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Neutrophilic Dermatoses

A Review of Current Treatment Options

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Abstract

Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic dermatoses – conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. The neutrophils are usually located within the dermis in Sweet syndrome and pyoderma gangrenosum; however, in subcorneal pustular dermatosis, they are found in the upper layers of the epidermis. Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by pyrexia, elevated neutrophil count, painful erythematous cutaneous lesions that have an infiltrate of mature neutrophils typically located in the upper dermis, and prompt clinical improvement following the initiation of systemic corticosteroid therapy. Classical, malignancy-associated, and drug-induced variants of Sweet syndrome exist. Pyoderma gangrenosum is characterized by painful, enlarging necrotic ulcers with bluish undermined borders surrounded by advancing zones of erythema; its clinical variants include: ulcerative or classic, pustular, bullous or atypical, vegetative, peristomal, and drug-induced. Subcorneal pustular dermatosis is an uncommon relapsing symmetric pustular eruption that involves flexural and intertriginous areas; it can be idiopathic or associated with cancer, infections, medications, and systemic diseases. Since Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis share not only the same inflammatory cell but also similar associated systemic diseases, it is not surprising that the concurrent or sequential development of these neutrophilic dermatoses has been observed in the same individual. Also, it is not unexpected that several of the effective therapeutic interventions – including systemic drugs, topical agents, and other treatment modalities – for the management of these dermatoses are the same.

The treatment of choice for Sweet syndrome and idiopathic pyoderma gangrenosum is systemic corticosteroids; however, for subcorneal pustular dermatosis, dapsone is the drug of choice. Yet, tumor necrosis factor-α antagonists are becoming the preferred choice when pyoderma gangrenosum is accompanied by inflammatory bowel disease or rheumatoid arthritis. Potassium iodide and colchicine are alternative first-line therapies for Sweet syndrome and indomethacin (indometacin), clofazimine, cyclosporine (ciclosporin), and dapsone are second-line treatments. Cyclosporine is effective in the acute management of pyoderma gangrenosum; however, when tapering the drug, additional systemic agents are necessary for maintaining the clinical response. In some patients with subcorneal pustular dermatosis, systemic corticosteroids may be effective; yet, systemic retinoids (such as etretinate and acitretin) have effectively been used for treating this neutrophilic dermatosis – either as monotherapy or in combination with dapsone or as a component of phototherapy with psoralen andUVAradiation. Topical agents can have an adjuvant role in themanagement of these neutrophilic dermatoses; however, high-potency topical corticosteroids may successfully treat localized manifestations of Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis. Intralesional corticosteroid therapy for patients with Sweet syndrome and pyoderma gangrenosum, hyperbaric oxygen and plasmapheresis for patients with pyoderma grangrenosum, and phototherapy for patients with subcorneal pustular dermatosis are other modalities that have been used effectively for treating individuals with these neutrophilic dermatoses.

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References

  1. Cohen PR. Neutrophilic dermatoses occurring in oncology patients. Int J Dermatol 2007; 46: 106–11

    Article  PubMed  Google Scholar 

  2. Callen JP. Neutrophilic dermatoses. Dermatol Clin 2002; 20: 409–19

    Article  PubMed  Google Scholar 

  3. Cohen PR. Sweet’s syndrome: a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2007; 2: 34

    Article  PubMed  Google Scholar 

  4. Cohen PR, Almeida L, Kurzrock R. Acute febrile neutrophilic dermatosis. Am Fam Physician 1989; 39 (3): 199–204

    PubMed  CAS  Google Scholar 

  5. Cohen PR, Kurzrock R. Diagnosing the Sweet syndrome. Ann Intern Med 1989; 110: 573–4

    PubMed  CAS  Google Scholar 

  6. Cohen PR, Kurzrock R. Sweet’s syndrome: a neutrophilic dermatosis classically associated with acute onset and fever. Clin Dermatol 2000; 18: 265–82

    Article  PubMed  CAS  Google Scholar 

  7. Cohen PR, Kurzrock R. The pathogenesis of Sweet’s syndrome [letter]. J Am Acad Dermatol 1991; 25: 734

    Article  PubMed  CAS  Google Scholar 

  8. Cohen PR, Kurzrock R. Sweet’s syndrome revisited: a review of disease concepts. Int J Dermatol 2003; 42: 761–78

    Article  PubMed  Google Scholar 

  9. Cohen PR. Preganancy-associated Sweet’s syndrome: world literature review. Obstet Gynecol Survey 1993; 48: 584–7

    Article  CAS  Google Scholar 

  10. Satra KH, Zalka A, Cohen PR, et al. Sweet’s syndrome and pregnancy. J Am Acad Dermatol 1994; 30: 297–300

    Article  PubMed  CAS  Google Scholar 

  11. Cohen PR, Kurzrock R. Sweet’s syndrome and cancer. Clin Dermatol 1993; 11: 149–57

    Article  PubMed  CAS  Google Scholar 

  12. Cohen PR, Talpaz M, Kurzrock R. Malignancy-associated Sweet’s syndrome: review of the world literature. J Clin Oncol 1988; 6: 1887–97

    PubMed  CAS  Google Scholar 

  13. Cohen PR, Kurzrock R. Chonic myelogenous leukemia and Sweet syndrome. Am J Hematol 1989; 32: 134–7

    Article  PubMed  CAS  Google Scholar 

  14. Cohen PR, Kurzrock R. Paraneoplastic Sweet’s syndrome. Emerg Med 1994; 26 (2): 37–8

    Google Scholar 

  15. Cohen PR, Kurzrock R. Sweet’s syndrome and malignancy. Am J Med 1987; 82: 1220–6

    Article  PubMed  CAS  Google Scholar 

  16. Cohen PR, Holder WR, Tucker SB, et al. Sweet syndrome in patients with solid tumors. Cancer 1993; 72: 2723–31

    Article  PubMed  CAS  Google Scholar 

  17. 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

    Article  PubMed  CAS  Google Scholar 

  18. Cohen PR. Skin lesions of Sweet syndrome and its dorsal hand variant contain vasculitis: an oxymoron or an epiphenomenon? Arch Dermatol 2002; 138: 400–3

    Article  PubMed  Google Scholar 

  19. Cohen PR. Subcutaneous Sweet’s syndrome: a variant of acute febrile neutrophilic dermatosis that is included in the histopathologic differential diagnosis of neutrophilic panniculitis. J Am Acad Dermatol 2005; 52: 927–8

    Article  PubMed  Google Scholar 

  20. Cohen PR, Holder WR, Rapini RP. Concurrent Sweet’s syndrome and erythema nodosum: a report, world literature review and mechanism of pathogenesis. J Rheumatol 1992; 19: 814–20

    PubMed  CAS  Google Scholar 

  21. Cohen PR. Sweet’s syndrome presenting as conjunctivitis. Arch Ophthalmol 1993; 111: 587–8

    Article  PubMed  CAS  Google Scholar 

  22. Cohen PR, Kurzrock R. Extracutaneous manifestations of Sweet’s syndrome: steroid-responsive culture-negative pulmonary lesions [letter]. Am Rev Respir Dis 1992; 146: 269

    PubMed  CAS  Google Scholar 

  23. Cohen PR. Sweet syndrome and erythema nodosum [letter]. South Med J 2007; 100: 1057–8

    Article  PubMed  Google Scholar 

  24. Cohen PR. Sweet’s syndrome and relapsing polychondritis: is their appearance in the same patient a coincidental occurrence or a bona fide association of these conditions? Int J Dermatol 2004; 43: 772–7

    Article  PubMed  Google Scholar 

  25. Callen JP, Jackson JM. Pyoderma gangrenosum: an update. Rheum Dis Clin North Am 2007; 33: 787–803

    Article  PubMed  Google Scholar 

  26. Brooklyn T, Dunnill G, Probert C. Diagnosis and treatment of pyoderma gangrenosum. BMJ 2006; 333: 181–4

    Article  PubMed  Google Scholar 

  27. Wollina U. Pyoderma gangrenosum: a review. Orphanet J Rare Dis 2007; 2: 19

    Article  PubMed  Google Scholar 

  28. Powell FC, Collins S. Pyoderma gangrenosum. Clin Dermatol 2000; 18: 283–93

    Article  PubMed  CAS  Google Scholar 

  29. Crowson AN, Mihm Jr MC, Magro C. Pyoderma gangrenosum: a review. J Cutan Pathol 2003; 30: 97–107

    Article  PubMed  Google Scholar 

  30. Reed J, Wilkinson J. Subcorneal pustular dermatosis. Clin Dermatol 2000; 18: 301–13

    Article  PubMed  CAS  Google Scholar 

  31. Bordignon M, Zattra E, Montesco MC, et al. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with absence of desmoglein 1 and 3 antibodies case report and literature review. Am J Clin Dermatol 2008; 9: 51–5

    Article  PubMed  Google Scholar 

  32. Cheng S, Edmonds E, Ben-Gashir M, et al. Subcorneal pustular dermatosis: 50 years on. Clin Exp Dermatol 2008; 33: 229–33

    Article  PubMed  CAS  Google Scholar 

  33. Castro-Fernandez M, Sanchez-Munos D, Ruiz-Granados E, et al. Coexistence of pyoderma gangrenosum and Sweet’s syndrome in a patient with ulcerative colitis [letter]. Am J Gastroenterol 2007; 102: 2865–6

    Article  PubMed  Google Scholar 

  34. Caughman W, Stern R, Haynes H. Neutrophilic dermatosis of myeloproliferative disorders. J Am Acad Dermatol 1983; 9: 751–8

    Article  PubMed  CAS  Google Scholar 

  35. Lear JT, Byrne JPH. Bullous pyoderma gangrenosum, Sweet’s syndrome and malignancy [letter]. Br J Dermatol 1997; 136: 296–7

    Article  PubMed  CAS  Google Scholar 

  36. Salmon P, Rademaker M, Edwards L. A continuum of neutrophilic disease occurring in a patient with ulcerative colitis. Australas J Dermatol 1998; 39: 116–8

    Article  PubMed  CAS  Google Scholar 

  37. Wilson DM, John GR, Callen JP. Peripheral ulcerative keratitis: an extracutaneous neutrophilic disorder. Report of a patient with rheumatoid arthritis, pustular vasculitis, pyoderma gangrenosum, and Sweet’s syndrome with an excellent response to cyclosporine therapy. J Am Acad Dermatol 1999; 40: 331–4

    CAS  Google Scholar 

  38. Marsden JR, Millard LG. Pyoderma gangrenosum, subcorneal pustular dermatosis and IgA paraproteinaemia. Br J Dermatol 1986; 114: 125–9

    Article  PubMed  CAS  Google Scholar 

  39. Kohl PK, Hartschuh W, Tilgen W, et al. Pyoderma gangrenosum followed by subcorneal pustular dermatosis in a patient with IgA paraproteinemia. J Am Acad Dermatol 1991; 24: 325–8

    Article  PubMed  CAS  Google Scholar 

  40. Cartier H, Plantin P, Leroy JP, et al. Pyoderma gangrenosum, subcorneal IgA pustulosis and recurrent neutrophilic pleural and pulmonary disease in a patient with IgA gammopathy. Ann Dermatol Venereol (Paris) 1995; 122: 97–101

    CAS  Google Scholar 

  41. Freire Murgueytio P, Allegue F, Martin Gonzalez M, et al. Gangrenous pyoderma associated with subcorneal pustular dermatosis (Sneddon-Wilkinson disease). Med Cutan Ibero Lat Am 1989; 17: 105–9

    PubMed  CAS  Google Scholar 

  42. Venning VA, Ryan TJ. Subcorneal pustular dermatosis followed by pyoderma gangrenosum. Br J Dermatol 1986; 115: 117–8

    Article  PubMed  CAS  Google Scholar 

  43. Puechguiral-Renaud I, Carpentier O, Piette F, et al. Subcorneal pustulosis and pyoderma gangrenosum associated with a biclonal gammopathy. Eur J Dermatol 2006; 16: 687–90

    PubMed  CAS  Google Scholar 

  44. Scerri L, Zaki I, Allen BR. Pyoderma gangrenosum and subcorneal pustular dermatosis, without monoclonal gammopathy. Br J Dermatol 1994; 130: 398–9

    Article  PubMed  CAS  Google Scholar 

  45. Wolff K. Subcorneal pustulose dermatose (Sneddon-Wilkinson): pyoderma gangrenosum mit IgA paraproteinamie [abstract]. Dermatol Wocherschrift 1971; 157: 842

    Google Scholar 

  46. Dallot A, Decazes JM, Drouault Y, et al. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with amicrobial lymph node suppuration and aseptic spleen abscess. Br J Dermatol 1988; 119: 803–7

    Article  PubMed  CAS  Google Scholar 

  47. Cohen PR, Kurzrock R. Sweet’s syndrome: a review of current treatment options. Am J Clin Dermatol 2002; 3: 117–31

    Article  PubMed  Google Scholar 

  48. Cohen PR, Holder WR. Pentoxifylline for Sweet’s syndrome. J Am Acad Dermatol 1995; 32: 533–4

    Article  PubMed  CAS  Google Scholar 

  49. Cohen PR, Kurzrock R. Treatment of Sweet’s syndrome [letter]. Am J Med 1990; 89: 396

    Article  PubMed  CAS  Google Scholar 

  50. Delluc A, Limal N, Puechal X, et al. Efficacy of anakinra, an IL1 receptor antagonist, in refractory Sweet syndrome. Ann Rheum Dis 2008; 67: 278–9

    Article  PubMed  CAS  Google Scholar 

  51. Reichrath J, Bens G, Bonowitz A, et al. 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

    Article  PubMed  Google Scholar 

  52. Gettler S, Rothe M, Grin C, et al. Optimal treatment of pyoderma gangrenosum. Am J Clin Dermatol 2003; 4: 597–608

    Article  PubMed  Google Scholar 

  53. Fonder MA, Cummins DL, Ehst BD, et al. Adalimumab therapy for recalcitrant pyoderma gangrenosum. J Burns Wounds 2006; 5: e8

    Google Scholar 

  54. Juillerat P, Christen-Zach S, Troillet F-X, et al. Infliximab for the treatment of disseminated pyoderma gangrenosum associated with ulcerative colitis: case report and literature review. Dermatology 2007; 215: 245–51

    Article  PubMed  Google Scholar 

  55. Chiba T, Isomura I, Suzuki A, et al. Topical tacrolimus therapy for pyoderma gangrenosum. J Dermatol 2005; 32: 199–203

    PubMed  Google Scholar 

  56. Foss CE, Clark AR, Inabinet R, et al. An open-label pilot study of alefacept for the treatment of pyoderma gangrenosum. J Eur Acad Dermatol Venereol 2008 Aug; 22: 943–9

    Article  PubMed  CAS  Google Scholar 

  57. Meyer N, Ferraro V, Mignard M-H, et al. Pyoderma gangrenosum treated with high-dose intravenous immunoglobulins: two cases and review of the literature. Clin Drug Invest 2006; 26: 541–6

    Article  CAS  Google Scholar 

  58. Cummins DL, Anhalt GJ, Monahan T, et al. Treatment of pyoderma gangrenosum with intravenous immunoglobulin. Br J Dermatol 2007; 157: 1235–9

    Article  PubMed  CAS  Google Scholar 

  59. Wenzel J, Gerdsen R, Phillipp-Dormston W, et al. Topical treatment of pyoderma gangrenosum. Dermatology 2002; 205: 221–3

    Article  PubMed  CAS  Google Scholar 

  60. Tutrone WD, Green K, Weinberg JM, et al. Pyoderma gangrenosum: dermatologic application of hyperbaric oxygen therapy. J Drugs Dermatol 2007; 6: 1214–9

    PubMed  Google Scholar 

  61. Marliere V, Beylot-Barry M, Beylot C, et al. Successful treatment of subcorneal pustular dermatosis (Sneddon-Wilkinson disease) by acitretin: report of a case. Dermatology 1999; 199: 153–6

    Article  PubMed  CAS  Google Scholar 

  62. Voigtlander C, Luftl M, Schuler G, et al. 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

    PubMed  CAS  Google Scholar 

  63. Bonifati C, Trento E, Cordiali Fei P, et al. Early but not lasting improvement of recalcitrant subcorneal pustular dermatosis (Sneddon-Wilkinson disease) after infliximab therapy: relationships with variations in cytokine levels in suction blister fluids. Clin Exp Dermatol 2005; 30: 662–5

    Article  PubMed  CAS  Google Scholar 

  64. Kawaguchi M, Mitsuhashi Y, Kondo S. A case of subcorneal pustular dermatosis treated with tacalcitol (1alpha, 24-dihydroxyvitaminD3). J Dermatol 2000; 27: 669–72

    PubMed  CAS  Google Scholar 

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Acknowledgments

No sources of funding were used to assist in the preparation of this review. The author has no conflicts of interest that are directly relevant to the content of this review.

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Authors and Affiliations

  1. The University of Houston Health Center, University of Houston, Houston, Texas, USA

    Philip R. Cohen

  2. The Department of Dermatology, University of Texas-Houston Medical School, Houston, Texas, USA

    Philip R. Cohen

  3. The Department of Dermatology, University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA

    Philip R. Cohen

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Correspondence to Philip R. Cohen.

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Cohen, P.R. Neutrophilic Dermatoses. AM J Clin Dermatol 10, 301–312 (2009). https://doi.org/10.2165/11310730-000000000-00000

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