Skip to main content

An Histological Approach to the Diagnosis of Neutrophilic Dermatoses

  • Chapter
  • First Online:
Neutrophilic Dermatoses

Abstract

A broad analysis of histological lesions delineate the history of the neutrophilic dermatoses. In all steps of the description of these diseases, the cutaneous biopsy has been essential to ascertain their specificity. Moreover, some of them have been distinguished in accordance with their histological presentation.

The cutaneous biopsy is also one of the major criteria to group dermatoses that are clinically completely different with the common denominator of an aseptic infiltration by neutrophils. They comprise Sweet syndrome, pyoderma gangrenosum, subcorneal pustulosis and erythema elevatum diutinum. Many other dermatoses are characterized by a cutaneous infiltration by neutrophilic have been recognized later and linked to this group.

Skin biopsy is always required for the diagnosis and classification of these rare dermatoses. Finally, it is essential to understand the mechanisms which contribute to cutaneous infiltration by neutrophils.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Wallach D, Vignon-Pennamen MD. Pyoderma gangrenosum and Sweet syndrome: the prototypic neutrophilic dermatoses. Br J Dermatol. 2015. https://doi.org/10.1111/bjd.13955.

  2. Wallach D, Vignon Pennamen MD. From acute febrile neutrophilic dermatosis to neutrophilic disease: forty years of clinical research. J Am Acad Dermatol. 2006;55:1066–71.

    Article  Google Scholar 

  3. Cheng S, Edmonds E, Ben-Gashir M, Yu RC. Subcorneal pustular dermatosis: 50 years on. Clin Exper Dermatol. 2008;33:229–33.

    Article  CAS  Google Scholar 

  4. Wallach D. Intraepidermal IgA pustulosis. J Am Acad Dermatol. 1992;27:993–1000.

    Article  CAS  Google Scholar 

  5. Koester G, Tarnower A, Levisohn D, Burgdorf W. Bullous pyoderma gangrenosum. J Am Acad Dermatol. 1993;29:875–8.

    Article  CAS  Google Scholar 

  6. Boms S, Gambichler T. Review of literature on amicrobial pustulosis of the folds associated with autoimmune disorders. Am J Clin Dermatol. 2006;7:369–74.

    Article  Google Scholar 

  7. Shangar S, Sterling JC, Rytina E. Pustular pyoderma gangrenosum. Clin Exper Dermatol. 2003;28:600–3.

    Article  Google Scholar 

  8. Naik HB, Cowen EW. Autoinflammatory pustular neutrophilic diseases. Dermatol Clin. 2013;31:405–25.

    Article  CAS  Google Scholar 

  9. Jordaan HF. Acute febrile neutrophilic dermatosis. A histopathological study of 37 patients and review of the literature. Am J Dermatopathol. 1989;11:99–11.

    Article  CAS  Google Scholar 

  10. Ratzinger G, Burgdorf W, Zelger BG, Zelger B. Acute febrile neutrophilic dermatosis: a histopathologic study of 31 cases with review of literature. Am J Dermatopathol. 2007;29:125–33.

    Article  Google Scholar 

  11. Malone JC, Slone SP, Wills-Frank LA, Fearneyhough PK, Lear SC, Jane Goldsmith L, Hood AF, Callen JP. Vascular inflammation (vasculitis) in Sweet syndrome. A clinicopathologic study of 28 biopsy specimens from 21 patients. Arch Dermatol. 2002;138:345–9.

    Article  Google Scholar 

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

    Article  Google Scholar 

  13. Bachmeyer C, Aractingi S. Neutrophilic eccrine hidradenitis. Clin Dermatol. 2000;18:319–30.

    Article  CAS  Google Scholar 

  14. Magro CM, Crowson AN. The spectrum of cutaneous lesions in rheumatoid arthritis: a clinical and pathological study of 43 patients. J Cutan Pathol. 2003;30:1–10.

    Article  CAS  Google Scholar 

  15. Leboit PE, Yen TSB, Wintroub B. The evolution of lesions in erythemaelevatum diutinum. Am J Dermatopathol. 1986;8:392–402.

    Article  CAS  Google Scholar 

  16. Wilson-Jones E, Winkelmann RK. Superficial granulomatous pyoderma: a localized vegetative form of pyoderma gangrenosum. J Am Acad Dermatol. 1988;18:511–21.

    Article  CAS  Google Scholar 

  17. Karadogan SK, Baskan EB, Alkan G, Saricaoglu H, Tunali S. Generalized Sweet syndrome lesions associated with Behçet disease: a true association or simply co-morbidity? Am J Clin Dermatol. 2009;10:331–5.

    Article  Google Scholar 

  18. Kieffer C, Cribier B, Lipsker D. Neutrophilic urticaial dermatosis: a variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 new cases and review of the literature. Medicine (Baltimore). 2009;88:23–31.

    Article  Google Scholar 

  19. Larson AR, Laga AC, Granter SR. The spectrum of histopathologic findings in cutaneous lesions in patients with Still disease. Am J Clin Pathol. 2015;144:945–51.

    Article  CAS  Google Scholar 

  20. Wilkerson A, King R, Googe PB, Page RN, Fulk CS. Sweet’s syndrome-like blastomycosis. Am J Dermatopathol. 2003;25:152–4.

    Article  Google Scholar 

  21. Haydee Chacon A, Farooq U, Shiman MI, Nolan B, Elgart GW. Cutaneous aspergillosis masquerading as Sweet’s syndrome in a patient with acute myelogenous leukemia. J Cutan Pathol. 2013;40:66–8.

    Article  Google Scholar 

  22. Ko JS, Fernandez AP, Anderson KA, Burdick LM, Billings SD, Procop GW, McMahon JT, Bergfeld WF, Piliang MP. Morphologic mimickers of Cryptococcus occurring within inflammatory infiltrates in the setting of neutrophilic dermatitis: a series of three cases highlighting clinical dilemmas associated with a novel histopathologic pitfall. J Cutan Pathol. 2013;40:38–45.

    Article  Google Scholar 

  23. Byekova YA, Shedd AD, Schiro JA, Barrett TL, Lewin MR. An additional case of neutrophilic dermatosis histopathogically mimicking Cryptococcus in a patient with Sweet’s syndrome. J Cutan Pathol. 2014;41:972–4.

    Article  Google Scholar 

  24. Wilson TC, Stone MS, Swick BL. Histiocytoid Sweet syndrome with haloed myeloid cells masquerading as a cryptococcal infection. Am J Dermatopathol. 2014;36:264–9.

    Article  Google Scholar 

  25. Gulati A, Paige D, Yaqoob M, Proby CM, Cerio R, Harwood CA. Palisaded neutrophilic granulomatous dermatitis associated with systemic lupus erythematosus presenting with the burning rope sign. J Am Acad Dermatol. 2009;61:711–4.

    Article  Google Scholar 

  26. de Unamuno Bustos B, Rabasco AG, de Miquel VA. Palisaded neutrophilic and granulomatous dermatitis associated with ankylosing spondylitis. Am J Dermatopathol. 2013;35:847–50.

    Article  Google Scholar 

  27. Hu CH, O’Loughlin S, Winkelmann RK. Cutaneous manifestations of Wegener granulomatosis. Arch Dermatol. 1977;113:175–82.

    Article  CAS  Google Scholar 

  28. Stephenson SR, Campbell SM, Drew GS, Magro CM. Palisaded neutrophilic and granulomatous dermitis presenting in a patient with rheumatoid arthritis on adalimumab. J Cutan Pathol. 2011;38:644–8.

    Article  Google Scholar 

  29. Rochael MC, Pantaleao L, Guedes Vilar EA, Zacaron LH, Quelhas Spada E, Xavier MH, Strong Rodrigues KS. Sweet’s syndrome; study of 73 cases emphasizing histopathological findings. An Bras Dermatol. 2011;86:278–83.

    Article  Google Scholar 

  30. Battistella M, Bourrat E, Fardet L, Saada V, Janin A, Vignon-Pennamen MD. Sweet-like reaction due to arthropod bites: a histopathologic pitfall. Am J Dermatopathol. 2012;34:442–5.

    Article  Google Scholar 

  31. Ortonne N, Wechsler J, Bagot M, Grosshans E, Cribier B. Granuloma faciale: a clinicopathologic study of 66 patients. J Am Acad Dermatol. 2005;53:1002–9.

    Article  Google Scholar 

  32. Larson AR, Granter SR. Systemic lupus erythrmatosus-associated neutrophilic dermatosis. An underrecognized neutrophilic dermatosis in patients with systemic lupus erythematosus. Hum Pathol. 2014;45:598–605.

    Article  Google Scholar 

  33. Hau E, Vignon Pennamen MD, Battistella M, Saussine A, Bergis M, Cavelier-Balloy B, Janier M, Cordoliani F, Bagot M, Rybojad M, Bouaziz JD. Neutrophilic skin lesions in autoimmune connective tissue diseases. 9 cases and a literature review. Medicine. 2014;93:e346.

    Article  CAS  Google Scholar 

  34. Vignon Pennamen MD, Juillard C, Rybojad M, Wallach D, Daniel MT, Morel P, Verola O, Janin A. Chronic recurrent lymphocytic Sweet syndrome as a predictive marker of myelodysplasia. Arch Dermatol. 2006;142:1170–6.

    Article  Google Scholar 

  35. Kulasekararaj AG, Kordasti S, Basu T, Salisbury JR, Mufti GJ, du Vivier AWP. Chronic relapsing remitting Sweet syndrome – a harbinger of myelodysplastic syndrome. Br J Haematol. 2015;170:649–56.

    Article  CAS  Google Scholar 

  36. Requena L, Kutzner H, Palmedo G, Pascual M, Fernandez-Herrera J, Fraga J, Garcia-Diez A, Sanchez Yus E. Histiocytoid Sweet syndrome. A dermal infiltration of immature neutrophilic granulocytes. Arch Dermatol. 2005;141:834–42.

    Article  Google Scholar 

  37. Delabie J, De Wolf-Peeters C, Morren M, Marien K, Roskams T, Desmet V. Histiocytes in Sweet’s syndrome. Br J Dermatol. 1991;124:348–53.

    Article  CAS  Google Scholar 

  38. Morioka N, Otsuka F, Nogita T, Igisu K, Urabe A, Ishibashi Y. Neutrophilic dermatosis with myelodysplastic syndrome: nuclear segmentation anomalies of neutrophils in the skin lesion and in peripheral blood. J Am Acad Dermatol. 1990;23:247–9.

    Article  CAS  Google Scholar 

  39. Chow S, Pasternak S, Green P, Tremaine R, Reardon M, Murray S, Northgrave S, Walsh N. Histiocytoid neutrophilic dermatoses and panniculitides: variations on a theme. Am J Dermatopathol. 2007;29:334–41.

    Article  Google Scholar 

  40. Chavan RN, Cappel MA, Ketterling RP, Wada DA, Rochet NM, Knudson R, Gibson LE. Histiocytoid Sweet syndrome may indicate leukemia cutis: a novel application of fluorescent in situ hybridization. J Am Acad Dermatol. 2014;70:1021–7.

    Article  Google Scholar 

  41. Magro CM, Momtahen S, Nguyen GH, Wang X. Histiocytoid Sweet’s syndrome: a localized cutaneous proliferation of macrophages frequently associated with chronic myeloproliferative disease. Eur J Dermatol. 2015;25:335–41.

    CAS  PubMed  Google Scholar 

  42. Peroni A, Colato C, Schena D, Rongioletti F, Girolomoni G. Histiocytoid Sweet syndrome is infiltrated predominantly by M2-like macrophages. J Am Acad Dermatol. 2015;72:131–9.

    Article  CAS  Google Scholar 

  43. Bush JW, Wick MR. Cutaneous histiocytoid Sweet syndrome and its relationship to hematological diseases. J Cutan Pathol. 2016;43:394–9.

    Article  Google Scholar 

  44. Ghoufi L, Ortonne N, Ingen-Housz-Oro S, Barhoumi W, Begon E, Haioun C, Pautas C, Beckerich F, Robin C, Wolkenstein P, Cordonnier C, Chosidow O, Toma A. Histiocytoid Sweet syndrome is more frequently associated with myelodysplastic syndromes than the classical neutrophilic variant. A comparative series of 62 patients. Medicine. 2016;95:e3033.

    Article  CAS  Google Scholar 

  45. Alegria-Landa V, Rodriguez-Pinilla SM, Santos-Briz A, Rodriguez-Peralto JL, Alegre V, Cerroni L, Kutzner H, Requena L. Clinicopathologic, immunohistochemical, and molecular features of histocytoid Sweet syndrome. JAMA Dermatol. 2017;153:651–9.

    Article  Google Scholar 

  46. Osio A, Battistella M, Feugeas JP, Cuccuini W, Petrella T, Raffoux E, Janin A, Vignon Pennamen MD. Myelodysplasia cutis versus leukemia cutis. J Invest Dermatol. 2015;135:2321–4.

    Article  CAS  Google Scholar 

  47. Van Loon K, Gill RM, McMahon P, Chigurupati R, Siddiqi I, Fox L, Damon L, McCalmont TH, Jordan R, Wolf J. 20q-clonality in a case of oral Sweet syndrome and myelodysplasia. Am J Clin Pathol. 2012;137:310–5.

    Article  Google Scholar 

  48. Sujobert P, Cuccuini W, Vignon-Pennamen D, Martin-Garcia N, Albertini AF, Uzunov M, Redjoul R, Dombret H, Raffoux E. Evidence of differenciation in myeloid malignancies associated neutrophilic dermatosis: a fluorescent in situ hybridization study of 14 patients. J Invest Dermatol. 2013;133:1111–4.

    Article  CAS  Google Scholar 

  49. Haga N, Iwata H, Yamaguchi Y, Shirato T, Nishimura K, Yamane N, Shinkuma S, Natsuga K, Kondo T, Shimizu H. Mucocutaneous pyoderma gangrenosum due to trisomy 8 neutrophilic infiltrates in a patient with myelodysplastic syndrome. Br J Dermatol. 2016;174:239–41.

    Article  Google Scholar 

  50. Lewis KG, Dill SW, Wilkel CS, Robinson-Bostom L. Mid-dermal elastolysis preceded by acute neutrophilic dermatosis. J Cutan Pathol. 2004;31:72–6.

    Article  Google Scholar 

  51. Webb K, Hlela C, Jordaan HF, Sulimlan S, Scriba T, Lipsker D, Scott C. A review and proposal approach to the neutrophilic dermatoses of childhood. Pediatr Dermatol. 2015;32:437–46.

    Article  Google Scholar 

  52. Weening RH, Davis MD, Dahl PR, Su WP. Skin ulcers misdiagnosed as pyoderma gangrenosum. N Engl J Med. 2002;347:1412–8.

    Article  Google Scholar 

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

    Article  Google Scholar 

  54. Sutra-Loubet C, Carlotti A, Guillemette J, Wallach D. Neutrophilic panniculitis. J Am Acad Dermatol. 2004;50:280–5.

    Article  Google Scholar 

  55. Becherer K, Golda N, Feldman M, Diaz-Arias A, Caldwell C. Neutrophilic panniculitis associated with myelodysplastic syndrome with abnormal nuclear forms. J Cutan Pathol. 2009;36:1024–6.

    Article  Google Scholar 

  56. Chan MP, Duncan LM, Nazarian RM. Subcutaneous Sweet syndrome in the setting of myeloid disorders: a case series and review of the literature. J Am Acad Dermatol. 2013;68:1006–15.

    Article  Google Scholar 

  57. Andre M, Piette JC, Kemeny JL, Ninet J, Jego P, Delevaux I, Wechsler B, Weiller PJ, Frances C, Bletry O, Wismans PJ, Rousset H, Colombel JF, Aumaître O, French Study Group on Aseptic Abscesses. Aseptic abscesses: a study of 30 patients with or without inflammatory bowel disease and review of the literature. Medicine. 2007;86:145–61.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Marie-Dominique Vignon-Pennamen M.D. .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Vignon-Pennamen, MD. (2018). An Histological Approach to the Diagnosis of Neutrophilic Dermatoses. In: Wallach, D., Vignon-Pennamen, MD., Valerio Marzano, A. (eds) Neutrophilic Dermatoses. Springer, Cham. https://doi.org/10.1007/978-3-319-72649-6_15

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-72649-6_15

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-72648-9

  • Online ISBN: 978-3-319-72649-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics