Skin nontumor
Other diseases of skin appendages
Neutrophilic eccrine hidradenitis
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Editor-in-Chief: Debra L. Zynger, M.D.
Last author update: 20 July 2020
Last staff update: 19 May 2021
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PubMed Search: Neutrophilic eccrine hidradenitis[TI]
Table of Contents
Definition / general | Essential features | Sites | Pathophysiology | Etiology | Clinical features | Diagnosis | Prognostic factors | Case reports | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Saleh J, Speiser J. Neutrophilic eccrine hidradenitis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorneh.html. Accessed April 25th, 2024.
Definition / general
- A benign, self limiting chemotherapy associated neutrophilic dermatosis characterized by neutrophilic infiltration of eccrine sweat glands (Case Rep Dermatol 2013;5:340)
Essential features
- Most commonly associated with induction chemotherapy for lymphoproliferative disorders (Case Rep Dermatol 2013;5:340, StatPearls: Neutrophilic Eccrine Hidradenitis [Accessed 28 April 2020])
- Presents as a polymorphous eruption consisting of variably asymptomatic or tender erythematous to violaceous macules, papules, plaques, nodules and pustules (Arch Dermatol 1986;122:809, J Am Acad Dermatol 2007;56:693)
- Characterized by neutrophils infiltrating and surrounding the eccrine secretory coils in the deeper reticular dermis or subcutaneous fat
- A benign, self limiting condition in which lesions desquamate and usually heal spontaneously within 1 - 3 weeks (Case Rep Dermatol 2013;5:340)
Sites
- Most commonly affecting the trunk and upper limbs but may affect the face and bilateral ears (Arch Dermatol 1986;122:809, J Am Acad Dermatol 2007;56:693)
Pathophysiology
- Postulated to arise secondary to a direct cytotoxic effect of a therapeutic agent secreted in the sweat on the eccrine glands or a form of paraneoplastic syndrome (Case Rep Dermatol 2013;5:340, Indian J Dermatol 2016;61:683)
- Damage of the glands may stimulate an inflammatory response and neutrophilic chemotaxis, which would lead to cellular damage and necrosis of the epithelial cells (Case Rep Dermatol 2013;5:340)
Etiology
- Most commonly associated with induction chemotherapy for lymphoproliferative disorders (Case Rep Dermatol 2013;5:340, StatPearls: Neutrophilic Eccrine Hidradenitis [Accessed 28 April 2020])
- Less commonly described as a prodromal manifestation of acute myelogenous leukemia in the absence of chemotherapy (J Am Acad Dermatol 1984;11:584)
- Reported in patients with HIV and other malignancies such as breast carcinoma, Wilms tumor, osteosarcoma and testicular carcinoma (Case Rep Dermatol 2013;5:340, Int J Dermatol 2007;46:59)
- May be caused by infectious agents such as Serratia, Enterobacter and Staphylococcus aureus (Actas Dermosifiliogr 2014;105:e7, Case Rep Dermatol 2013;5:340)
- Associated chemotherapeutic agents include cytarabine (most common), doxorubicin, bleomycin, chlorambucil, mitoxantrone, daunorubicin, dactinomycin, vincristine, lomustine, mitoxantrone, cisplatin, vinblastine, topotecan, cyclophosphamide and 5-fluorouracil (Arch Dermatol 1986;122:809, Acta Dermatovenerol Alp Pannonica Adriat 2018;27:207, Indian J Pharmacol 2013;45:91, Clin Case Rep 2019;7:533, StatPearls: Neutrophilic Eccrine Hidradenitis [Accessed 28 April 2020], Acta Haematol 1992;87:167)
Clinical features
- Clinically manifests 2 days to 3 weeks following the induction of chemotherapy (J Am Acad Dermatol 2007;56:693)
- Presents as a polymorphous eruption consisting of variably asymptomatic or tender erythematous to violaceous macules, papules, plaques, nodules and pustules (Arch Dermatol 1986;122:809, J Am Acad Dermatol 2007;56:693)
- Fever or neutropenia commonly present (Arch Dermatol 1986;122:809, Indian J Dermatol 2016;61:683, J Am Acad Dermatol 2007;56:693)
Diagnosis
- Skin biopsy
Prognostic factors
- Lesions desquamate and usually heal spontaneously within 1 - 3 weeks (Case Rep Dermatol 2013;5:340)
Case reports
- 35 year old HIV positive man on antiretroviral therapy (Skinmed 2017;15:297)
- 37 year old man receiving FOLFOX chemotherapy for colon cancer (Acta Dermatovenerol Alp Pannonica Adriat 2018;27:207)
- 47 year old man undergoing chemotherapy for acute myeloid leukemia (Pan Afr Med J 2014;18:727)
- 66 year old man receiving chemotherapy for acute myeloid leukemia (JAAD Case Rep 2019;5:691)
Treatment
- Treatment is symptomatic and mainly involves topical or systemic corticosteroids (Case Rep Dermatol 2013;5:340)
Clinical images
Images hosted on other servers:
Violaceous plaques on the upper back
Erythematous plaques on the chest and abdomen
Solitary papules on the forearm
Erythematous, papular lesions on bilateral palms
Microscopic (histologic) description
- Neutrophils infiltrating and surrounding the eccrine secretory coils in the deeper reticular dermis or subcutaneous fat
- Neutrophils may be sparse or lacking when patients are neutropenic (J Cutan Pathol 1987;14:272)
- May be accompanied by dermal edema, syringosquamous metaplasia and necrosis of the eccrine coils and glands (Dermatol Online J 2011;17:14)
- Periadnexal fibroadipose tissue typically shows mucinous degeneration (J Am Acad Dermatol 1984;11:584)
- Epidermal spongiosis, basal vacuolization and focal necrotic keratinocytes may also be present (Arch Dermatol 1982;118:263)
- No evidence of vasculitis (Dermatol Pract Concept 2016;6:55, J Am Acad Dermatol 1984;11:584)
Microscopic (histologic) images
Contributed by Jasmine Saleh, M.D., M.P.H. and Jodi Speiser, M.D.
Interface dermatitis
Neutrophils and squamous syringometaplasia
Sample pathology report
- Skin, upper back, biopsy:
- Neutrophilic eccrine hidradenitis
Differential diagnosis
- Idiopathic plantar hidradenitis (neutrophilic eccrine hidradenitis in children):
- Neutrophilic inflammation is centered on the coiled duct and proximal straight duct, with the secretory apparatus usually being spared or only minimally affected
- Eccrine syringometaplasia is not seen
- Sweet syndrome:
- Dense diffuse infiltration of neutrophils in the dermis
- No eccrine involvement
- Cellulitis:
- Diffuse infiltration of neutrophils
- Positive culture or special stains
- No eccrine involvement
- Pyoderma gangrenosum:
- Nonspecific ulceration with dermal acute and chronic inflammation
- No eccrine involvement
Board review style question #1
Which of the following is true about neutrophilic eccrine hidradenitis (shown in the image)?
- Dermal edema, squamous syringometaplasia and necrosis of the eccrine coils and glands may be present
- It is highly malignant with poor prognosis
- It most frequently occurs in infants and children after viral infection
- It presents as a tan-brown pustule
Board review style answer #1
A. Dermal edema, squamous syringometaplasia and necrosis of the eccrine coils and glands may be present
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Board review style question #2
Which of the following can be seen in neutrophilic eccrine hidradenitis?
- Dense diffuse infiltration of neutrophils in the dermis
- Mucinous degeneration in periadnexal fibroadipose tissue
- Ulceration
- Vasculitis
Board review style answer #2
B. Mucinous degeneration in periadnexal fibroadipose tissue
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Reference: Neutrophilic eccrine hidradenitis
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