Understanding the HS subtypes
According to Ginette A. Okoye, MD, FAAD, professor and chair of the Department of Dermatology at Howard University College of Medicine, physicians should ask themselves 3 questions to determine the best way to treat their individual hidradenitis suppurativa (HS) patients. DC, and co-chair of the Skin of Color Society Scientific Committee, which presented at the Skin of Color Update 2021, held virtually from September 10-12.
These 3 essential questions include:
- Why does the patient have the type of HS?
- Has the patient identified any triggers for the development of new lesions or flare-ups?
- What comorbidities does the patient have or is he at risk of having?
Based on Okoye’s observations and her knowledge of the available research, she suggested several clinical pearls based on these questions to help physicians determine which therapy is best for their patients with HS.
In his presentation, Okoye stressed the importance of knowing the specific type of HS that patients have to better understand the pathogenesis of the disease and its subsequent treatment.
She identified 4 clinical subtypes of HS, including axillary-mammary-inguinal, follicular, gluteal and syndromic.
For the axillary-mammary-inguinal HS subtype, Okoye observed that lesions most commonly appear on the breasts, armpits, and inguinal fold. In addition, this subtype of HS patients tends to be predominantly female.
“These patients tend to report hormonal fluctuations with the severity of their HS, and these are the patients who might respond to weight loss. Certainly obesity is not the cause of HS, so you need to be careful when bringing this topic up with your patients, ”Okoye said.
The pathogenesis of this subtype, according to Okoye, includes diet, obesity, and hormonal influences. Due to these factors, his recommended treatments in addition to anti-inflammatory therapy involve spironolactone 50-200 mg per day, metformin 2000 mg per day, laser hair removal, as well as in certain circumstances the use of pills. FDA-approved oral contraceptives for acne. Okoye also suggests discouraging the use of progesterone-only hormonal contraceptives and discussing nutritional support and dietary modifications with your HS patient.
” What I am saying [patients] is based on how little data we have and what patients have told me over the years, I think a low carb diet or a low glycemic index diet might help. While it isn’t helpful for their HS, it can be helpful for their co-morbidities, ”Okoye said.
For the follicular subtype of HS, Okoye said these patients have HS in typical locations, but the lesions tend to be smaller and less severe. However, they also have other types of lesions such as open comedones, epidermal cysts, pilonidal cysts, and severe acne.
“The other thing about these patients is that they can get HS in what we would call atypical areas like the lateral thigh, chest, back, but these lesions are smaller and very follicular compared to your typical HS lesion, ”Okoye said.
This subtype is mostly made up of males and can affect current and former smokers, according to Okoye’s observations. She added that the follicular subtype has moderate disease severity, but also includes earlier onset of disease, as well as longer disease duration.
For the pathogenesis of this subtype, Okoye believes that the altered notch signaling is caused by a mutation in gamma secretase or smoking. “I think people with the more severe phenotype both have this mutation and are smokers,” Okoye said.
Regarding treatment, Okoye’s preferred therapies include oral and topical retinoids such as isotretinoin and acitretin instead of anti-inflammatory therapy. Also, early surgical excision of epidermal inclusion cysts (EICs) can be removed in cosmetically sensitive areas, but Okoye says to leave other EICs alone unless they are symptomatic. Most importantly, she recommends that these types of patients with HS quit smoking.
This HS subtype, according to Okoye, consists of patients with follicular occlusion tetrad and autoinflammatory syndromes such as:
- PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, HS)
- PASH (pyogenic arthritis, HS)
- HS and inflammatory arthritis
- SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis)
Okoye believes that neutrophils, interleukin (IL) -17, IL-1, and tumor necrosis factor (TNF) -α are associated with the pathogenesis of patients with syndromic HS.
“Now these patients, I think, are squarely in the inflammatory camp. And so, for them, organic products are my choice, ”Okoye said.
Her preferred biologics include adalimumab, ustekinumab, guselkumab (for ulcerative HS), secukinumab (for HS and inflammatory arthritis), and anakinra. She also mentioned her use of high dose prednisone (80 mg per day) with prolonged slow tapering for acute control at the start of biologic therapy, or as maintenance therapy with 20 mg once a week or 20-40 mg. once at the start of a burst.
“It’s very similar to how you would treat a patient with pyoderma gangrenosum (PG), so I’m kind of justifying myself that these are people I’m trying to keep out of the hospital,” Okoye said. . “They walk around with 10cm ulcers. So I’m using prednisone in a high dose, with a long, slow taper similar to what you would for the PG.
In the gluteal subtype, HS lesions mostly appear on the buttocks and consist of follicular papules and folliculitis, according to Okoye. Patients tend to be current or former smokers, males, and have a lower BMI than patients with the axillary-mammary-inguinal HS subtype.
“These patients, I believe, are at increased risk for squamous cell carcinoma (SCC),” Okoye said. “I don’t believe this is in the literature per se, but if you look at the literature on SCC and HS you will see that most lesions occur in the buttocks, SCC and HS are more common in men, smokers, and people with this disease for 25 years or more. It really sounds like those patients to me. “
For the treatment of this subtype, Okoye suggests using surgical excision and biologicals, as well as monitoring for SCC in the affected area and stopping smoking.
“So my favorite therapy for these patients is surgery. I don’t find that they respond very well to medical treatment. In fact, for some of these patients, I think it’s a pilonidal cyst that has gotten completely out of control and just needs to be removed. And then, for most of them, they’ll do some sort of organic product, ”Okoye said.
1. Okoye G. My approach to the treatment of HS in patients with colored skin. Featured at: Skin of Color Update; September 10, 2021; Virtual.
Disclosure: Okoye is a consultant for Janssen and Unilever. She also received a grant from Pfizer and serves on an advisory board for Lilly, Novartis, UCB and Pfizer.