Chronic pruritic neutrophilic eccrine hidradenitis in a patient with Behcet's disease

Abstract

Neutrophilic eccrine hidradenitis (NEH) is a rare distinct entity that usually presents as asymptomatic erythematous papules that disappear spontaneously in 1-3 weeks. However, its appearance may be polymorphic, pruritic, recurrent or even chronic as is described in this case. The histological combination of neutrophilic infiltration in and necrosis of the eccrine secretory gland epithelium is highly characteristic for NEH. It typically occurs in patients receiving chemotherapeutic drugs for malignancies, but other associations have also been reported. To our knowledge, we report the first case of NEH in a patient with Behçet's disease (BD). Cutaneous manifestations of BD, an inflammatory systemic disorder of unknown origin, include neutrophilic dermatoses such as Sweet's syndrome and pyoderma gangrenosum, although these are unusual in BD. NEH could be another neutrophilic dermatosis related to BD. This observation suggests that NEH is not strictly related to chemotherapeutic drugs and malignancies. It appears to be a reactive dermatosis associated with other factors as well, including BD. Treatment was successful with dapsone 100 mg daily.

Therapy-Resistant, Spontaneously Remitting Generalized Neutrophilic Eccrine Hidradenitis in a Healthy Patient Decreases the Expression of Dermcidin in Affected Eccrine Glands

“…Other associations include nonchemotherapeutic drugs such as granulocyte G-CSF, hemodialysis, Behçet's disease and infectious diseases (e.g. Serratia marcescens , Enterobacter cloacae , Staphylococcus aureus , streptococcal endocarditis, Nocardia , HIV-1) [2, 3, 6, 7, 8, 9]. However, generalized NEH without any disorder is extremely rare.…”

“…Previous reports suggest that NEH normally develops in patients with malignancies following chemotherapy such as cytarabine, although hemodialysis, Behçet's disease or infectious diseases, or nonchemotherapeutic drugs such as granulocyte colony-stimulating factor (G-CSF) are also associated with NEH [2, 3]. However, generalized NEH in healthy adults without any disorders is extremely rare and its precise pathogenesis is still under consideration.…”

Diseases of cutaneous appendages

“…Involvement of the ears and circumferential involvement of the breasts have been reported. 1545 The lesions may resolve after 2-3 weeks. 1550 Lesions are usually asymptomatic, but pruritic lesions sometimes develop.…”

“…1539,1540 In one of these cases, acetaminophen (paracetamol) was implicated. 1545 It is rarely due to infection. 1545 It is rarely due to infection.…”

Successful Treatment of Recurrent Idiopathic Plantar Eccrine Hidradenitis With Colchicine

“…[9][10][11][12][13] Four case reports mention the beneficial use of dapsone in adults with NEH in association with chemotherapy for acute myelogeneous leukemia, myelodysplastic syndrome, and non-small-cell lung cancer, and with Behçet disease. [9][10][11][12] As for colchicine, there exists a single case report of a 56-year-old female patient with facial NEH who experienced full recovery after treatment with colchicine. 13 On the basis of these 2 reports and the histologic picture of a neutrophilic infiltrate in our patient, we started treatment with colchicine at a dose of 0.5 mg/d at the beginning of another episode.…”