eMedicine Specialties > Dermatology > Diseases of the Adnexa

Neutrophilic Eccrine Hidradenitis

Author: Joseph C Pierson, MD, Consulting Staff, Department of Dermatology, Keller Army Community Hospital
Coauthor(s): Christine C Tam, MD,
Contributor Information and Disclosures

Updated: Aug 31, 2009

Introduction

Background

Neutrophilic eccrine hidradenitis (NEH) was initially described in acute myelogenous leukemia (AML) patients undergoing chemotherapy.1 Neutrophilic eccrine hidradenitis has since been reported in persons with various neoplastic and nonneoplastic conditions and in otherwise healthy individuals; however, most documented cases have continued to be observed in the setting of AML, usually in association with chemotherapy; the name toxic erythema of chemotherapy has been proposed.2 Patients with this uncommon, self-limited condition usually present with fever and nonspecific cutaneous lesions. A skin biopsy specimen demonstrating characteristic pathologic changes of the eccrine glands is required to confirm a diagnosis of neutrophilic eccrine hidradenitis.

Also see the eMedicine articles Acute Myelogenous Leukemia and Chronic Myelogenous Leukemia.

Pathophysiology

The mechanism(s) of neutrophilic eccrine hidradenitis is unknown, although neutrophilic eccrine hidradenitis pathologic changes observed with intradermal bleomycin injections support a direct toxic effect of chemotherapy. More than 70% of oncology patients who develop neutrophilic eccrine hidradenitis do so after their first course of chemotherapy.3 Cases linked to chemotherapeutic agents have developed at a wide range of 2 days to 2 years after initiation. Some patients experience recurrences of the cutaneous eruption upon reintroduction of the chemotherapeutic regimens. A diagnosis of toxic erythema of chemotherapy has been proposed for this group of disorders, to emphasize the overlapping clinical and histological features with similar eruptions.2

Reports of neutrophilic eccrine hidradenitis heralding the onset of both AML4 and chronic myelogenous leukemia,5 the relapse of AML,6 and being induced by granulocyte colony-stimulating factor7  suggest that the condition is in the spectrum of other neutrophilic dermatoses that have been observed in patients with cancer: erythema elevatum diutinum, intraepidermal immunoglobulin A (IgA) pustulosis, pyoderma gangrenosum, subcorneal pustular dermatosis, Sweet syndrome (and it localized variant, neutrophilic dermatosis/pustular vasculitis of the dorsal hand), and vasculitis. The inflammatory infiltrate of mature polymorphonuclear leukocytes is the unifying characteristic of this group of conditions.3  

Cases of neutrophilic eccrine hidradenitis in otherwise healthy individuals,8 with acetaminophen use,9 with cyclophosphamide therapy for lupus,10 with methotrexate use for actinic reticuloid syndrome,11  in Behçet disease,12 and in infections (HIV, Serratia, Enterobacter, Nocardia, Staphylococcus, Streptococcus, Pseudomonas) suggest it could simply be an altered inflammatory response to nonspecific stimuli. Neutrophilic eccrine hidradenitis in young children may be triggered by thermal damage of eccrine glands.13

Frequency

International

The frequency of neutrophilic eccrine hidradenitis is unknown.

Mortality/Morbidity

Neutrophilic eccrine hidradenitis is typically a self-limited process. It does not appear to portend a worse prognosis for the underlying malignancy when occurring in that setting.

Sex

A slight male predominance is found in cases of neutrophilic eccrine hidradenitis.14

Age

Neutrophilic eccrine hidradenitis has been reported in individuals as young as 6 months and as old as 79 years.

Clinical

History

Most reported cases of neutrophilic eccrine hidradenitis (NEH) have been in patients with AML who are undergoing chemotherapy, frequently with cytarabine. Granulocytopenia may be found in such cases. Other malignancy and chemotherapy associations exist. As noted previously, cases have been documented in AML and chronic myelogenous leukemia patients who were not on chemotherapy. Some otherwise healthy individuals have inexplicably developed biopsy-proven lesions of neutrophilic eccrine hidradenitis. Regardless of the clinical setting, patients with neutrophilic eccrine hidradenitis develop skin lesions and frequently report fever. Half of the patients are asymptomatic, but pain and tenderness are not uncommon.

Physical

The cutaneous lesions of neutrophilic eccrine hidradenitis are protean. Neutrophilic eccrine hidradenitis lesions may be solitary or multiple. Erythematous or purpuric macules, papules, nodules, or plaques are described most frequently. Hyperpigmented plaques, annular lesions,15 and sclerodermoid changes16 have also been noted. Tenderness may be elicited. The trunk or limbs are most often involved. Neutrophilic eccrine hidradenitis simulating orbital cellulitis,17 facial cellulitis,18 and symmetrical ear swelling19 have been documented.

Courtesy of Jeffrey P. Callen, MD.

Courtesy of Jeffrey P. Callen, MD.

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Courtesy of Jeffrey P. Callen, MD.

Courtesy of Jeffrey P. Callen, MD.

Causes

The cause of neutrophilic eccrine hidradenitis is unknown. A direct toxic effect of chemotherapy and a paraneoplastic mechanism have both been proposed to explain neutrophilic eccrine hidradenitis in the context of malignancy. Cases of neutrophilic eccrine hidradenitis resolving after withdrawal of chemotherapy and recurring upon reinstitution of the same regimen favor the former. Also supporting a direct toxic drug response is a study showing that the intradermal injection of bleomycin can yield local neutrophilic eccrine hidradenitis changes.20 However, skin lesions arising after chemotherapy have developed anywhere from 2 days to 2 years later. A diagnosis of toxic erythema of chemotherapy has been proposed for this group of disorders, to emphasize the overlapping clinical and histologic features with similar eruptions.2
 
Favoring a paraneoplastic process are case reports of neutrophilic eccrine hidradenitis heralding the onset of both AML4 and chronic myelogenous leukemia5 and the relapse of AML.6 Neutrophilic eccrine hidradenitis has also been observed in otherwise healthy individuals8 ; in Behçet disease12 ; with acetaminophen9 ; with granulocyte colony-stimulating factor7 ; with cyclophosphamide therapy for lupus10 ; with methotrexate therapy for actinic reticuloid11 ; and with HIV, Serratia, Enterobacter, Nocardia, Staphylococcus, Streptococcus, and Pseudomonas infections.

More on Neutrophilic Eccrine Hidradenitis

Overview: Neutrophilic Eccrine Hidradenitis
Differential Diagnoses & Workup: Neutrophilic Eccrine Hidradenitis
Treatment & Medication: Neutrophilic Eccrine Hidradenitis
Follow-up: Neutrophilic Eccrine Hidradenitis
Multimedia: Neutrophilic Eccrine Hidradenitis
References
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References

  1. Harrist TJ, Fine JD, Berman RS, Murphy GF, Mihm MC Jr. Neutrophilic eccrine hidradenitis. A distinctive type of neutrophilic dermatosis associated with myelogenous leukemia and chemotherapy. Arch Dermatol. Apr 1982;118(4):263-6. [Medline].

  2. Bolognia JL, Cooper DL, Glusac EJ. Toxic erythema of chemotherapy: a useful clinical term. J Am Acad Dermatol. Sep 2008;59(3):524-9. [Medline].

  3. Cohen PR. Neutrophilic dermatoses occurring in oncology patients. Int J Dermatol. Jan 2007;46(1):106-11. [Medline].

  4. Pierson JC, Helm TN, Taylor JS, Elston DM, Tuthill RJ. Neutrophilic eccrine hidradenitis heralding the onset of acute myelogenous leukemia. Arch Dermatol. Jun 1993;129(6):791-2. [Medline].

  5. Gomez Vazquez M, Peteiro C, Toribio J. Neutrophilic eccrine hidradenitis heralding the onset of chronic myelogenous leukaemia. J Eur Acad Dermatol Venereol. May 2003;17(3):328-30. [Medline].

  6. Saada V, Aractingi S, Leblond V, Marinho E, Frances C, Chosidow O. [Neutrophilic eccrine hidradenitis associated with relapse of acute myeloblastic leukemia]. Ann Dermatol Venereol. Jul 1998;125(6-7):420-2. [Medline].

  7. Bachmeyer C, Chaibi P, Aractingi S. Neutrophilic eccrine hidradenitis induced by granulocyte colony-stimulating factor. Br J Dermatol. Aug 1998;139(2):354-5. [Medline].

  8. Morice A, Penven K, Comoz F, Cribier B, Dompmartin A, Leroy D. [Neutrophilic eccrine hidradenitis in a healthy patient]. Ann Dermatol Venereol. Aug-Sep 2005;132(8-9 Pt 1):686-8. [Medline].

  9. EL Sayed F, Ammoury A, Chababi M, Dhaybi R, Bazex J. Neutrophilic eccrine hidradenitis to acetaminophen. J Eur Acad Dermatol Venereol. Nov 2006;20(10):1338-40. [Medline].

  10. Lienesch DW, Mutasim DF, Singh RR. Neutrophilic eccrine hidradenitis mimicking cutaneous vasculitis in a lupus patient: a complication of cyclophosphamide. Lupus. 2003;12(9):707-9. [Medline].

  11. Tojo M, Iwatsuki K, Furukawa H, Takahashi M, Kaneko F. Neutrophilic eccrine hidradenitis in actinic reticuloid syndrome. Eur J Dermatol. Mar-Apr 2002;12(2):198-200. [Medline].

  12. Bilic M, Mutasim DF. Neutrophilic eccrine hidradenitis in a patient with Behçet's disease. Cutis. Aug 2001;68(2):107-11. [Medline].

  13. Shih IH, Huang YH, Yang CH, Yang LC, Hong HS. Childhood neutrophilic eccrine hidradenitis: a clinicopathologic and immunohistochemical study of 10 patients. J Am Acad Dermatol. Jun 2005;52(6):963-6. [Medline].

  14. Bachmeyer C, Aractingi S. Neutrophilic eccrine hidradenitis. Clin Dermatol. May-Jun 2000;18(3):319-30. [Medline].

  15. Headley CM, Ioffreda MD, Zaenglein AL. Neutrophilic eccrine hidradenitis: a case report of an unusual annular presentation. Cutis. Feb 2005;75(2):93-7. [Medline].

  16. Yasukawa K, Kato N, Aikawa K, Kodama K, Hamasaka A, Hata H. Neutrophilic eccrine hidradenitis with sclerodermoid change heralding the relapse of acute myelogenous leukemia: is this a paraneoplastic phenomenon?. Dermatology. 2007;215(3):261-4. [Medline].

  17. Bardenstein DS, Haluschak J, Gerson S, Zaim MT. Neutrophilic eccrine hidradenitis simulating orbital cellulitis. Arch Ophthalmol. Nov 1994;112(11):1460-3. [Medline].

  18. Srivastava M, Scharf S, Meehan SA, Polsky D. Neutrophilic eccrine hidradenitis masquerading as facial cellulitis. J Am Acad Dermatol. Apr 2007;56(4):693-6. [Medline].

  19. Ostlere LS, Wells J, Stevens HP, Prentice G, Rustin MH. Neutrophilic eccrine hidradenitis with an unusual presentation. Br J Dermatol. Jun 1993;128(6):696-8. [Medline].

  20. Templeton SF, Solomon AR, Swerlick RA. Intradermal bleomycin injections into normal human skin. A histopathologic and immunopathologic study. Arch Dermatol. May 1994;130(5):577-83. [Medline].

  21. Belot V, Perrinaud A, Corven C, de Muret A, Lorette G, Machet L. [Adult idiopathic neutrophilic eccrine hidradenitis treated with colchicine]. Presse Med. Oct 2006;35(10 Pt 1):1475-8. [Medline].

  22. Shear NH, Knowles SR, Shapiro L, Poldre P. Dapsone in prevention of recurrent neutrophilic eccrine hidradenitis. J Am Acad Dermatol. Nov 1996;35(5 Pt 2):819-22. [Medline].

  23. Antonovich DD, Berke A, Grant-Kels JM, Fung M. Infectious eccrine hidradenitis caused by Nocardia. J Am Acad Dermatol. Feb 2004;50(2):315-8. [Medline].

  24. Bernstein EF, Spielvogel RL, Topolsky DL. Recurrent neutrophilic eccrine hidradenitis. Br J Dermatol. Nov 1992;127(5):529-33. [Medline].

  25. Laffitte E, Hohl D, Panizzon RG. [Pseudomonas eccrine hidradenitis in a child revealing acute lymphoblastic leukemia]. Ann Dermatol Venereol. Nov 2004;131(11):975-8. [Medline].

  26. Takai T, Matsunaga A. A case of neutrophilic eccrine hidradenitis associated with streptococcal infectious endocarditis. Dermatology. 2006;212(2):203-5. [Medline].

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Further Reading

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Keywords

neutrophilic eccrine hidradenitis, NEH, acute myelogenous leukemia, AML, chemotherapy-associated eccrine hidradenitis, drug-associated eccrine hidradenitis, infectious eccrine hidradenitis, neutrophilic dermatoses, chemotherapy, intradermal bleomycin injections, Sweet syndrome, atypical pyoderma gangrenosum, toxic erythema of chemotherapy, eccrine squamous syringometaplasia, idiopathic palmoplantar eccrine hidradenitis

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Contributor Information and Disclosures

Author

Joseph C Pierson, MD, Consulting Staff, Department of Dermatology, Keller Army Community Hospital
Joseph C Pierson, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Christine C Tam, MD, 
Disclosure: Nothing to disclose.

Medical Editor

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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