84: Hidradenitis Suppurativa Flashcards
What is Hidradenitis Suppurativa (HS) and its primary characteristics?
Hidradenitis suppurativa (HS) is a chronic inflammatory disorder of hair follicles affecting intertriginous and anogenital regions. It is characterized by inflammatory nodules, subcutaneous abscesses, sinus tracts, recurrent painful lesions, and hypertrophic scarring.
What are the long-term sequelae associated with Hidradenitis Suppurativa?
The long-term sequelae of Hidradenitis Suppurativa include chronic pain, skin contractures, and disfigurement.
How does the epidemiology of Hidradenitis Suppurativa differ between genders?
Hidradenitis Suppurativa is disproportionately affected by women, with a female-to-male ratio of 3.3:1. Women are more likely to develop axillary and genitofemoral lesions, while men tend to develop perineal and perianal disease.
What is the typical clinical course of Hidradenitis Suppurativa?
Hidradenitis Suppurativa is a chronic relapsing and remitting disease with an unpredictable clinical course. Lesions may start as tenderness or pruritus, progressing to painful nodules that can resolve or develop into abscess-like lesions.
What is the underlying pathophysiology of foul-smelling drainage from sinus tracts in HS?
The foul-smelling drainage is due to repeated episodes of follicular rupture and inflammation, leading to sinus tract formation and intermittent drainage of serosanguinous and/or purulent material.
What causes delayed healing in patients with HS experiencing persistent open wounds?
Delayed healing in HS is often due to repeated episodes of inflammation and rupture, leading to persistent open wounds with granulation tissue.
What are the key characteristics of Hidradenitis Suppurativa (HS) and its clinical features?
Hidradenitis Suppurativa (HS) is characterized by chronic inflammatory disorder of hair follicles, recurrent painful lesions, and an unpredictable clinical course with long-term sequelae including chronic pain and disfigurement.
What are the long-term complications associated with Hidradenitis Suppurativa?
The long-term complications of Hidradenitis Suppurativa (HS) include dermal contractures and disfigurement due to chronic lesions and scarring.
What is the impact of Hidradenitis Suppurativa (HS) on the Dermatology Life Quality Index?
The Dermatology Life Quality Index for HS is more severe than for other dermatologic conditions, with the highest scores associated with disease-related pain and discomfort.
What systemic complications may arise from Hidradenitis Suppurativa?
Systemic complications of HS may include normocytic and/or microcytic anemia, rare cases of renal amyloidosis, and sepsis from infected lesions.
What local complications can arise from Hidradenitis Suppurativa?
Local complications of HS include fibrosis and dermal contractures, strictures from chronic inflammation, urethral fistulization, and disfiguring conditions requiring surgical reconstruction.
What is the relationship between Hidradenitis Suppurativa and Squamous Cell Carcinoma (SCC)?
Squamous Cell Carcinoma (SCC) is an infrequent complication of HS, occurring in 4.6% of cases, particularly in males with anogenital disease.
What factors contribute to the etiology of Hidradenitis Suppurativa?
The etiology of HS is multifactorial, involving genetic predisposition, aberrant immunity, hormonal dysregulation, and environmental modifiers.
What is follicular occlusion and its significance in Hidradenitis Suppurativa?
Follicular occlusion is a histologic finding in early HS, characterized by keratinous occlusion of the terminal hair follicle, leading to plugging and dilation of hair follicles.
What local complication might limit joint mobility in patients with HS?
Fibrosis and dermal contractures can limit joint mobility in patients with HS.
What complication should be suspected in a patient with longstanding HS presenting with a new ulcerated lesion?
Suspect squamous cell carcinoma (SCC), which is a serious complication of longstanding HS, especially in males with anogenital disease.
What is the likely cause of anemia in patients with HS?
Normocytic and/or microcytic anemia in HS is typically secondary to chronic inflammation.
What is the relative risk of malignancy for patients with HS compared to healthy individuals?
Patients with HS are at a 50% greater risk of developing malignancies compared to healthy individuals.
What role does follicular occlusion play in the pathogenesis of HS?
Follicular occlusion, caused by keratinous plugging of terminal hair follicles, is the primary pathophysiologic event in HS.
What is the impact of anogenital involvement on the Dermatology Life Quality Index in HS?
Anogenital involvement is associated with a significantly worse Dermatology Life Quality Index, indicating greater disease-associated pain and discomfort.
What are the systemic complications that may arise in patients with Hidradenitis Suppurativa?
Systemic complications may include normocytic and/or microcytic anemia, rare cases of renal amyloidosis, and sepsis from infected lesions.
What local complications can develop from chronic anogenital and perineal inflammation in HS?
Local complications can include fibrosis and dermal contractures, strictures leading to incontinence, urethral fistulization, and disfiguring conditions requiring surgical reconstruction.
How does the risk of developing squamous cell carcinoma (SCC) compare between patients with HS and healthy individuals?
Patients with Hidradenitis Suppurativa are at a 50% greater risk of developing malignancies, with SCC occurring in 4.6% of HS cases.
What are the primary factors thought to contribute to the etiology and pathogenesis of Hidradenitis Suppurativa?
The etiology of Hidradenitis Suppurativa is multifactorial, involving genetic predisposition, aberrant immunity, hormonal dysregulation, and environmental modifiers.
What is the role of smoking in the development and severity of Hidradenitis Suppurativa (HS)?
Smoking is associated with a higher likelihood of having HS and is linked to more severe disease. Cessation of smoking may improve symptoms and reduce new lesions after surgery.
What genetic factors are associated with Hidradenitis Suppurativa (HS)?
HS can develop sporadically or be inherited, with about one-third of patients reporting a positive family history and mutations in the β-secretase complex genes identified.
How does obesity influence Hidradenitis Suppurativa (HS)?
Obesity is associated with greater odds of having HS, exacerbating the condition by promoting sweat retention and increasing friction in skinfolds.
What is the significance of adnexal structures in the pathogenesis of Hidradenitis Suppurativa (HS)?
Adnexal structures, particularly sebaceous glands, play a crucial role in HS pathogenesis, with sebaceous gland atrophy preceding follicular inflammation.
What is the potential role of androgens in Hidradenitis Suppurativa (HS)?
The propensity for HS to develop after puberty suggests an androgenic basis for the disease, although the exact role remains unclear.
What role does immune dysregulation play in Hidradenitis Suppurativa (HS)?
Immune dysregulation is implicated in HS pathogenesis, with elevated proinflammatory cytokines found in lesional skin.
What are the common bacterial organisms associated with Hidradenitis Suppurativa lesions?
Coagulase-negative staphylococcus and anaerobic bacteria are the most frequently isolated organisms in HS lesions.
What comorbidities are frequently associated with Hidradenitis Suppurativa?
HS is frequently associated with other diseases of follicular occlusion, including acne conglobata, dissecting cellulitis of the scalp, and pilonidal cyst.
What are the common bacterial organisms associated with Hidradenitis Suppurativa lesions?
Coagulase-negative staphylococcus and anaerobic bacteria are the most frequently isolated organisms in HS lesions, with deep tissue samples showing a predominance of coagulase-negative staphylococcus.
What comorbidities are frequently associated with Hidradenitis Suppurativa?
HS is frequently associated with other diseases of follicular occlusion, including acne conglobata, dissecting cellulitis of the scalp, and pilonidal cysts. It is also linked to autoinflammatory syndromes like PASH, PAPASH, and PsAPASH, as well as inflammatory bowel disease.
How does the coexistence of Crohn disease affect Hidradenitis Suppurativa?
The coexistence of Crohn disease and HS is associated with a more fulminant disease course, particularly in patients with perianal involvement, and clinical improvement has been reported with anti-TNF therapy.
What are the implications of metabolic syndrome in patients with Hidradenitis Suppurativa?
Patients with HS have significantly higher odds of having metabolic syndrome, which includes conditions such as diabetes mellitus, obesity, and hypertension.
What role do proinflammatory cytokines play in the pathogenesis of HS?
Proinflammatory cytokines such as TNF and IL-1 are markedly elevated in HS lesions, driving inflammation and disease progression.
What role do bacteria play in the pathogenesis of HS?
Bacterial involvement in HS lesions may trigger an overzealous immune response to commensal flora, leading to follicular inflammation.
What role does immune dysregulation play in the pathogenesis of HS?
Immune dysregulation, characterized by elevated levels of TNF, IL-1, IL-12, and IL-23, is an integral part of HS pathogenesis.
What are the three criteria required for the clinical diagnosis of Hidradenitis Suppurativa (HS)?
- Typical lesions must be present, including deep-seated painful nodules, abscesses, draining sinuses, double-open comedones, and bridged scars.
- Lesions must exhibit a typical distribution in areas such as the axillae, groin, buttocks, perineal, or inframammary region, with possible nonclassical sites present.
- There must be a clear history of symptom chronicity and recurrence.
How does the Hurley staging system classify the severity of Hidradenitis Suppurativa?
The Hurley staging system classifies HS severity into three stages:
Stage | Characteristics |
|——-|—————-|
| I | Recurrent abscesses without scarring or sinus tracts |
| II | Recurrent abscesses with scarring and sinus tract(s), separated by normal skin |
| III | Recurrent abscesses with diffuse scarring and interconnected sinus tracts with minimal to no normal skin between lesions |
What are the clinical phenotypes of Hidradenitis Suppurativa?
- Classic axillary mammary subtype: Characterized by breast and axillary involvement with hypertrophic scarring, accounting for 48% of patients.
- Follicular subtype: Characterized by a predilection for follicular lesions (e.g., epidermal cysts, pilonidal sinus, comedones, severe acne) and atypical topography involving the ears, chest, back, or legs, comprising 26% of patients.
What are the characteristics of patients with the follicular subtype of Hidradenitis Suppurativa (HS)?
Patients with the follicular subtype of HS are more likely to be male smokers with a family history of HS and greater disease severity compared to those with the classic axillary-mammary subtype.
What laboratory tests are recommended for patients with acute lesions of Hidradenitis Suppurativa?
Patients with acute lesions may exhibit:
- Leukocytosis
- Elevated erythrocyte sedimentation rate
- Low serum iron levels
- Serum protein abnormalities
Additionally, chemistries, complete blood counts, and blood cultures should be sent for febrile or toxic patients, and purulent drainage should be sent for bacterial cultures and sensitivities.
What imaging techniques may be used for staging and surgical planning in Hidradenitis Suppurativa?
Ultrasonography and MRI may be used to visualize lesions for staging and surgical planning, although these strategies are rarely utilized.
What is a universal histopathologic finding in Hidradenitis Suppurativa, regardless of disease duration?
Follicular occlusion is a nonspecific but universal histopathologic finding in Hidradenitis Suppurativa, irrespective of disease duration.
What are the characteristics of early lesions in Hidradenitis Suppurativa?
Early lesions in Hidradenitis Suppurativa are characterized by:
- Follicular hyperkeratosis of the terminal hair follicles
- Hyperplasia of the follicular infundibulum
- Perifolliculitis
These processes lead to keratinous plugging of the terminal hair follicles, a consistent histologic finding that precedes follicular dilation and rupture.
What types of inflammatory cells are predominantly found in acute lesions of Hidradenitis Suppurativa?
In acute lesions of Hidradenitis Suppurativa, the inflammatory infiltrate is largely comprised of T-lymphocytes and neutrophils at the follicular epithelium, with variable extension into the adnexal structures.
How does the ratio of CD8+ cytotoxic T-lymphocytes to CD4+ helper T-lymphocytes change over the lifetime of active lesions in Hidradenitis Suppurativa?
The ratio of CD8+ cytotoxic T-lymphocytes to CD4+ helper T-lymphocytes appears to increase over the lifetime of active lesions in Hidradenitis Suppurativa.
What is the typical age range for the onset of Hidradenitis Suppurativa (HS)?
The typical age range for the onset of Hidradenitis Suppurativa is from 16 to 81 years, usually after puberty.
How does smoking cessation impact the remission rates in Hidradenitis Suppurativa patients?
Nonsmokers achieved a higher rate of remission (40%) compared to active smokers (29%).
What are the treatment approaches for Hurley stage II disease in Hidradenitis Suppurativa?
Hurley stage II disease (moderate) may require both medical therapy and localized surgical excisions.
What is the significance of weight loss after bariatric surgery for patients with Hidradenitis Suppurativa?
Weight loss of 15% or more from baseline after bariatric surgery was associated with a 20% reduction in the number of active, eruptive sites.
What are the overall goals of therapy for Hidradenitis Suppurativa?
The overall goals of therapy are to prevent the formation of primary lesions and to reduce the impact of disease sequelae (e.g., fibrosis, contractures, sinus tracts) on quality of life.
What conservative measures can help alleviate symptoms of Hidradenitis Suppurativa?
Conservative measures include stress reduction, warm baths, warm compresses, and hydrotherapy.
What is the role of bleach baths in the management of Hidradenitis Suppurativa?
Taking bleach baths (one-quarter cup of regular bleach diluted in a full tub of water) can reduce bacterial load and decrease malodor when done 2 to 3 times per week.
What are the characteristics of Hurley stage III disease in Hidradenitis Suppurativa?
Hurley stage III disease (severe) often requires extensive wide excisional surgical procedures with advanced grafting and flap procedures.
What are the characteristics and clinical implications of the follicular and gluteal subtypes of Hidradenitis Suppurativa (HS)?
The follicular subtype is more common in male smokers with a family history of HS and greater disease severity. The gluteal subtype, comprising 26% of patients, is characterized by follicular papules and folliculitis, and these patients tend to have lower body mass indexes and more indolent disease compared to the axillary mammary subtype.
What is adenitis Suppurativa?
Taking bleach baths (one-quarter cup of regular bleach diluted in a full tub of water) can reduce bacterial load and decrease malodor when done 2 to 3 times per week.
What lifestyle modifications would you recommend for a 35-year-old female with HS?
Smoking cessation and weight management are critical. Nonsmokers have a higher remission rate (40%) compared to active smokers (29%). Weight loss of 15% or more from baseline can reduce the number of active sites by 20%.
What potential benefit could bariatric surgery provide for a patient with HS?
Weight loss of more than 15% after bariatric surgery is associated with a 20% reduction in the number of active, eruptive sites.
What procedures might be required for a patient with Hurley stage III HS considering surgical options?
Extensive wide excisional surgical procedures with advanced grafting and flap procedures are often required for Hurley stage III disease.
What role does stress reduction play in the management of HS?
Stress reduction, along with warm baths, warm compresses, and hydrotherapy, can help alleviate symptoms of HS.
What is the typical age range for the onset of Hidradenitis Suppurativa (HS)?
The typical age range for the onset of HS is from 16 to 81 years. Remissions are significantly more likely in nonsmokers, those who have quit smoking, and in nonobese individuals.
How does the severity of Hidradenitis Suppurativa influence treatment decisions?
Treatment decisions for HS are generally guided by disease severity:
- Hurley Stage I (mild): Typically amenable to medical therapy alone.
- Hurley Stage II (moderate): May require both medical therapy and localized surgical excisions.
- Hurley Stage III (severe): Often requires extensive wide excisional surgical procedures with advanced grafting and flap procedures.
What lifestyle modifications can significantly impact the management of Hidradenitis Suppurativa?
Key lifestyle modifications that can impact HS management include:
- Smoking cessation: Nonsmokers achieved a 40% remission rate compared to 29% in active smokers.
- Weight management: A weight loss of 15% or more from baseline after bariatric surgery was associated with a 20% reduction in the number of active, eruptive sites.
- Stress reduction and hydrotherapy: Conservative measures like warm baths and compresses may help alleviate symptoms.
What are the goals of therapy for Hidradenitis Suppurativa?
The overall goals of therapy for HS are to:
- Prevent formation of primary lesions.
- Reduce the impact of disease sequelae such as fibrosis, contractures, and sinus tracts on quality of life.
What are some effective home remedies for alleviating symptoms of Hidradenitis Suppurativa?
Effective home remedies for alleviating HS symptoms include:
- Bleach baths: Diluting one-quarter cup of bleach in a full tub of water can reduce bacterial load and decrease malodor.
- Topical agents: Using resorcinol 10% to 15% cream can improve pain and reduce the duration of painful abscesses.
- Dressings: Useful for managing drainage and decreasing malodor.
- Nonsteroidal anti-inflammatory drugs: Short courses can help alleviate pain and reduce inflammation.
What lifestyle modifications should patients with hidradenitis suppurativa consider to avoid triggers?
Patients should avoid tight clothing, prolonged exposure to heat and humidity, and shaving if these are noted as triggers.
What is the role of insulinotropic milk and hyperglycemic foods in hidradenitis suppurativa?
Consumption of insulinotropic milk and hyperglycemic foods may upregulate the PI3K/Akt-signaling pathway, leading to nuclear deficiency of FoxO1 transcription factor, which is thought to play a role in acne vulgaris and acne-like eruptions.
What are the mainstays of medical therapy for mild-to-moderate hidradenitis suppurativa?
Topical and oral antibiotic therapy are the mainstays, with clindamycin 1% lotion applied twice daily being effective in reducing pustules, inflammatory nodules, and abscesses.
What alternative treatments can be used for patients with more severe hidradenitis suppurativa?
For more severe disease, a regimen of 300 mg of clindamycin and 300 mg of rifampin taken 2 to 3 times daily may be effective, along with triple therapy using rifampin, moxifloxacin, and metronidazole.
What biological therapies are available for moderate-to-severe hidradenitis suppurativa?
Biological therapies include adalimumab, infliximab, ustekinumab, and anakinra, which may be used for patients unresponsive to or intolerant of antibiotic therapy.
What is the evidence for hormonal therapy in the treatment of hidradenitis suppurativa?
Androgen modulation therapy, including spironolactone and finasteride, can be trialed as second-line therapy for patients with mild-to-moderate HS, showing some improvement in small case series.
What is the evidence supporting the use of dapsone in HS?
There is weak-to-moderate evidence supporting a course of dapsone (50 to 200 mg daily for 4 to 12 weeks) in refractory HS.
What antibiotic regimen would you consider for a patient with Hurley stage II HS who has not responded to topical clindamycin and oral tetracyclines?
A regimen of 300 mg of clindamycin and 300 mg of rifampin taken 2 to 3 times daily may be effective for refractory Hurley stage II disease.
What alternative biologic therapy could be considered for a patient with HS prescribed adalimumab but shows no improvement?
Infliximab, a chimeric monoclonal antibody inhibitor of TNF, dosed at 5 mg/kg at weeks 0, 2, and 6, followed by every 8 weeks thereafter, could be considered.
What is the rationale for using spironolactone in HS?
Spironolactone, an androgen modulation therapy, can be trialed as a second-line therapy for patients with mild-to-moderate HS.
What immediate treatment could be considered for a patient with HS experiencing severe pain and inflammation in a single lesion?
Intralesional steroid injections of triamcinolone acetonide suspension, 5 to 10 mg/mL, can decrease inflammation in acutely flaring lesions.
What role does environmental modification play in the management of HS?
Avoiding tight clothing, prolonged heat and humidity, and shaving can help reduce triggers for HS lesions.
What role does diet play in the management of HS?
Decreasing exposure to dairy and high-glycemic-index foods may reduce HS lesions by downregulating the PI3K/Akt-signaling pathway.
What lifestyle modifications should patients with hidradenitis suppurativa be advised to make to avoid triggers?
Patients should avoid:
- Tight clothing
- Prolonged exposure to heat and humidity
- Shaving if noted as a trigger.
What is the role of insulinotropic milk and hyperglycemic foods in the management of hidradenitis suppurativa?
Consumption of insulinotropic milk and hyperglycemic foods may:
- Upregulate the PI3K/Akt-signaling pathway
- Lead to nuclear deficiency of FoxO1 transcription factor, which is thought to play a role in acne vulgaris and acne-like eruptions.
What are the recommended antibiotic therapies for mild-to-moderate hidradenitis suppurativa?
The mainstays of management include:
- Clindamycin 1% lotion applied twice daily
- Topical metronidazole 0.75% or erythromycin 2% as alternatives
- A 4-month trial of oral tetracyclines for their anti-inflammatory effect.
What is the significance of biologic therapy in the treatment of moderate-to-severe hidradenitis suppurativa?
Biologic therapy is significant for patients who are unresponsive to or intolerant of antibiotic therapy.
What are the potential benefits of hormonal therapy in managing hidradenitis suppurativa?
Hormonal therapy can be trialed as a second-line treatment for patients with mild-to-moderate HS. Benefits observed in small case series include:
- Spironolactone (100 to 150 mg daily)
- Finasteride (5 mg daily).
What is the role of Metformin in the management of Hidradenitis Suppurativa (HS)?
Metformin is an insulin-sensitizing biguanide agent used as a second-line option for women with HS and comorbid polycystic ovarian syndrome and/or metabolic syndrome.
What are the surgical options available for managing chronic Hidradenitis Suppurativa?
Surgical options include:
- Simple incision and drainage for milder disease.
- Local excisions and/or deroofing of sinus tracts.
- Wide local excisions with primary closure for more severe cases.
How do laser therapies assist in the treatment of Hidradenitis Suppurativa?
Laser therapies are used as adjunctive treatments for HS by:
- Mitigating flares through debulking tissue.
- Reducing the number of hair follicles, sebaceous glands, and bacteria in affected areas.
What are the pain management strategies for patients with Hidradenitis Suppurativa?
Pain management strategies include:
- Topical analgesics and oral acetaminophen.
- Oral nonsteroidal anti-inflammatory drugs as first-line therapy.
What is the efficacy of cryotherapy in treating Hidradenitis Suppurativa?
Cryotherapy has shown to improve symptoms in 8 out of 10 patients with limited, painful nodules.
What are the benefits of laser therapy for a patient with HS?
Laser therapies mitigate flares by debulking tissue and reducing the number of hair follicles, sebaceous glands, and bacteria in affected areas.
What medication might be particularly beneficial for a 28-year-old female with HS and polycystic ovarian syndrome (PCOS)?
Metformin, an insulin-sensitizing agent, is appealing for women with HS and comorbid PCOS as it improves insulin sensitivity and counters insulin resistance-induced ovarian androgen production.
What first-line pain management strategies would you recommend for a patient with HS?
Topical analgesics, oral acetaminophen, and oral nonsteroidal anti-inflammatory drugs are considered first-line therapy for pain in HS.
