A 51-year-old woman presented with a 30-year history of refractory hidradenitis suppurativa (HS) with severe, extensive involvement of the genital and perineal areas. She initially developed isolated nodules, which gradually enlarged into a painful, coalescent, hypertrophic, exudative lesion. Aggressive evolution took place despite multiple courses of topical and systemic antibiotics, surgical treatments with complex wound closure and systemic immunosuppressive agents.^1,2 Examination revealed a large genital mass with multiple new abscesses and draining fistulae with purulent discharge, associated with fibrotic, thickened scar tissue in the inguinal (Fig.1) and intergluteal regions (Fig.2). Her longstanding HS has been upstaged to Hurley stage III and treatment with adalimumab initiated.^1,2 Currently, despite continuous treatment over three years, there have been no improvements and the decision was made to switch to infliximab.^1,2

The pathophysiology of this highly morbid, chronic inflammatory skin disease remains unclear.^2,3 Hopefully this severe case raises awareness of this disabling and disfiguring condition.

Figura I

Large genital mass associated with multiple abscesses, fistulae and scar tissue in the inguinal region.

Figura II

Large genital mass associated with fibrotic, thickened scar tissue in the intergluteal region.

  BIBLIOGRAFIA  

1.Zouboulis CC, Desai N, Emtestam L, Hunger RE, Ioannides D, Juhász I, et al. European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa. J Eur Acad Dermatol Venereol. 2015;29(4):619-44.

2.Jemec GB. Hidradenitis suppurativa. N Engl J Med. 2012;366:158-64.

3. Pironi D, Caruso F, Panarese A, Vendettuoli M, Mascagni D, Moraldi L, et al. Chronic hidradenitis suppurativa in the inguinal, perineal and scrotal regions. A case report and review of the literature. Ann Ital Chir. 2010;81(6):465-70.