Auto inflammatory diseases (AIDs) are a group of disorders characterized by the dysregulation of innate immune system and the absence of circulating autoantibodies and autoreactive T-cells. The AIDs characterized by neutrophilic dermatoses includes PAPA (pyogenic arthritis, pyoderma gangrenosum (PG) and acne), PASH (PG, acne and hidradenitis suppurativa), PAPASH (pyogenic arthritis, acne, PG and hidradenitis suppurativa) ,PASS (pyoderma gangrenosum, acne and sterile spondyloarthritis), PsAPASH (psoriatic arthritis, PG, acne, and hidradenitis suppurativa) and PAC (PG, acne, and ulcerative colitis). PASH syndrome is an autoinflammatory syndrome distinct from PAPA syndrome due to lack of intensive joint inflammation.
We present a case of a 20-year-old woman with a three-month history of non-healing crusted ulcers, associated with fever and symmetric polyarthralgia. Examination revealed multiple erythematous papules, plaques, nodules, pustules, with open and closed comedones on the face, hyperpigmented nodules and ulcers on the buttocks and posterior thigh and erythematous, nodules and ulcers with rolled violaceous borders topped with thick yellow crusts located on the shoulders, upper and lower extremities. Pathergy test revealed a pustular formation on the site after 48 hours. Hematologic test showed predominantly neutrophilia. Erythrocyte sedimentation rate and c-reactive protein were also elevated. Histopathology was consistent of neutrophilic dermatosis.
Patient was started with prednisone at 20 mg/day (0.5 mg/kg/day) for five months. Dapsone 50 mg/day was added after five months of prednisone as steroid-sparing agent, with significant improvement.
Ng, J, Visitacion, L & Bunagan, MJ. (2017). Pyoderma ganrenosum, acne, hidradenitis suppurativa and arthralgia: PASH or PAPA syndrome? Journal of the Philippine Dermatological Society, 26(2), 88-90.
Pyogenic granuloma, acne, hidradenitis suppurativa, prednisone, dapsone
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