Neutrophilic eccrine hidradenitis L73.2

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.

Epidermis that is largely unremarkable; less commonly, showing spongiotic changes. Edema of the dermis. Neutrophilic or (mild) lymphocytic infiltrate in the area of the sweat glands, particularly around the excretory ducts or terminal ends of eccrine sweat glands. The inflammation may extend subcutaneously. Occasional necrosis of sweat gland cells. “Squamous syringometaplasia” may occur in association with this sweat gland reaction.

Clinical:

Acute febrile neutrophilic dermatosis: fever, altered white blood cell count, large papules and plaques 

Folliculitis: pustules of varying sizes, no direct link to chemotherapy, detection of bacteria

Whirlpool dermatitis: folliculitis predominantly affecting the trunk, detection of Pseudomonas aeruginosa,    

Palmoplantar eccrine hidradenitis, which occurs on the palms and soles of the feet after prolonged exposure to a cold, damp environment. 

The clinical picture known as“palmoplantar eccrine hidradenitis”—a condition with a nearly identical name—refers to an acute inflammation of the sweat glands without signs of a systemic disease, characterized by deep-seated, painful, red papules, plaques, and/or nodules that occur primarily on the soles of the feet and, less commonly, on the palms of the hands.   

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