Advertisement

Therapeutic Strategies

  • Courtney R. Schadt
  • Jeffrey P. Callen
Chapter

Abstract

There is significant overlap among the therapeutic strategies of neutrophilic dermatoses given the targeting of neutrophils. In addition, there is a paucity of quality, randomized controlled trials. Most treatments have been reported through case series and reports. Systemic corticosteroids are effective for most neutrophilic conditions. However, side effects limit long-term use.

References

  1. 1.
    Rozen SM, Nahabedian MY, Manson PN. Management strategies for pyoderma gangrenosum: case studies and review of literature. Ann Plast Surg. 2001;47:310–5.PubMedCrossRefGoogle Scholar
  2. 2.
    Aydin S, Aydin CA, Ugurlucan FG, Yasa C, Dural O. Recurrent pyoderma gangrenosum after cesarean delivery successfully treated with vacuum-assisted closure and split thickness skin graft: a case report. J Obstet Gynaecol Res. 2015;41:635–9.PubMedCrossRefGoogle Scholar
  3. 3.
    Cohen PR, Kurzrock R. Sweet’s syndrome and cancer. Clin Dermatol. 1993;11:149–57.CrossRefPubMedGoogle Scholar
  4. 4.
    Reichrath J, Bens G, Bonowitz A, Tilgen W. Treatment recommendations for pyoderma gangrenosum: an evidence-based review of the literature based on more than 350 patients. J Am Acad Dermatol. 2005;53:273–83.PubMedCrossRefGoogle Scholar
  5. 5.
    Wang MZ, Camilleri MJ, Guo R, Wieland CN. Amicrobial pustulosis of the folds: report of 4 cases. J Cutan Pathol. 2016;44(4):367–72.CrossRefGoogle Scholar
  6. 6.
    Lyon CC, Stapleton M, Smith AJ, Mendelsohn S, Beck MH, Griffiths CE. Topical tacrolimus in the management of peristomal pyoderma gangrenosum. J Dermatol Treat. 2001;12:13–7.CrossRefGoogle Scholar
  7. 7.
    Walker DC, Cohen PR. Trimethoprim-sulfamethoxazole-associated acute febrile neutrophilic dermatosis: case report and review of drug-induced Sweet’s syndrome. J Am Acad Dermatol. 1996;34:918–23.CrossRefPubMedGoogle Scholar
  8. 8.
    Su WP, Davis MD, Weenig RH, Powell FC, Perry HO. Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria. Int J Dermatol. 2004;43:790–800.PubMedCrossRefGoogle Scholar
  9. 9.
    Aseni P, Di Sandro S, Mihaylov P, Lamperti L, De Carlis LG. Atypical presentation of pioderma gangrenosum complicating ulcerative colitis: rapid disappearance with methylprednisolone. World J Gastroenterol. 2008;14:5471–3.PubMedPubMedCentralCrossRefGoogle Scholar
  10. 10.
    Ormerod AD, Thomas KS, Craig FE, et al. Comparison of the two most commonly used treatments for pyoderma gangrenosum: results of the STOP GAP randomised controlled trial. BMJ. 2015;350:h2958.PubMedPubMedCentralCrossRefGoogle Scholar
  11. 11.
    Binus AM, Qureshi AA, Li VW, Winterfield LS. Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br J Dermatol. 2011;165:1244–50.PubMedCrossRefGoogle Scholar
  12. 12.
    von den Driesch P. Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol. 1997;137:1000–5.PubMedCrossRefGoogle Scholar
  13. 13.
    Cohen PR, Kurzrock R. Sweet’s syndrome: a review of current treatment options. Am J Clin Dermatol. 2002;3:117–31.PubMedCrossRefGoogle Scholar
  14. 14.
    el Sherif AI, Bharija SC, Belhaj MS, Singh G. Dapsone in Sweet syndrome. Int J Dermatol. 1990;29:737.PubMedGoogle Scholar
  15. 15.
    Yamanaka K, Murota H, Goto H, et al. Pyoderma gangrenosum and annular erythema associated with Sjogren’s syndrome controlled with minocycline. J Dermatol. 2015;42:834–6.PubMedCrossRefGoogle Scholar
  16. 16.
    Sau M, Hill NC. Pyoderma gangrenosum of the vulva. BJOG. 2001;108:1197–8.PubMedGoogle Scholar
  17. 17.
    Thibault MJ, Billick RC, Srolovitz H. Minocycline-induced Sweet’s syndrome. J Am Acad Dermatol. 1992;27:801–4.PubMedCrossRefGoogle Scholar
  18. 18.
    Khan Durani B, Jappe U. Drug-induced Sweet’s syndrome in acne caused by different tetracyclines: case report and review of the literature. Br J Dermatol. 2002;147:558–62.PubMedCrossRefGoogle Scholar
  19. 19.
    Ritter S, George R, Serwatka LM, Elston DM. Long-term suppression of chronic Sweet’s syndrome with colchicine. J Am Acad Dermatol. 2002;47:323–4.PubMedCrossRefGoogle Scholar
  20. 20.
    Maillard H, Leclech C, Peria P, Avenel-Audran M, Verret JL. Colchicine for Sweet’s syndrome. A study of 20 cases. Br J Dermatol. 1999;140:565–6.CrossRefPubMedGoogle Scholar
  21. 21.
    Paolini O, Hebuterne X, Flory P, Charles F, Rampal P. Treatment of pyoderma gangrenosum with colchicine. Lancet. 1995;345:1057–8.PubMedCrossRefGoogle Scholar
  22. 22.
    von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol. 1994;31:535–56; quiz 57–60.CrossRefPubMedGoogle Scholar
  23. 23.
    Cohen PR, Holder WR, Tucker SB, Kono S, Kurzrock R. Sweet syndrome in patients with solid tumors. Cancer. 1993;72:2723–31.PubMedCrossRefGoogle Scholar
  24. 24.
    Horio T, Danno K, Okamoto H, Miyachi Y, Imamura S. Potassium iodide in erythema nodosum and other erythematous dermatoses. J Am Acad Dermatol. 1983;9:77–81.PubMedCrossRefGoogle Scholar
  25. 25.
    Sanchez MR. Miscellaneous treatments: thalidomide, potassium iodide, levamisole, clofazimine, colchicine, and D-penicillamine. Clin Dermatol. 2000;18:131–45.PubMedCrossRefGoogle Scholar
  26. 26.
    Jeanfils S, Joly P, Young P, Le Corvaisier-Pieto C, Thomine E, Lauret P. Indomethacin treatment of eighteen patients with Sweet’s syndrome. J Am Acad Dermatol. 1997;36:436–9.CrossRefPubMedGoogle Scholar
  27. 27.
    von den Driesch P, Steffan C, Zobe A, Hornstein OP. Sweet’s syndrome—therapy with cyclosporin. Clin Exp Dermatol. 1994;19:274–7.CrossRefPubMedGoogle Scholar
  28. 28.
    Yasuda F, Fujio Y, Kakuta R, Funakoshi T, Amagai M, Ohyama M. Use of cyclosporin A for successful management of steroid-resistant Sweet’s syndrome patient with possible myelodysplastic syndrome. J Dermatol. 2014;41:465–6.PubMedCrossRefGoogle Scholar
  29. 29.
    Ma EH, Akikusa JD, MacGregor D, Ng J, Su JC. Sweet’s syndrome with postinflammatory elastolysis and Takayasu arteritis in a child: a case report and literature review. Pediatr Dermatol. 2012;29:645–50.PubMedCrossRefGoogle Scholar
  30. 30.
    Kluger N, Gil-Bistes D, Guillot B, Bessis D. Efficacy of anti-interleukin-1 receptor antagonist anakinra (Kineret(R)) in a case of refractory Sweet’s syndrome. Dermatology. 2011;222:123–7.CrossRefPubMedGoogle Scholar
  31. 31.
    Browning CE, Dixon JE, Malone JC, Callen JP. Thalidomide in the treatment of recalcitrant Sweet’s syndrome associated with myelodysplasia. J Am Acad Dermatol. 2005;53:S135–8.CrossRefPubMedGoogle Scholar
  32. 32.
    Case JD, Smith SZ, Callen JP. The use of pulse methylprednisolone and chlorambucil in the treatment of Sweet’s syndrome. Cutis. 1989;44:125–9.PubMedGoogle Scholar
  33. 33.
    Seminario-Vidal L, Guerrero C, Sami N. Refractory Sweet’s syndrome successfully treated with rituximab. JAAD Case Rep. 2015;1:123–5.PubMedPubMedCentralCrossRefGoogle Scholar
  34. 34.
    Karamlou K, Gorn AH. Refractory sweet syndrome with autoimmune organizing pneumonia treated with monoclonal antibodies to tumor necrosis factor. J Clin Rheumatol. 2004;10:331–5.PubMedCrossRefGoogle Scholar
  35. 35.
    Ambrose NL, Tobin AM, Howard D. Etanercept treatment in Sweet’s syndrome with inflammatory arthritis. J Rheumatol. 2009;36:1348–9.PubMedCrossRefGoogle Scholar
  36. 36.
    Yaghmour G, Wiedower E, Yaghmour B, Nunnery S, Duncavage E, Martin MG. Sweet’s syndrome associated with clonal hematopoiesis of indeterminate potential responsive to 5-azacitidine. Ther Adv Hematol. 2017;8:91–5.PubMedCrossRefGoogle Scholar
  37. 37.
    Trickett HB, Cumpston A, Craig M. Azacitidine-associated Sweet’s syndrome. Am J Health Syst Pharm. 2012;69:869–71.PubMedCrossRefGoogle Scholar
  38. 38.
    Biswas SN, Chakraborty PP, Gantait K, Bar C. Azathioprine-induced bullous Sweet’s syndrome: a rare association. BMJ Case Rep. 2016;2016:10.1136/bcr-2016-215192.  https://doi.org/10.1136/bcr-2016-215192.
  39. 39.
    Cyrus N, Stavert R, Mason AR, Ko CJ, Choi JN. Neutrophilic dermatosis after azathioprine exposure. JAMA Dermatol. 2013;149:592–7.CrossRefPubMedGoogle Scholar
  40. 40.
    Banse C, Sobocinski V, Savoye G, Avenel G, Vittecoq O. Occurrence of Sweet syndrome under anti-TNF. Clin Rheumatol. 2015;34:1993–4.CrossRefPubMedGoogle Scholar
  41. 41.
    Brooklyn TN, Dunnill MG, Shetty A, et al. Infliximab for the treatment of pyoderma gangrenosum: a randomised, double blind, placebo controlled trial. Gut. 2006;55:505–9.PubMedPubMedCentralCrossRefGoogle Scholar
  42. 42.
    Callen JP, Jackson JM. Pyoderma gangrenosum: an update. Rheum Dis Clin North Am. 2007;33:787–802, vi.PubMedCrossRefGoogle Scholar
  43. 43.
    Del Giacco SR, Firinu D, Lorrai MM, et al. Idiopathic pyoderma gangrenosum: successful resolution with infliximab therapy and pro-inflammatory cytokines assessment. Acta Derm Venereol. 2012;92:439–40.PubMedCrossRefGoogle Scholar
  44. 44.
    Kaur MR, Lewis HM. Severe recalcitrant pyoderma gangrenosum treated with infliximab. Br J Dermatol. 2005;153:689–91.PubMedCrossRefGoogle Scholar
  45. 45.
    Alkhouri N, Hupertz V, Mahajan L. Adalimumab treatment for peristomal pyoderma gangrenosum associated with Crohn’s disease. Inflamm Bowel Dis. 2009;15:803–6.PubMedCrossRefGoogle Scholar
  46. 46.
    Hubbard VG, Friedmann AC, Goldsmith P. Systemic pyoderma gangrenosum responding to infliximab and adalimumab. Br J Dermatol. 2005;152:1059–61.PubMedCrossRefGoogle Scholar
  47. 47.
    Sagami S, Ueno Y, Tanaka S, Nagai K, Hayashi R, Chayama K. Successful use of adalimumab for treating pyoderma gangrenosum with ulcerative colitis under corticosteroid-tapering conditions. Intern Med. 2015;54:2167–72.PubMedCrossRefGoogle Scholar
  48. 48.
    Campanati A, Brisigotti V, Ganzetti G, et al. Finally, recurrent pyoderma gangrenosum treated with Adalimumab: case report and review of the literature. J Eur Acad Dermatol Venereol. 2015;29:1245–7.PubMedCrossRefGoogle Scholar
  49. 49.
    Kim FS, Pandya AG. The use of etanercept in the treatment of peristomal pyoderma gangrenosum. Clin Exp Dermatol. 2012;37:442–3.PubMedCrossRefGoogle Scholar
  50. 50.
    Rogge FJ, Pacifico M, Kang N. Treatment of pyoderma gangrenosum with the anti-TNFalpha drug—etanercept. J Plast Reconstr Aesthet Surg. 2008;61:431–3.PubMedCrossRefGoogle Scholar
  51. 51.
    Li J, Kelly R. Treatment of pyoderma gangrenosum with mycophenolate mofetil as a steroid-sparing agent. J Am Acad Dermatol. 2013;69:565–9.PubMedCrossRefGoogle Scholar
  52. 52.
    Teitel AD. Treatment of pyoderma gangrenosum with methotrexate. Cutis. 1996;57:326–8.PubMedGoogle Scholar
  53. 53.
    Duffill MB. Cyclosporine, azathioprine and local therapy for pyoderma gangrenosum. Australas J Dermatol. 1994;35:15–8.PubMedCrossRefGoogle Scholar
  54. 54.
    Behm K, Larson DW, Colibaseanu D. Intravenous immunoglobulin use in managing severe, perioperative peristomal pyoderma gangrenosum following subtotal colectomy with end ileostomy for medically refractory chronic ulcerative colitis. J Surg Case Rep. 2015;2015(3). pii: rjv019.PubMedPubMedCentralCrossRefGoogle Scholar
  55. 55.
    Gupta AK, Shear NH, Sauder DN. Efficacy of human intravenous immune globulin in pyoderma gangrenosum. J Am Acad Dermatol. 1995;32:140–2.PubMedCrossRefGoogle Scholar
  56. 56.
    Hurabielle C, Schneider P, Baudry C, Bagot M, Allez M, Viguier M. Certolizumab pegol—a new therapeutic option for refractory disseminated pyoderma gangrenosum associated with Crohn’s disease. J Dermatolog Treat. 2016;27:67–9.PubMedCrossRefGoogle Scholar
  57. 57.
    Goldminz AM, Botto NC, Gottlieb AB. Severely recalcitrant pyoderma gangrenosum successfully treated with ustekinumab. J Am Acad Dermatol. 2012;67:e237–8.PubMedCrossRefGoogle Scholar
  58. 58.
    Burruss JB, Farmer ER, Callen JP. Chlorambucil is an effective corticosteroid-sparing agent for recalcitrant pyoderma gangrenosum. J Am Acad Dermatol. 1996;35:720–4.PubMedCrossRefGoogle Scholar
  59. 59.
    Kaminska R, Ikaheimo R, Hollmen A. Plasmapheresis and cyclophosphamide as successful treatments for pyoderma gangrenosum. Clin Exp Dermatol. 1999;24:81–5.PubMedCrossRefGoogle Scholar
  60. 60.
    Jones RR, Kobza Black A, Donaghy M, Moshtael O, Pinching AJ. Defective monocyte function in pyoderma gangrenosum with IgG kappa paraproteinaemia. Clin Exp Immunol. 1983;52:685–92.PubMedPubMedCentralGoogle Scholar
  61. 61.
    Kolios AG, Maul JT, Meier B, et al. Canakinumab in adults with steroid-refractory pyoderma gangrenosum. Br J Dermatol. 2015;173:1216–23.PubMedCrossRefGoogle Scholar
  62. 62.
    Andre MF, Piette JC, Kemeny JL, et al. Aseptic abscesses: a study of 30 patients with or without inflammatory bowel disease and review of the literature. Medicine. 2007;86:145–61.PubMedCrossRefGoogle Scholar
  63. 63.
    Yildiz H, Munting A, Komuta M, Danse E, Lefebvre C. Aseptic lung and liver abscesses: a diagnostic challenge. Acta Clin Belg. 2016;72(4):1–5.Google Scholar
  64. 64.
    Fukuda S, Nanki T, Morio T, Hasegawa H, Koike R, Miyasaka N. Recurrent mitral valve regurgitation with neutrophil infiltration in a patient with multiple aseptic abscesses. Mod Rheumatol. 2014;24:537–9.PubMedCrossRefGoogle Scholar
  65. 65.
    Nakanishi K, Kinjo M. Mimicker of necrotising fasciitis with systemic inflammatory response syndrome: recurrent necrotising Sweet’s syndrome associated with chronic myelogenous leukaemia. BMJ Case Rep. 2016;2016. pii: bcr2016214461.  https://doi.org/10.1136/bcr-2016-214461.
  66. 66.
    Qian L, Shen J, Cen J, Yin W, Ma Y. Myelodysplastic syndrome with neutrophilic panniculitis: a report of two cases and a literature review. Oncol Lett. 2015;9:1954–6.PubMedPubMedCentralCrossRefGoogle Scholar
  67. 67.
    Katz SI, Gallin JI, Hertz KC, Fauci AS, Lawley TJ. Erythema elevatum diutinum: skin and systemic manifestations, immunologic studies, and successful treatment with dapsone. Medicine. 1977;56:443–55.PubMedCrossRefGoogle Scholar
  68. 68.
    Chan Y, Mok CC, Tang WY. Erythema elevatum diutinum in systemic lupus erythematosus. Rheumatol Int. 2011;31:259–62.PubMedCrossRefGoogle Scholar
  69. 69.
    Takahashi H, Fukami Y, Honma M, Ishida-Yamamoto A, Iizuka H. Successful combination therapy with dapsone and cyclosporine for erythema elevatum diutinum with unusual appearance. J Dermatol. 2012;39:486–7.PubMedCrossRefGoogle Scholar
  70. 70.
    Momen SE, Jorizzo J, Al-Niaimi F. Erythema elevatum diutinum: a review of presentation and treatment. J Eur Acad Dermatol Venereol. 2014;28:1594–602.PubMedCrossRefGoogle Scholar
  71. 71.
    Jiao T, Wang M, Zhu X. A case of erythema elevatum diutinum associated with peripheral ulcerative keratitis. Australas J Dermatol. 2012;53:78–80.PubMedCrossRefGoogle Scholar
  72. 72.
    Wilkinson SM, English JS, Smith NP, Wilson-Jones E, Winkelmann RK. Erythema elevatum diutinum: a clinicopathological study. Clin Exp Dermatol. 1992;17:87–93.PubMedCrossRefGoogle Scholar
  73. 73.
    Gibson LE, el-Azhary RA. Erythema elevatum diutinum. Clin Dermatol. 2000;18:295–9.PubMedCrossRefGoogle Scholar
  74. 74.
    Kocaturk E, Memet B, Topal IO, Yuksel T, Ulkumen PK, Kiziltac U. A case of erythema elevatum diutinum with pancytopenia: focus on dapsone-induced hematologic side effects and colchicine as a safe treatment option. J Drugs Dermatol. 2015;14:1090–2.PubMedGoogle Scholar
  75. 75.
    Henriksson R, Hofer PA, Hornqvist R. Erythema elevatum diutinum—a case successfully treated with colchicine. Clin Exp Dermatol. 1989;14:451–3.PubMedCrossRefGoogle Scholar
  76. 76.
    Frieling GW, Williams NL, Lim SJ, Rosenthal SI. Novel use of topical dapsone 5% gel for erythema elevatum diutinum: safer and effective. J Drugs Dermatol. 2013;12:481–4.PubMedGoogle Scholar
  77. 77.
    Zacaron LH, Goncalves JC, Curty VM, D’Acri AM, Lima RB, Martins CJ. Clinical and surgical therapeutic approach in erithema elevatum diutinum—case report. An Bras Dermatol. 2013;88:15–8.PubMedPubMedCentralCrossRefGoogle Scholar
  78. 78.
    Thomas CL, Ffolkes L, Akhras V. A case of mistaken identity: unilateral erythema elevatum diutinum associated with IgA paraproteinaemia. Clin Exp Dermatol. 2015;40:761–4.PubMedCrossRefGoogle Scholar
  79. 79.
    Manni E, Cervadoro E, Papineschi F. Case of erythema elevatum diutinum associated with IgA paraproteinemia successfully controlled with thalidomide and plasma exchange. Ther Apher Dial. 2015;19:195–6.PubMedCrossRefGoogle Scholar
  80. 80.
    Atallah J, Garces JC, Loayza E, Carlson JA. Chronic localized fibrosing leukocytoclastic vasculitis associated with lymphedema, intralymphatic and intravascular lymphocytosis, and chronic myelogenous leukemia: a case report of unilateral erythema elevatum diutinum. Am J Dermatopathol. 2016;39(6):479–84.CrossRefGoogle Scholar
  81. 81.
    Sandhu K, Saraswat A, Gupta S. Inverse subcorneal pustular dermatosis. J Eur Acad Dermatol Venereol. 2003;17:348–9.PubMedCrossRefGoogle Scholar
  82. 82.
    Roger H, Thevenet JP, Souteyrand P, Sauvezie B. Subcorneal pustular dermatosis associated with rheumatoid arthritis and raised IgA: simultaneous remission of skin and joint involvements with dapsone treatment. Ann Rheum Dis. 1990;49:190–1.PubMedPubMedCentralCrossRefGoogle Scholar
  83. 83.
    Scalvenzi M, Palmisano F, Annunziata MC, Mezza E, Cozzolino I, Costa C. Subcorneal pustular dermatosis in childhood: a case report and review of the literature. Case Rep Dermatol Med. 2013;2013:424797.PubMedPubMedCentralGoogle Scholar
  84. 84.
    Kocak M, Birol A, Erkek E, Bozdogan O, Atasoy P. Juvenile subcorneal pustular dermatosis: a case report. Pediatr Dermatol. 2003;20:57–9.PubMedCrossRefGoogle Scholar
  85. 85.
    Marliere V, Beylot-Barry M, Beylot C, Doutre M. Successful treatment of subcorneal pustular dermatosis (Sneddon-wilkinson disease) by acitretin: report of a case. Dermatology. 1999;199:153–5.PubMedCrossRefGoogle Scholar
  86. 86.
    Canpolat F, Akpinar H, Cemil BC, Eskioglu F, Ozturk E. A case of subcorneal pustular dermatosis in association with monoclonal IgA gammopathy successfully treated with acitretin. J Dermatol Treat. 2010;21:114–6.CrossRefGoogle Scholar
  87. 87.
    Berk DR, Hurt MA, Mann C, Sheinbein D. Sneddon-Wilkinson disease treated with etanercept: report of two cases. Clin Exp Dermatol. 2009;34:347–51.PubMedCrossRefGoogle Scholar
  88. 88.
    de Encarnacao Roque Diamantino F, Dias Coelho JM, Macedo Ferreira AM, Fidalgo AI. Subcorneal pustular dermatosis treated successfully with adalimumab. Eur J Dermatol. 2010;20:512–4.PubMedGoogle Scholar
  89. 89.
    Voigtlander C, Luftl M, Schuler G, Hertl M. Infliximab (anti-tumor necrosis factor alpha antibody): a novel, highly effective treatment of recalcitrant subcorneal pustular dermatosis (Sneddon-Wilkinson disease). Arch Dermatol. 2001;137:1571–4.PubMedCrossRefGoogle Scholar
  90. 90.
    Pavithran K. Colchicine in the treatment of subcorneal pustular dermatosis. Indian J Dermatol Venereol Leprol. 1995;61:56–7.PubMedGoogle Scholar
  91. 91.
    Cameron H, Dawe RS. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with narrowband (TL-01) UVB phototherapy. Br J Dermatol. 1997;137:150–1.PubMedCrossRefGoogle Scholar
  92. 92.
    Bauwens M, De Coninck A, Roseeuw D. Subcorneal pustular dermatosis treated with PUVA therapy. A case report and review of the literature. Dermatology. 1999;198:203–5.PubMedCrossRefGoogle Scholar
  93. 93.
    Karadogan SK, Aydogan K, Baskan EB, Tunali S. A case of subcorneal pustular dermatosis treated successfully with a combination of cyclosporin and prednisolone. J Eur Acad Dermatol Venereol. 2007;21:536–7.PubMedGoogle Scholar
  94. 94.
    Zachariae CO, Rossen K, Weismann K. An unusual severe case of subcorneal pustular dermatosis treated with cyclosporine and prednisolone. Acta Derm Venereol. 2000;80:386–7.PubMedGoogle Scholar
  95. 95.
    Hoshina D, Tsujiwaki M, Furuya K. Successful treatment of subcorneal pustular dermatosis with maxacalcitol. Clin Exp Dermatol. 2016;41:102–3.PubMedCrossRefGoogle Scholar
  96. 96.
    Kawaguchi M, Mitsuhashi Y, Kondo S. A case of subcorneal pustular dermatosis treated with tacalcitol (1alpha,24-dihydroxyvitamin D3). J Dermatol. 2000;27:669–72.PubMedCrossRefGoogle Scholar
  97. 97.
    Moreno AC, Santi CG, Gabbi TV, Aoki V, Hashimoto T, Maruta CW. IgA pemphigus: case series with emphasis on therapeutic response. J Am Acad Dermatol. 2014;70:200–1.PubMedCrossRefGoogle Scholar
  98. 98.
    Niimi Y, Kawana S, Kusunoki T. IgA pemphigus: a case report and its characteristic clinical features compared with subcorneal pustular dermatosis. J Am Acad Dermatol. 2000;43:546–9.PubMedCrossRefGoogle Scholar
  99. 99.
    Wallach D. Intraepidermal IgA pustulosis. J Am Acad Dermatol. 1992;27:993–1000.PubMedCrossRefGoogle Scholar
  100. 100.
    Yasuda H, Kobayashi H, Hashimoto T, Itoh K, Yamane M, Nakamura J. Subcorneal pustular dermatosis type of IgA pemphigus: demonstration of autoantibodies to desmocollin-1 and clinical review. Br J Dermatol. 2000;143:144–8.PubMedCrossRefGoogle Scholar
  101. 101.
    Ruiz-Genao DP, Hernandez-Nunez A, Hashimoto T, Amagai M, Fernandez-Herrera J, Garcia-Diez A. A case of IgA pemphigus successfully treated with acitretin. Br J Dermatol. 2002;147:1040–2.PubMedCrossRefGoogle Scholar
  102. 102.
    Tagami H, Iwatsuki K, Iwase Y, Yamada M. Subcorneal pustular dermatosis with vesiculo-bullous eruption. Demonstration of subcorneal IgA deposits and a leukocyte chemotactic factor. Br J Dermatol. 1983;109:581–7.PubMedCrossRefGoogle Scholar
  103. 103.
    Zillikens D, Miller K, Hartmann AA, Burg G. IgA pemphigus foliaceus: a case report. Dermatologica. 1990;181:304–7.PubMedCrossRefGoogle Scholar
  104. 104.
    Gruss C, Zillikens D, Hashimoto T, et al. Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin. J Am Acad Dermatol. 2000;43:923–6.PubMedCrossRefGoogle Scholar
  105. 105.
    Hodak E, Lapidoth M, David M. Effect of colchicine in the subcorneal pustular dermatosis type of IgA pemphigus. J Am Acad Dermatol. 1999;40:91–4.PubMedCrossRefGoogle Scholar
  106. 106.
    Howell SM, Bessinger GT, Altman CE, Belnap CM. Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. J Am Acad Dermatol. 2005;53:541–3.PubMedCrossRefGoogle Scholar
  107. 107.
    Sibley Hash K, Rencic A, Hernandez MI, Hashimoto T, Nousari HC. Aggressive immunosuppressive therapy for a refractory case of IgA pemphigus. Arch Dermatol. 2002;138:744–6.PubMedCrossRefGoogle Scholar
  108. 108.
    Iskandarli M, Gerceker Turk B, Ertam I, Yaman B, Ozturk G. Successful treatment of Wilson disease-associated IgA pemphigus with IVIG. J Eur Acad Dermatol Venereol. 2016;30:1055–6.PubMedCrossRefGoogle Scholar
  109. 109.
    Bliziotis I, Rafailidis P, Vergidis P, Falagas ME. Regression of subcorneal pustular dermatosis type of IgA pemphigus lesions with azithromycin. J Infect. 2005;51:E31–4.PubMedCrossRefGoogle Scholar
  110. 110.
    Ozawa A, Ohkido M, Haruki Y, et al. Treatments of generalized pustular psoriasis: a multicenter study in Japan. J Dermatol. 1999;26:141–9.CrossRefPubMedGoogle Scholar
  111. 111.
    Zelickson BD, Muller SA. Generalized pustular psoriasis. A review of 63 cases. Arch Dermatol. 1991;127:1339–45.PubMedCrossRefGoogle Scholar
  112. 112.
    Lau BW, Lim DZ, Capon F, Barker JN, Choon SE. Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia. Int J Dermatol. 2017;56(4):392–9.PubMedCrossRefGoogle Scholar
  113. 113.
    Tsang V, Dvorakova V, Enright F, Murphy M, Gleeson C. Successful use of infliximab as first line treatment for severe childhood generalized pustular psoriasis. J Eur Acad Dermatol Venereol. 2016;30:e117–e9.PubMedGoogle Scholar
  114. 114.
    Viguier M, Aubin F, Delaporte E, et al. Efficacy and safety of tumor necrosis factor inhibitors in acute generalized pustular psoriasis. Arch Dermatol. 2012;148:1423–5.PubMedCrossRefGoogle Scholar
  115. 115.
    Matsumoto A, Komine M, Karakawa M, Kishimoto M, Ohtsuki M. Adalimumab administration after infliximab therapy is a successful treatment strategy for generalized pustular psoriasis. J Dermatol. 2016;44(2):202–4.PubMedCrossRefGoogle Scholar
  116. 116.
    Collamer AN, Battafarano DF. Psoriatic skin lesions induced by tumor necrosis factor antagonist therapy: clinical features and possible immunopathogenesis. Semin Arthritis Rheum. 2010;40:233–40.PubMedCrossRefGoogle Scholar
  117. 117.
    Imafuku S, Honma M, Okubo Y, et al. Efficacy and safety of secukinumab in patients with generalized pustular psoriasis: a 52-week analysis from phase III open-label multicenter Japanese study. J Dermatol. 2016;43:1011–7.PubMedCrossRefGoogle Scholar
  118. 118.
    Saeki H, Nakagawa H, Ishii T, et al. Efficacy and safety of open-label ixekizumab treatment in Japanese patients with moderate-to-severe plaque psoriasis, erythrodermic psoriasis and generalized pustular psoriasis. J Eur Acad Dermatol Venereol. 2015;29:1148–55.PubMedCrossRefGoogle Scholar
  119. 119.
    Yamasaki K, Nakagawa H, Kubo Y, Ootaki K, Japanese Brodalumab Study Group. Efficacy and safety of brodalumab in patients with generalized pustular psoriasis and psoriatic erythroderma: results from a 52-week, open-label study. Br J Dermatol. 2016;176(3):741–51.PubMedCrossRefGoogle Scholar
  120. 120.
    Honigsmann H, Gschnait F, Konrad K, Wolff K. Photochemotherapy for pustular psoriasis (von Zumbusch). Br J Dermatol. 1977;97:119–26.PubMedCrossRefGoogle Scholar
  121. 121.
    Hunt MJ, Lee SH, Salisbury EL, Wills EJ, Armati R. Generalized pustular psoriasis responsive to PUVA and oral cyclosporin therapy. Australas J Dermatol. 1997;38:199–201.PubMedCrossRefGoogle Scholar
  122. 122.
    Varman KM, Namias N, Schulman CI, Pizano LR. Acute generalized pustular psoriasis, von Zumbusch type, treated in the burn unit. A review of clinical features and new therapeutics. Burns. 2014;40:e35–9.PubMedCrossRefGoogle Scholar
  123. 123.
    Torii H, Terui T, Matsukawa M, et al. Safety profiles and efficacy of infliximab therapy in Japanese patients with plaque psoriasis with or without psoriatic arthritis, pustular psoriasis or psoriatic erythroderma: results from the prospective post-marketing surveillance. J Dermatol. 2016;43:767–78.PubMedCrossRefGoogle Scholar
  124. 124.
    Shoham NG, Centola M, Mansfield E, et al. Pyrin binds the PSTPIP1/CD2BP1 protein, defining familial Mediterranean fever and PAPA syndrome as disorders in the same pathway. Proc Natl Acad Sci U S A. 2003;100:13501–6.PubMedPubMedCentralCrossRefGoogle Scholar
  125. 125.
    Caorsi R, Picco P, Buoncompagni A, Martini A, Gattorno M. Osteolytic lesion in PAPA syndrome responding to anti-interleukin 1 treatment. J Rheumatol. 2014;41:2333–4.PubMedCrossRefGoogle Scholar
  126. 126.
    Ter Haar N, Lachmann H, Ozen S, et al. Treatment of autoinflammatory diseases: results from the Eurofever Registry and a literature review. Ann Rheum Dis. 2013;72:678–85.PubMedCrossRefGoogle Scholar
  127. 127.
    Demidowich AP, Freeman AF, Kuhns DB, et al. Brief report: genotype, phenotype, and clinical course in five patients with PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne). Arthritis Rheum. 2012;64:2022–7.PubMedCrossRefGoogle Scholar
  128. 128.
    Fathalla BM, Al-Wahadneh AM, Al-Mutawa M, Kambouris M, El-Shanti H. A novel de novo PSTPIP1 mutation in a boy with pyogenic arthritis, pyoderma gangrenosum, acne (PAPA) syndrome. Clin Exp Rheumatol. 2014;32:956–8.PubMedGoogle Scholar
  129. 129.
    Lee H, Park SH, Kim SK, Choe JY, Park JS. Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome (PAPA syndrome) with E250K mutation in CD2BP1 gene treated with the tumor necrosis factor inhibitor adalimumab. Clin Exp Rheumatol. 2012;30:452.PubMedGoogle Scholar
  130. 130.
    Tofteland ND, Shaver TS. Clinical efficacy of etanercept for treatment of PAPA syndrome. J Clin Rheumatol. 2010;16:244–5.PubMedCrossRefGoogle Scholar
  131. 131.
    Geusau A, Mothes-Luksch N, Nahavandi H, et al. Identification of a homozygous PSTPIP1 mutation in a patient with a PAPA-like syndrome responding to canakinumab treatment. JAMA Dermatol. 2013;149:209–15.PubMedCrossRefGoogle Scholar
  132. 132.
    Murphy B, Morrison G, Podmore P. Successful use of adalimumab to treat pyoderma gangrenosum, acne and suppurative hidradenitis (PASH syndrome) following colectomy in ulcerative colitis. Int J Colorectal Dis. 2015;30:1139–40.PubMedCrossRefGoogle Scholar
  133. 133.
    Ursani MA, Appleyard J, Whiteru O. Pyogenic arthritis, pyoderma gangrenosum, acne, suppurative hidradenitis (PA-PASH) syndrome: an atypical presentation of a rare syndrome. Am J Case Rep. 2016;17:587–91.PubMedPubMedCentralCrossRefGoogle Scholar
  134. 134.
    Wargo JJ, Emmer BT. Systemic inflammation gone awry: PASH syndrome and temporomandibular joint ankylosis. Am J Med. 2016;129:e1–3.PubMedCrossRefGoogle Scholar
  135. 135.
    Staub J, Pfannschmidt N, Strohal R, et al. Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone. J Eur Acad Dermatol Venereol. 2015;29:2243–7.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Courtney R. Schadt
    • 1
  • Jeffrey P. Callen
    • 1
  1. 1.Division of DermatologyUniversity of Louisville School of MedicineLouisvilleUSA

Personalised recommendations