Hidradenitis suppurativa in Child
See also in: Cellulitis DDx,AnogenitalAlerts and Notices
Synopsis
The nodules of hidradenitis suppurativa are seen most commonly on the buttocks, breasts, and in the groin and axillae. Usually, the onset of the disease occurs soon after puberty, and patients typically report recurring "boils." Symptoms may include local pain and tenderness during a flare-up and arthralgias. Shaving, depilation, deodorants, and mechanical irritation can worsen this condition, but irritation of the skin is usually not a major factor.
Obesity and cigarette smoking are associated with hidradenitis suppurativa. Hidradenitis suppurativa and metabolic syndrome are strongly associated. Regional ileitis (Crohn disease) has a statistical association with hidradenitis suppurativa, while ulcerative colitis does not. A familial form of the disease has been supported by studies, including a molecular genetic study of 4 generations in a large Chinese family, through which a novel hidradenitis suppurativa locus on chromosome 1p21.1-1q25.3 was identified. Furthermore, many patients report a positive family history. Rare cases of hidradenitis suppurativa are associated with reticulate pigmented anomaly of the flexures (Dowling-Degos disease) and heterozygous mutations of PSENEN (gamma-secretase protein presenilin precursor). Hidradenitis suppurative-like lesions have been reported to very frequently occur during therapy with a gamma secretase inhibitor.
Hidradenitis suppurativa shares similar clinical features (severe inflammation, occlusion of the follicle, and scarring) with dissecting cellulitis of the scalp and acne conglobata. Collectively, these 3 conditions are referred to as the follicular occlusion triad, and more than 1 may occur in a given patient. Some consider the pilonidal sinus (pilonidal cyst) to be an additional member of this group.
Syndromes include PASH (pyoderma gangrenosum, acne, and hidradenitis suppurativa), PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa), and PsAPASH (psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa).
With a prevalence of up to 1% in some population-based studies, hidradenitis suppurativa is a common disease.
Pediatric patient considerations: Hidradenitis suppurativa usually presents after puberty. It is more common in girls. In children presenting with early-onset disease, there is a strong association with widespread involvement, hormonal imbalance, and a strong family history. In pediatric patients overall, there is a higher reported rate of coexisting obesity, anxiety disorders, acne vulgaris, and acne conglobata.
Codes
ICD10CM:L73.2 – Hidradenitis suppurativa
SNOMEDCT:
59393003 – Hidradenitis suppurativa
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Differential Diagnosis & Pitfalls
- Furunculosis
- Folliculitis
- Epidermoid cysts
- Abscesses, including recurrent Staphylococcus aureus infection and CA-MRSA
- Pilonidal sinus (gluteal cleft)
- Candidiasis
- Ulcerative colitis (genital region)
- Bartholin gland abscess (genital region)
- Crohn disease (perianal)
- Granuloma inguinale (genital region)
- Tuberculosis
- Actinomycosis
- Lymphogranuloma venereum (genital region)
- Tularemia
- Cat-scratch disease
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Last Reviewed:08/11/2019
Last Updated:10/18/2021
Last Updated:10/18/2021
Hidradenitis suppurativa in Child
See also in: Cellulitis DDx,Anogenital