Authors: Janice Natasha C. Ng, MD, Mary Jo Kristine S. Bunagan, MD, FPDS

Abstract

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.

Citation

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.

Keywords

Pyogenic granuloma, acne, hidradenitis suppurativa, prednisone, dapsone

4. 1. Vinkel C, Thomsen SF. Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne. Int J Dermatol. 2017 Mar 1:1-8.1. Vinkel C, Thomsen SF. Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne. Int J Dermatol. 2017 Mar 1:1-8.
5. 2. Cugno M, Borghi A, Marzano AV. PAPA, PASH and PAPASH Syndromes: Pathophysiology, Presentation and Treatment. Am J Clin Dermatol. 2017 Feb 25:1-8.

4. 3. Braun-Falco M, Kovnerystyy O, Lohse P, Ruzicka T. Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH)–a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol. 2012 Mar 31;66(3):409-15.

4. 4. Philippine Dermatological Society Health Information Systems. Philippine Dermatological Society. c2011 . Available by request from: pdshis@outlook.com

4. 5. Calderón‐Castrat X, Bancalari‐Díaz D, Román‐Curto C, Romo‐Melgar A, Amorós‐Cerdán D, Alcaraz‐Mas LA, Fernández‐López E, Cañueto J. PSTPIP1 gene mutation in a pyoderma gangrenosum, acne and suppurative hidradenitis (PASH) syndrome. Br J Dermatol. 2016 Jul 1;175(1):1-5.

4. 6. Niv D, Ramirez JA, Fivenson DP. Pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome with recurrent vasculitis. JAAD Case Reports. 2017 Jan;3(1):70-73.

4. 7. Sterba G, Sterba Y. Controlling Inflammation: Contemporary Treatments for Autoinflammatory Diseases and Syndromes. Dermatol Clin.2013 ;31(3):507-11.

4. 8. Staub J, Pfannschmidt N, Strohal R, Braun‐Falco M, Lohse P, Goerdt S, Leverkus M. Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone. J Eur Acad Dermatol Venereol. 2015 Nov 1;29(11):2243-7.

4. 9. Join-Lambert O, Duchatelet S, Delage M, Miskinyte S, Coignard H, Lemarchand N, Alemy-Carreau M, Lortholary O, Nassif X, Hovnanian A, Nassif A. Remission of refractory pyoderma gangrenosum, severe acne, and hidradenitis suppurativa (PASH) syndrome using targeted antibiotic therapy in 4 patients. J Am Acad Dermatol. 2015 Nov 30;73(5):S66-9.

More Articles