EM@3AM: Hidradenitis Suppurativa
- Jul 27th, 2024
- Cassandra Mackey
- categories:
Authors: Cassandra Mackey, MD (Assistant Professor, UMass Chan Medical School); Clifford Rodgers, MD (EM Resident Physician, UTSW / Parkland Health and Hospital System); Gilberto Salazar, MD (EM Attending Physician, UTSW / Parkland Health and Hospital System) // Reviewed by: Alex Koyfman, MD (@EMHighAK, EM Attending Physician, UTSW / Parkland Memorial Hospital); Sophia Görgens, MD (EM Physician, Northwell, NY); Brit Long, MD (@long_brit)
Welcome to EM@3AM, an emDOCs series designed to foster your working knowledge by providing an expedited review of clinical basics. We’ll keep it short, while you keep that EM brain sharp.
A 27-year-old woman with a history of obesity and diabetes presents to the emergency department for lesions on her axilla. The patient states that she has noticed these lesions for the past two years and assumed they were abscesses. The lesions have been tender, itchy, and with malodorous drainage. Her vital signs are T 37°C, BP 124/79, HR 89, RR 18, and SpO2 100% on room air. There are multiple hyperpigmented, palpable, tender masses on her bilateral axillae which exhibit scant drainage of fluid as pictured below.
Question: What is the diagnosis?
Answer: Hidradenitis Suppurativa (acne inversa)
Definition2
- Chronic inflammatory condition involving the apocrine gland-bearing areas of skin
- Characterized by recurrent painful draining nodules.
- Typically affected areas include:
- Axillae (most common)
- Gluteal folds
- Inframammary areas
- Perianal and pubic/genitofemoral areas.
Differential Diagnosis
- Pilonidal cyst
- Cellulitis
- Granuloma inguinale
- Furuncle
- Abscess
- Lymphogranuloma venereum
- Keloid
Epidemiology3-6
- More common in women and black or biracial individuals
- Western countries => female > male
- Asian countries => male > female
- Prevalence of ~1% with a wide range of prevalence globally citing between 0.03%- 4%.
- Age of onset
- Rarely before puberty
- Women: peak age 11 to 20 years
- Men: peak age 21 to 30
- Major risk factors include:
- Obesity
- Tobacco
- Diabetes Mellitus
Pathophysiology
- Three pathogenetic events (see image 2)
- Epidermal hyperkeratosis with occlusion and dilation
- Follicular epithelial wall rupture resulting in infiltration of inflammatory cells
- Formation of interconnecting sinus tracts
- Hurley Staging is a standardized staging system that describes the severity of HS and helps to guide treatment.
Complications9
- Repeated episodes => can cause morbidity and decrease quality of life
- Cutaneous complications:
- Sinus tracts
- Fistulae
- Scarring/contractures
- Squamous cell carcinoma
- Lymphedema
- Systemic complications:
- Chronic pain
- Amyloidosis
- Anemia
- Cutaneous complications:
Treatment10,11
- Consider dermatology follow up early in disease
- See table 2 for treatment based on severity of disease
- Of note, avoid I&D of abscesses in the ED if possible when HS is suspected as recurrence rate is nearly 100%.
- I&D may be needed for pain relief or source control if patient is meeting sepsis criteria
Prognosis12-19
- HS can lead to substantial complications such as squamous cell carcinoma (SCC), with a higher ratio in males than females.
- About 61% of patients with HS may develop SCC in the perineum or buttocks
- 48% of these patients died within two years
- SCC may arise within an area of chronic inflammation or a pre-existing scar.
- A probable factor in the development of SCC could be infection with human papillomavirus.
- About 61% of patients with HS may develop SCC in the perineum or buttocks
- HS can also lead to anemia, anal and urethral strictures and fistulas, lumbosacral epidural abscess, and sacral bacterial osteomyelitis.
Pearls18
- Early dermatology referral to prevent morbidity and mortality.
- Consider HS in obese patients, especially diabetics, with multiple recurrent abscesses.
- Avoid I&D of abscesses in the ED if possible when HS is suspected as recurrence rate is nearly 100%.
- Most cases of mild HS may be managed in the ED with topical clindamycin 1% solution and prompt PCP or dermatology follow-up and referral.
A 26-year-old man with a history of type 2 diabetes and hypertension presents with multiple lumps under his bilateral axillae that occasionally hurt and drain foul-smelling, purulent fluid. He initially thought the lumps were abscesses, but the pain and drainage of fluid seem to resolve spontaneously before recurring again after a few weeks. He reports no fever, chills, weight loss, chest pain, palpitations, or shortness of breath. On physical exam, there are bands of scar tissue and several inflamed dermal nodules and tunnels draining a modest amount of purulent fluid in the bilateral axillae and left groin fold. Which of the following best describes the pathophysiology of this condition?
A) Bacterial proliferation in the dermis with a neutrophil-predominant inflammatory response
B) Chronic bacterial infection of hair follicles
C) Cutaneous proliferation of Klebsiella granulomatis
D) Cystic outpouching of the hair follicle lining with keratin impaction
E) Occlusion of hair follicles, local immune dysregulation, and chronic inflammation
Answer: E
Hidradenitis suppurativa is a chronic, relapsing inflammatory skin condition caused by hyperkeratosis, hair follicle occlusion and rupture, and dysregulated immune response to local antigens. It often presents with flares of extremely tender, inflamed, deep dermal nodules and tunnels that may open and drain purulent or serosanguinous fluid. High-friction intertriginous areas are typically affected, such as the axillary, inguinal, inframammary, infra-abdominal, intergluteal, perianal, and waistline regions.
The average onset is between 18 and 29 years of age, with relatively few cases occurring before puberty or after menopause, suggesting a possible influence of androgenic hormones on skin keratinization. It is not thought to be caused by bacterial overgrowth, although antibiotic therapy has an anti-inflammatory effect on active lesions.
First-line therapies include topical clindamycin 1%, oral doxycycline, combined oral contraceptive pills, spironolactone, and metformin. Severe or refractory lesions can be treated with incision and drainage, intralesional corticosteroids, oral retinoids, dapsone, tumor necrosis factor inhibitors, and surgical excision.
Bacterial proliferation in the dermis with a neutrophil-predominant inflammatory response (A) would be expected in an acute soft tissue infection such as cellulitis or abscess formation. Most abscesses are caused by Staphylococcus bacteria, while cellulitis is typically caused by Staphylococcus or Streptococcus bacteria.
Chronic bacterial infection of hair follicles (B) describes bacterial folliculitis and is most commonly caused by Staphylococcus aureus or Pseudomonas aeruginosa. Inflammatory follicular pustules and papules are characteristic. Pseudomonas folliculitis is associated with skin exposure to improperly treated water sources and may be pruritic.
Cutaneous proliferation of Klebsiella granulomatis (C) occurs in granuloma inguinale, or donovanosis. Often sexually transmitted, it results in chronic, suppurative, painless anogenital ulcerations and granuloma formation.
Cystic outpouching of the hair follicle lining with keratin impaction (D) describes an epidermal inclusion cyst. Inclusion cysts can be distinguished from abscesses by the presence of a central punctum (opening of the occluded follicle) and lack of fever or other systemic symptoms.
Further Reading:
- Ajayi A, Richmond J, Okoye G and Byrd A (2023) Hidradenitis Suppurativa—Painful Boils That Affect Much More Than Skin. Front. Young Minds. 11:782192. doi: 10.3389/frym.2022.782192
- Morrell D, Bowers E. Skin disorders: Groin and skinfolds. In Tintinalli J, ed. Tintinalli’s Emergency Medicine: A Comprehensive Study Guide (8th ed). New York: McGraw Hill Medical; 2011:1669-70
- Garg A, et al. JAMA Dermatol. 2017;153:760-4.
- Chu CB, Yang CC, Tsai SJ. Hidradenitis suppurativa: Disease pathophysiology and sex hormones. Chin J Physiol 2021;64:257-65
- Calao M, Wilson JL, Spelman L, Billot L, Rubel D, Watts AD, Jemec GBE. Hidradenitis Suppurativa (HS) prevalence, demographics and management pathways in Australia: A population-based cross-sectional study. PLoS One. 2018 Jul 24;13(7)
- Palmer R, Keefe M. Clin Dermatol.2001;26:501-3
- Chu CB, Yang CC, Tsai SJ. Hidradenitis suppurativa: Disease pathophysiology and sex hormones. Chin J Physiol 2021;64:257-65
- Ovadja ZN, Schuit MM, van der Horst CMAM, Lapid O. Inter- and intrarater reliability of Hurley staging for hidradenitis suppurativa. Br J Dermatol. 2019 Aug;181(2):344-349. doi: 10.1111/bjd.17588. Epub 2019 Mar 14. PMID: 30585304; PMCID: PMC6850108.
- Yuan JT, Naik HB. Complications of hidradenitis suppurativa. Semin Cutan Med Surg. 2017 Jun;36(2):79-85. doi: 10.12788/j.sder.2017.022. PMID: 28538749.
- Saunte DML, Jemec GBE. Hidradenitis Suppurativa: Advances in Diagnosis and Treatment. 2017;318(20):2019–2032. doi:10.1001/jama.2017.16691
- Nesbitt E, Clements S, Driscoll M. A concise clinician’s guide to therapy for hidradenitis suppurativa. Int J Womens Dermatol. 2019;6(2):80-84. Published 2019 Dec 27. doi:10.1016/j.ijwd.2019.11.004
- Maclean GM, Coleman DJ. Three fatal cases of squamous cell carcinoma arising in chronic perineal hidradenitis suppurativa. Ann R Coll Surg Engl. 2007;89:709–712.
- Cosman BC, O’Grady TC, Pekarske S. Verrucous carcinoma arising in hidradenitis suppurativa. Int J Colorectal Dis. 2000;15:342–346.
- Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol. 2009;60(4):539–561.
- Russ E, Castillo M. Lumbosacral epidural abscess due to hidradenitis suppurativa. AJR Am J Roentgenol. 2002;178:770–781.
- Lapins J, Ye W, Nyrén O, Emtestam L. Incidence of cancer among patients with hidradenitis suppurativa. Arch Dermatol. 2001;137:730–734.
- Chawla S, Toale C, Morris M, Tobin AM, Kavanagh D. Surgical Management of Hidradenitis Suppurativa: A Narrative Review. J Clin Aesthet Dermatol. 2022 Jan;15(1):35-41. PMID: 35309275; PMCID: PMC8903230.
- Serrano L, Ulschmid C, Szabo A, Roth G, Sokumbi O. Racial disparities of delay in diagnosis and dermatologic care for hidradenitis suppurativa. J Natl Med Assoc. 2022 Dec;114(6):613-616. doi: 10.1016/j.jnma.2022.08.002. Epub 2022 Oct 28. PMID: 36511276.
- Racanelli E, Jfri A, Gefri A, O’Brien E, Litvinov IV, Zubarev A, Savin E, Netchiporouk E. Cutaneous Squamous Cell Carcinoma in Patients with Hidradenitis Suppurativa. Cancers. 2021; 13(5):1153.
