Name
#71 - Taking the Roof Off Gamma Secretase Inhibitor Induced Hidradenitis Suppurativa
Date & Time
Tuesday, February 13, 2024, 12:00 PM
Description

Hidradenitis suppurativa (HS) is a poorly understood chronic inflammatory disorder of the hair follicle that affects apocrine gland rich and intertriginous areas. The prevalence in the United States is approximately 0.1% and the disease disproportionally affects women, African Americans, and biracial population subsets. HS is a clinical diagnosis characterized by deep seated, painful nodules, frequently in the axilla or groin, that may spontaneously rupture and drain malodorous, purulent, or sanguineous material. As the disease progresses, deep dermal tunnels can develop between nodules, forming extensive epithelialized sinus tracts. We present a case of new onset HS in a 29-year-old active duty otherwise healthy male of Latino ancestry following initiation of a novel, chemotherapeutic agent for treatment of a desmoid tumor of the right leg. He had previously failed treatment with sorafenib and doxorubicin, prompting initiation of nirogacestat, an investigational, small-molecule, selective gamma-secretase inhibitor. Within four months, he had developed Hurley Stage III HS. The most widespread grading system for HS used in clinical practice is the Hurley Staging System (HSS), which categorizes disease severity from Grade I-III. The United States and Canadian Hidradenitis Suppurativa Foundations recommend the use of HSS in the clinical setting due to ease of application, and have published therapeutic treatment ladder based on this scale (Alikahn et al., 2019). Based on these treatment guidelines and due to the investigational nature of nirogacestat, our patient was not a candidate for systemic medical therapy and was initially treated with benzoyl peroxide body wash, topical clindamycin and intralesional Kenalog. Unfortunately, his disease continued to progress and he elected to proceed with adjunctive procedural management. Surgical deroofing is a straightforward in-office procedure performed with local anesthetic. The technique was described by van der Zee et al in 2010 as a tissue saving surgical technique which demonstrated that lesions did not recur in a large majority of patients treated with this procedure compared to simple incision and drainage which approaches 100%. Furthermore, patients treated with this method reported high satisfaction scores. A recent review asserted surgical deroofing as superior to standard full-thickness excision with significantly lower rates of recurrence and complications as well (Saylor et al., 2020). Our patient was treated with in-office deroofing and reports high satisfaction with the clinical outcome.

Location Name
Prince Georges Exhibit Hall A/B
Content Presented on Behalf of
Navy
Learning Outcomes
1. Increase awareness of hidradenitis suppurativa (HS), its clinical presentation and the demographic that it disproportionally afflicts.<br />
2. Recognize that HS like lesions may represent an adverse cutaneous effect of nirogacestat, an investigational medication used to treat desmoid tumors.<br />
3. Demonstrate improved understanding of the most widely used staging system for HS .<br />
4. Be able to formulate and confidently apply a therapeutic treatment ladder.<br />
5. Recognize that surgical deroofing procedure is a straight forward in office procedure with excellent functional and cosmetic results with high rates of patient satisfaction.
Session Type
Posters