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Neutrophilic Eccrine Hidradenitis Clinical Presentation

  • Author: Joseph C Pierson, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Oct 13, 2015
 

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. Some healthy patients, children,[11] and adults,[12] have developed generalized urticarial-like papules and plaques of idiopathic neutrophilic eccrine hidradenitis in the summertime.

Regardless of the clinical setting, patients with neutrophilic eccrine hidradenitis develop skin lesions and frequently report fever. Half the patients are asymptomatic, but pain and tenderness are not uncommon. The palmoplantar variant of neutrophilic eccrine hidradenitis occurs in healthy children; however, one child in remission after acute lymphoblastic leukemia developed generalized extension.[13]

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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,[14] and sclerodermoid changes[15] have also been noted. Tenderness may be elicited. The trunk or limbs are most often involved. Neutrophilic eccrine hidradenitis simulating orbital cellulitis,[16] facial cellulitis,[17] and symmetrical ear swelling[18] have been documented.

Courtesy of Jeffrey P. Callen, MD. Courtesy of Jeffrey P. Callen, MD.
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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.[19] 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 AML[4] and chronic myelogenous leukemia[5] and the relapse of AML.[6] Neutrophilic eccrine hidradenitis has also been observed in otherwise healthy individuals[8] ; in Behçet disease[9] ; with acetaminophen[20] ; with granulocyte colony-stimulating factor[7] ; with cyclophosphamide therapy for lupus[21] ; with methotrexate therapy for actinic reticuloid[22] ; with carbamazepine usage[23] ; with cetuximab treatment[24] ; in azathioprine hypersensitivity syndrome[25] ; and with HIV, Serratia, Enterobacter,Nocardia, Staphylococcus, Streptococcus, and Mycobacterium chelonae[26] infections.

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Contributor Information and Disclosures
Author

Joseph C Pierson, MD Dermatology Residency Program Director, University of Vermont College of Medicine

Joseph C Pierson, MD is a member of the following medical societies: Association of Professors of Dermatology, New England Dermatological Society, American Academy of Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Christine C Tam, MD Managing Member, Certified Dermatologists

Christine C Tam, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

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

  3. Cohen PR. Neutrophilic dermatoses occurring in oncology patients. Int J Dermatol. 2007 Jan. 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. 1993 Jun. 129(6):791-2. [Medline].

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  15. 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].

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  23. Bhanu P, Santosh KV, Gondi S, Manjunath KG, Rajendaran SC, Raj N. Neutrophilic eccrine hidradenitis: a new culprit-carbamazepine. Indian J Pharmacol. 2013 Jan-Feb. 45 (1):91-2. [Medline].

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

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Courtesy of Jeffrey P. Callen, MD.
Neutrophilic infiltrate on hematoxylin and eosin stain (100X). Courtesy of Jeffrey P. Callen, MD, and Vilma Fabre, MD.
 
 
 
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