Neutrophilic Eccrine Hidradenitis in a Child with Chronic Granulomatous Disease.

Sir,

Neutrophilic eccrine hidradenitis (NEH)first described by Harris et al. is an uncommon, self-limiting reactive disorder with characteristic neutrophilic inflammation in eccrine coils, occurring most commonly in association with lymphoreticular malignancy (with or without chemotherapy.[1] It has been reported in individuals as young as 6 months to as old as 79 years. It is a self-limiting process with no adverse prognosis.

A 10-year-old boy, diagnosed case of chronic granulomatous disease (CGD) (by nitro-blue tetrazolium slide test), presented with two red, raised, tender, lentil-sized swellings over the right palm for 3 weeks, associated with fever and palmoplantar hyperhidrosis. There was no history of any other drug intake (cytotoxic/immunomodulators) or preceding trauma. The patient had completed a 2-week course of amoxicillin and clavulanic acid without any response. Cutaneous examination showed two discrete, tender, erythematous, violaceous nodules of size 1.5 × 1.5 to 2 × 2 cm over the right palm [Figure 1]. Profuse hyperhidrosis of the palms and soles was also observed. Laboratory investigations revealed neutropenia (total leukocyte count - 2800/mm3) with 12% neutrophils. All other hematological and radiological investigations were within normal limits. Histopathological examination of the nodule showed neutrophilic exocytosis in the epidermis, neutrophilic infiltrate around eccrine glands in superficial and mid dermis. The deep dermis showed an abscess with the presence of neutrophils and necrosis confirming NEH [Figure 2].

Figure 1

Erythematous nodules over the right palm

Figure 2

Neutrophilic abscess in dermis, (H and E, ×40)

NEH can occur due to direct cytotoxicity of a drug secreted in sweat from eccrine coils or can be a form of paraneoplastic syndrome.[2] Lesions in NEH can be solitary or multiple and present as erythematous or purpuric macules, papules, nodules, or plaques, mostly on the trunk or limbs. The palmoplantar form occurs in children without any context of malignancy. The condition should be differentiated from infectious dermatoses that may require treatment.[3] The definite diagnosis of NEH relies on histological features which reveal a dense neutrophilic infiltrate within and around eccrine glands, accompanied by necrosis of eccrine epithelial cells.[1]

Our patient had palmoplantar hyperhidrosis which could have been the primary inciting factor for NEH. Increased secretion of sweat components such as lactate, urea, electrolytes, and trace elements secondary to increased hyperhidrosis can result in microenvironmental alteration around the sweat gland, initiating an inflammatory cascade, and precipitating NEH. “Internal sweating” (extravasated sweat in the dermis and epidermis) can also result in sweat-gland-mediated cutaneous inflammation.[4] CGD an immunodeficiency disorder, which results from an inability of phagocytes to produce bactericidal superoxide anions (O2−). This leads to recurrent life-threatening bacterial and fungal infections. The inappropriate respiratory burst action of neutrophils in CGD could have aggravated the inflammatory process of NEH in our patient. Thus, there could be an association of NEH in CGD, or it could merely be a coincidence.

The child was put on anti-inflammatory drugs and topical aluminum hexahydrate 20% lotion along with trimethoprim-sulfamethoxazole (TMP-SMX) tablets twice daily for 5 days. The nodules completely resolved in 10 days. The patient is on follow-up for 1 year with no recurrence. TMP-SMX is the antibiotic of choice for prophylaxis in CGD patients. Apart from having antimicrobial activity, it also corrects the abnormalities of neutrophil motility. These combined features of TMP-SMX along with anti-inflammatory drugs could have resulted in resolution of the lesions in our patient.

This report describes an association of NEH with CGD, which has not been reported so far.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.