Hidradenitis suppurativa-associated cSCC
Marjolin-type cSCC arising in HS; Verneuil-disease-associated SCC; HS-cSCC
Hidradenitis suppurativa-associated cutaneous squamous cell carcinoma is an aggressive Marjolin-spectrum SCC that arises within long-standing, severe Hurley stage III hidradenitis suppurativa (HS), particularly in the perineal, perianal, buttock and groin regions of male patients with disease durations of 20–30 years. The risk is real but historically under-appreciated: pooled estimates suggest an approximately 2–5-fold increased risk of cSCC in HS depending on series, rising to substantially higher in long-standing severe perineal disease. The clinical course is aggressive — large, locally destructive tumours frequently extending to ischial/pelvic structures, regional lymph-node metastasis in 30–50% and a 5-year overall survival around 30–50%, comparable to other Marjolin SCCs. Recognition of the risk and surveillance of long-standing severe HS — with low threshold for biopsy of any new ulcer, induration or rapidly growing nodule — are the most effective interventions.
Risk & pathogenesis
- Pooled epidemiological estimates suggest approximately 2–5× baseline cSCC risk in HS depending on series (Lapins 2001; Sachdeva 2021 meta-analysis); substantially higher in long-standing severe perineal / buttock disease (Marjolin-type transformation).
- Mechanistic drivers: chronic inflammation, repeated cycles of ulceration / re-epithelialisation, scarring with disordered cellular kinetics, HPV co-infection in some cases.
- Risk factors within HS: male sex (HS-cSCC is markedly male-predominant despite HS being female-predominant overall); long disease duration (typically 20–30 years); Hurley stage III; perineal / perianal / buttock involvement; smoking; obesity.
Clinical features
- New ulcer, exophytic mass, induration or non-healing wound within long-standing HS sinus tracts.
- Foul-smelling discharge, pain disproportionate to background HS, bleeding.
- Most common sites: gluteal, perianal, perineal, vulval, scrotal; less often axillary or inguinal.
- Frequently misattributed to "another HS flare" — diagnostic delay common.
- Regional lymphadenopathy commonly palpable at diagnosis (inguinal, ischial).
- Differential: chronic HS sinus / abscess; verrucous carcinoma (Buschke-Löwenstein, see monograph); pyoderma vegetans; deep fungal infection; Crohn's perianal disease.
Diagnosis
- Multiple deep biopsies through the ulcer base, edge and any indurated areas.
- Repeat biopsies if first sample shows only chronic inflammation and clinical concern persists.
- Histology — usually well- or moderately-differentiated SCC; verrucous carcinoma also reported.
- HPV testing — high-risk types (especially HPV-16) detectable in a proportion of HS-cSCC.
- Imaging — MRI pelvis to define local extent, deep / pelvic invasion; CT chest/abdomen/pelvis for staging; PET-CT in selected cases.
- Discuss in skin cancer MDT with input from colorectal, urology / gynaecology and plastic surgery.
Management
- Wide local excision with at least 1–2 cm margins, en-bloc excision of all involved sinus tracts and HS-affected skin where feasible.
- Ano-rectal / sphincter-sparing approach where possible; abdominoperineal resection or pelvic exenteration for advanced perineal / pelvic disease.
- Sentinel lymph node biopsy or selective inguinal / ilio-inguinal dissection given high occult nodal disease rate.
- Reconstructive plastic surgery — V-Y flaps, fasciocutaneous flaps, free flaps.
- Adjuvant radiotherapy for incomplete margins, perineural invasion, multiple positive nodes, ENE.
- Cemiplimab (NICE TA802) for advanced / metastatic disease unsuitable for surgery / RT.
- Optimal long-term HS control to reduce recurrence — adalimumab (NICE TA392) or other biologic; lifestyle modification (smoking cessation, weight optimisation).
Prognosis & surveillance
5-year overall survival 30–50%; substantially worse than conventional cSCC. Adverse prognostic factors: large tumour size, deep / pelvic invasion, perineural / lymphovascular invasion, nodal metastasis, late presentation. Long-term clinical surveillance of any patient with severe long-standing HS is essential; counsel patients to flag any new ulcer, induration or rapidly growing lesion. Optimised HS control with biologic therapy may reduce future risk.
References
- Lapins J et al. Incidence of cancer among patients with hidradenitis suppurativa. Arch Dermatol; 2001.
- Sachdeva M et al. Squamous cell carcinoma arising within hidradenitis suppurativa — review. J Am Acad Dermatol; 2021.
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