Abstract

Background: Hidradenitis suppurativa (HS) is a chronic inflammatory disease of the pilosebaceous unit that typically occurs after puberty and is rarely reported in young children. Early-onset cases may indicate an underlying genetic or immunologic disorder.
Case presentation: We report an 8-year-old female with severe atopic dermatitis, bronchial asthma, and multiple food allergies who presented with recurrent painful abscesses involving the axilla and buttocks. Examination revealed inflammatory nodules and sinus tract formation in the right axilla consistent with HS. Laboratory testing showed markedly elevated serum IgE levels (12,609 IU/ml) and reduced IgM. Whole-exome sequencing identified a homozygous pathogenic deletion in exons 8 to 9 of the DOCK8 gene, confirming DOCK8 deficiency Hyper-IgE syndrome. The patient was treated with oral doxycycline, topical clindamycin, topical corticosteroids, antiseptic washes, and supportive skin care with partial improvement. Her course was complicated by severe ceftriaxone-induced anaphylaxis during hospitalization for influenza-associated pneumonia.
Conclusion: This case highlights the rare association between early-onset HS and DOCK8 deficiency and under scores the importance of considering inborn errors of immunity in pediatric patients with atypical or severe HS to guide appropriate multidisciplinary management.

Key words: Hidradenitis suppurativa, DOCK8 deficiency, Hyper-IgE syndrome, Dermatology, Pediatrics