH syndrome, a PAPA spectrum disorder complicated by colitis. This patient had PAPASH syndrome with colitis and was MPO-ANCA and anticardiolipin antibodies-positive; it is unclear whether these antibodies play a role in this disease, but it may provide clues to further elucidate its pathogenesis.