Neutrophilic eccrine hidradenitisL73.2

Rare, self-limited clinical picture, characterized by painful erythema, plaques or nodules, fever and neutrophil infiltration in the area of the eccrine sweat gland

Rare. Worldwide, < than 100 cases have been described casuistically so far.

Occurrence is most frequently described in connection with systemic chemotherapies (e.g. bleomycin, anthracyclines, BRAF inhibitors); this could be a toxic reaction of the chemotherapeutic agent on the glandular epithelium with reactive neutrophil infiltrates.

Also occurs as paraneoplasia (AML), in infections ( HIV infection, hepatitis C, Serratia marcescens infections, Enterobacter infections) and in chronic inflammatory diseases ( Behçet's disease).

Predominantly trunk, extremities and face.

Diverse clinic with acute, predominantly disseminated, asymmetrically localized, painful erythema, reddish-livid plaques or painful nodules. Rarely grouped standing, pinhead-sized papules, pustules, papulopustules or pressure sensitive nodules.

The general condition may be reduced.

The dermatological symptoms are usually accompanied by fever for several days.

The clinical picture is self-limiting and heals spontaneously after a few weeks.

Largely inconspicuous, more rarely spongiotically changed epidermis. Edema of the dermis. Neutrophilic or (low) lymphocytic infiltrate in the area of the sweat glands, especially around the excretory ducts or end pieces of eccrine sweat glands. Inflammatory processes can radiate subcutaneously. Occasional necrosis of the cells of the sweat glands. A "squamous syringometaplasia" may occur in connection with this reaction.

Clinical:

• Acute febrile neutrophilic dermatosis: fever, disorder of the AZ, larger papules and plaques
• Folliculitis: pustules of different sizes, no direct relation to chemotherapy, detection of bacteria
• Whirlpool dermatitis: trunk-related folliculitis, detection of Pseudomonas aeruginosa,
• Palmoplantar eccrine hidradenitis, which occurs on the palms of the hands and soles of the feet after prolonged exposure in a humid and cold environment

1. Bachmeyer C et al (2000) Neutrophilic eccrine hidradenitis. Clin Dermatol 18: 319-330
2. Callen JP et al (2002) Neutrophilic dermatoses. Dermatol Clin 20: 409-419
3. Combemale P et al (2003) Neutrophilic eccrine hidradenitis secondary to infection with Serratia marcescens. Br J Dermatol 142: 784-748
4. Crawford GH et al (2003) Erythematous facial plaques in a patient with leukemia. Neutrophilic eccrine hidradenitis. Arch Dermatol 139: 531-536
5. Gomez Vazquez M (2003) Neutrophilic eccrine hidradenitis heralding the onset of chronic myelogenous leukaemia. J Eur Acad Dermatol Venereol 17: 328-330
6. Gross PR et al (1999) Neutrophilic dermatosis versus neutrophilic eccrine hidradenitis. N Engl J Med 340: 1371
7. Harrist TJ et al (1982) Neutrophilic eccrine hidradenitis. A distinctive type of neutrophilic dermatosis associated with myelogenous leukemia and chemotherapy. Arch Dermatol 118: 263-266

8. Herms F et al (2017) Neutrophilic eccrine hidradenitis in two patients treated with BRAF inhibitors:
   anew cutaneous adverse event.Br J Dermatol 176:1645-1648.

9. Krahl B (2015) Nuetrophilic eccrine hidradenitis. Act Dertmatol 41: 223-224

10. Mercader-Garcia P et al (2003) Neutrophilic eccrine hidradenitis in a patient with Behcet's disease. Acta Derm Venereol 83: 395-396
11. Nijsten TE et al (2003) Chronic pruritic neutrophilic eccrine hidradenitis in a patient with Behcet's disease. Br J Dermatol 147: 797-800