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Hidradenitis Suppurativa: A Disease Under-diagnosed and Under-treated

by Debra Shelby, PhD, DNP, DCNP, FNP-BC, FACDNP, FAANP

Intro

Hidradenitis suppurativa (HS) is a chronic and progressive skin disease that is often misdiagnosed, incorrectly treated or under-treated. Providers need to be more vigilant for early diagnosis and intervention. Many patients are not referred to the dermatology specialist until the patient has progressed into the later stages due to this disease resembling staphylococcal furunculosis, carbuncle, ruptured cyst, cat-scratch fever, and lymphadenitis. Sinus tracts, double comedones, and hypertrophic
scars help distinguish HS from other diseases but still can deceive the inexperienced clinician. 1,2,3

Overview
HS targets the apocrine-gland bearing sites and is most commonly found in axillae and groin. This chronic condition affects more women than men and affects all races. It presents with recurring boils and draining sinus tracts at or after the age of puberty. The etiology is unknown and predisposing factors include obesity, genetic predisposition to acne, apocrine duct obstruction, and secondary bacterial infection. 1,2,3
Pathogenesis is believed to be caused by keratin plugging of the apocrine duct and hair follicle causing a dilatation of the apocrine duct and hair follicle. Inflammatory changes of the gland lead to bacterial growth and finally rupturing of the gland resulting in an extension of inflammation and infection. Suppuration and tissue destruction causes ulceration and fibrosis leading to sinus tract formation.1
Initially, the disease starts with inflammatory nodules and sterile abscesses in the groin, axillae, perianal and inframammary areas. As the disease progresses, sinus tracts and hypertrophic scars form. 2 Workup includes a thorough history and physical, culture for secondary bacterial infections. If a biopsy is taken, early disease shows keratin occlusion of apocrine duct and hair follicle, dilatation, inflammatory changes, limited to a single apocrine gland. Late disease shows the destruction of apocrine/eccrine and pilosebaceous apparatus, fibrosis, and hyperplasia in sinuses.1,2,3

Treatments
Patient often seeks a dermatology specialist out of frustration and desperation of dealing with this disease for years without relief. Many are prescribed years of the same medications without consideration for other advanced therapies. In fact, many patients have a poor understanding of what the disease is and life-style changes that can improve symptoms. A comprehensive approach and patient education is critical for successful treatment. Why is this so important? Complications can include depression, anemia, secondary amyloidosis, lymphedema, fistulas, arthropathy, and although rare, SCC arising from the scars. 2,3
Management requires combination therapy. Antibiotics are part of the treatment plan, but should not be the only intervention. Intralesional glucocorticoids, surgery, laser, oral antibiotics, and isotretinoin are used in the early stages of this disease. Prednisone may be given is pain and inflammation are severe. While different treatments have been tried, no therapy is successful for everyone. 1,2, 3 One treatment that is underutilized and not noted in many dermatology references is biologic therapy. Adalimumab is the first and only FDA approved treatment for the treatment of moderate to severe HS. This drug targets a source of HS inflammation which is a protein called TNF-alpha. The randomized control study Pioneer I and II primary endpoint was the proportion of patients achieving HiSCR at 12 weeks. HiSCR was defined as at least a 50% reduction in the total abscess and inflammatory nodule count with no increase in abscess count and no increase in draining fistula count relative to baseline. Referral to the dermatology specialist to evaluate the patient as a candidate for this medication should
not delayed. 4,5 Lifestyle changes should include healthy eating, reducing stress, avoid loose clothing and smoking
cessation. Psychological effects like depression also need to be evaluated. 5,6

Conclusion
HS is a chronic disease that is often misdiagnosed, under-treated or incorrectly treated. These patients suffer psychological and physical effects from pain, draining pus, odor, and from the embarrassing areas affected (perianal). Depression is commonly seen. Therefore, it is crucial for the provider to identify and intervene early with the appropriate therapies. Prolonged therapy without desired results causes frustration with the patient and provider. Assessing the need for advanced treatment like biologic
therapy is warranted with the first signs of disease progression or failure of commonly prescribed treatments. Primary care education focusing on early detection and referral to a dermatology provider will prove to be essential for improved patient outcomes.

1. Wolff, K.,Johnson, R. & Suumond, D. (2005). Fitzpatrick’s Color Atlas & Synopsis of Clinical
Dermatology 5th ed. McGraw-Hill: NY. pages 14-16
2. Bolognia, J., Schaffer, J., Duncan, K., and Ko, C. (2014). Dermatology Essentials. Elsevier:China. Page
277.
3. Bolognia,J. (2003 ).Dermatology Vol.1. Mosby page 566.
4. Retrieved 1/8/18: https://www.humirapro.com/dermatology/hidradenitis-suppurativa-clinical-data
5. Retrieved 1/7/18: https://www.humira.com/hidradenitis-suppurativa
6. Retrieved 1/8/18: https://www.aad.org/public/diseases/painful-skin-joints/hidradenitissuppurativa#tips
See Debra Shelby, PhD, DNP, DCNP, FNP-BC, FACDNP, FAANP in 2018 at a Skin, Bones, Hearts & Private Parts event in Myrtle Beach, Pensacola Beach, San Antonio, or Las Vegas.