Hidradenitis Suppurativa
Disease Details
Family Health Simplified
- Description
- Hidradenitis suppurativa is a chronic skin condition characterized by painful, swollen lumps under the skin, typically in areas where skin rubs together, such as the armpits, groin, and under the breasts.
- Type
- Hidradenitis suppurativa is a chronic inflammatory skin condition. The type of genetic transmission is believed to be autosomal dominant with incomplete penetrance, meaning that a single copy of the altered gene may be sufficient to increase the risk of developing the disorder, but not everyone who inherits the mutation will necessarily exhibit symptoms.
- Signs And Symptoms
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Hidradenitis suppurativa is a chronic skin condition characterized by painful, inflamed nodules and abscesses, typically in areas with skin folds such as the armpits, groin, buttocks, and under the breasts. Symptoms include:
1. Blackheads: Small pitted areas of skin containing blackheads, often in pairs.
2. Painful lumps: Red, tender bumps that can grow larger, become inflamed, and can rupture to leak pus.
3. Tunnels: Over time, the formation of tracts or tunnels connecting lumps under the skin.
4. Scarring: As the lesions heal, they can leave thick scars or pitted skin.
Hidradenitis suppurativa can have a significant impact on quality of life due to pain, scarring, and potential complications such as infection or restricted movement. - Prognosis
- In stage III disease, as classified by the Hurley's staging system, fistulae left undiscovered, undiagnosed, or untreated, can rarely lead to the development of squamous cell carcinoma in the anus or other affected areas. Other stage III chronic sequelae may also include anemia, multilocalized infections, amyloidosis, and arthropathy. Stage III complications have been known to lead to sepsis, but clinical data are still uncertain.
- Onset
- Hidradenitis suppurativa typically has an onset after puberty, most commonly in the late teens and early twenties.
- Prevalence
- The prevalence of hidradenitis suppurativa varies, but it is estimated to affect approximately 1-4% of the population. This skin condition is more common among women and tends to present after puberty, with peak incidence between the ages of 20 and 40.
- Epidemiology
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Hidradenitis suppurativa is a chronic inflammatory skin condition characterized by painful nodules, abscesses, and sinus tracts primarily affecting intertriginous areas. Its epidemiology includes:
- **Prevalence:** Ranges from 1% to 4% globally.
- **Age of Onset:** Typically manifests after puberty, most commonly between ages 20 and 29.
- **Gender:** More prevalent in females, with a female-to-male ratio of approximately 3:1.
- **Risk Factors:** Higher incidence in individuals with a family history of the condition, obesity, metabolic syndrome, and smoking.
- **Geographic Variability:** Prevalence may vary by region, likely influenced by genetic and environmental factors.
No information is available for the term “nan” in the context of hidradenitis suppurativa. - Intractability
- Hidradenitis suppurativa (HS) is considered challenging to manage and can often be intractable. While some patients may experience relief with treatment, the chronic and recurrent nature of HS means that many individuals continue to suffer from symptoms despite various interventions. Treatment strategies typically aim to manage symptoms, reduce flare-ups, and improve quality of life, but a definitive cure remains elusive in many cases.
- Disease Severity
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Hidradenitis suppurativa is a chronic inflammatory skin condition characterized by painful nodules, abscesses, and sinus tracts, commonly occurring in areas where skin rubs together, such as the armpits and groin.
**Disease Severity:**
Severity varies among individuals and is generally classified into three stages using the Hurley staging system:
1. **Hurley Stage I**: Single or multiple abscesses without sinus tracts or scarring.
2. **Hurley Stage II**: Recurrent abscesses with sinus tracts and scarring, separated by normal skin.
3. **Hurley Stage III**: Diffuse or near-diffuse involvement, with multiple interconnected sinus tracts and abscesses across an entire area.
**Nan**: This appears to be a typographical error. If you meant to inquire about additional specific aspects of the condition, please clarify. - Healthcare Professionals
- Disease Ontology ID - DOID:2280
- Pathophysiology
- Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition characterized by the occlusion and subsequent inflammation of hair follicles. It primarily affects areas of the body where skin rubs together, such as the armpits, groin, buttocks, and under the breasts. The exact pathogenesis is not completely understood, but it is believed to involve a combination of genetic, environmental, and immunological factors. The process begins with follicular occlusion, followed by follicular rupture and an immune response that leads to chronic inflammation, abscess formation, and the eventual development of sinus tracts and scarring.
- Carrier Status
- Hidradenitis suppurativa is not typically associated with carrier status as seen in genetic disorders. It is a chronic skin condition characterized by the inflammation of hair follicles, leading to painful lumps, abscesses, and scarring, primarily in areas with sweat glands. The exact cause is not fully understood, but it is believed to involve a combination of genetic, hormonal, and environmental factors.
- Mechanism
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Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition characterized by painful, recurrent nodules, abscesses, and sinus tracts, typically in apocrine gland-bearing areas such as the armpits, groin, and under the breasts. The precise mechanisms underlying HS are not fully understood, but several key factors have been identified.
**Mechanism:**
HS is believed to result from follicular occlusion followed by rupture of the hair follicle, leading to an inflammatory response. This inflammation involves the apocrine glands and surrounding tissues, eventually causing chronic lesions and scar formation.
**Molecular Mechanisms:**
1. **Genetics:**
- Mutations in the gamma-secretase complex genes (such as NCSTN, PSEN1, and PSENEN) have been linked to familial forms of HS. These genes are involved in the Notch signaling pathway, which plays a crucial role in skin homeostasis and immune response.
2. **Immune Dysregulation:**
- Increased levels of pro-inflammatory cytokines such as TNF-α, IL-1β, and IL-17A have been observed in HS lesions. These cytokines contribute to the chronic inflammatory environment.
- Altered function of both innate and adaptive immune cells, including neutrophils, macrophages, and T-cells, is noted in affected skin tissues.
3. **Microbiome Changes:**
- Dysbiosis or imbalanced microbial community on the skin in HS patients can contribute to inflammation and infection. Bacterial colonization by species such as *Staphylococcus aureus* or *Streptococcus* can exacerbate the condition.
4. **Keratinocyte Dysfunction:**
- Abnormal keratinocyte proliferation and differentiation can contribute to follicular occlusion and the development of HS lesions.
5. **Aberrant Wound Healing:**
- Impaired wound healing mechanisms can lead to the formation of sinus tracts and chronic, non-healing wounds in HS patients.
Understanding these molecular mechanisms provides insight into potential therapeutic targets for HS, such as inhibitors of TNF-α, IL-17, or other inflammatory pathways. - Treatment
- Treatment depends upon presentation and severity of the disease. Due to the poorly studied nature of the disease, the effectiveness of drugs and therapies were unclear. Clear and sensitive communication from health care professionals, social and psychological interventions can help managing the emotional impact of the condition and aid necessary lifestyle changes. In May 2023, the European Commission (EC) approved Cosentyx (secukinumab) for active moderate to severe hidradenitis suppurativa in adults.Other possible treatments include the following:
- Compassionate Use Treatment
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Hidradenitis suppurativa (HS) can be challenging to treat, and in certain cases, patients may seek compassionate use treatments or off-label/experimental therapies. Here are some potential options:
1. **Biologics:**
- *Adalimumab (Humira)*: Approved for HS, but used in cases of severe, refractory HS.
- *Infliximab (Remicade)*: Not FDA-approved for HS but has shown efficacy in some studies.
2. **Immunomodulatory Agents:**
- *Anakinra (Kineret)*: An IL-1 receptor antagonist; used off-label with some positive outcomes.
- *Ustekinumab (Stelara)*: Targets IL-12 and IL-23; showed promise in off-label use.
3. **Hormonal Treatments:**
- *Spironolactone*: Used off-label to manage hormonal influences on HS.
4. **Antibiotics:**
- *Clindamycin and Rifampin Combination*: Often used off-label for long-term management.
5. **Retinoids:**
- *Acitretin*: A systemic retinoid; off-label use has shown benefits in some patients.
6. **Surgery and Laser Therapy:**
- Various surgical interventions, including wide excision and laser hair removal, can be used, although they are not always classified as "experimental."
These treatments should be considered on an individual basis, typically managed by dermatologists or specialists experienced in treating HS. - Lifestyle Recommendations
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Lifestyle recommendations for managing hidradenitis suppurativa include:
1. **Maintain a Healthy Weight**: Excess weight can exacerbate symptoms. Achieving and maintaining a healthy weight may help reduce flare-ups.
2. **Avoid Tight Clothing**: Wear loose-fitting clothes and avoid clothing that causes friction, which can irritate your skin.
3. **Practice Good Hygiene**: Gently wash affected areas with a mild, non-irritating soap and water.
4. **Avoid Shaving**: Consider alternate hair removal methods to prevent irritation.
5. **Stay Cool and Dry**: Excessive sweating can worsen symptoms. Use antiperspirants and avoid overly warm environments when possible.
6. **Diet Adjustments**: Some patients find that certain foods trigger flare-ups. Keeping a food diary can help identify and avoid these triggers.
7. **Quit Smoking**: Smoking has been linked to worsening of symptoms, so quitting can provide some benefit.
8. **Stress Management**: Stress can trigger or exacerbate flare-ups. Techniques like yoga, meditation, or counseling might be beneficial.
Consult with healthcare professionals for personalized advice and treatment options. - Medication
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Antibiotics: taken by mouth, these are used for their anti-inflammatory properties rather than to treat infection. Most effective is a combination of rifampicin and clindamycin given concurrently for 2–3 months. Popular antibiotics also include tetracycline and minocycline. Topical clindamycin has been shown to have an effect in double-blind placebo controlled studies. In a retrospective review and telephone survey, intravenous ertapenem therapy showed clinical improvement with 80.3% of subjects reporting medium to high satisfaction and 90.8% would recommend ertapenem to other patients.
Corticosteroid injections, also known as intralesional steroids, can be particularly useful for localized disease, if the drug can be prevented from escaping via the sinuses.
Antiandrogen therapy, hormonal therapy with antiandrogenic medications such as spironolactone, flutamide, cyproterone acetate, ethinylestradiol, finasteride, dutasteride, and metformin, have been found to be effective in clinical studies. However, the quality of available evidence is low and does not presently allow for robust evidence-based recommendations.
Intravenous infusion or subcutaneous injection of anti-inflammatory (TNF inhibitors; anti-TNF-alpha) drugs such as infliximab, and etanercept This use of these drugs is not currently Food and Drug Administration (FDA) approved and is somewhat controversial, so may not be covered by insurance.
TNF inhibitor: Studies have supported that various TNF inhibitors have a positive effect on HS lesions. Specifically adalimumab at weekly intervals is useful. Adalimumab is the only medication approved by the FDA for the treatment of HS as of 2021.
Topical isotretinoin is usually ineffective in people with HS, and is more commonly known as a medication for the treatment of acne vulgaris. Individuals affected by HS who responded to isotretinoin treatment tended to have milder cases of the condition.
Zinc and Nicotinamide, at doses of 90 mg and 30 mg respectively, have shown efficacy against mild to moderate hidradenitis suppurativa in a controlled retrospective clinical trial. - Repurposable Drugs
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Repurposable drugs for hidradenitis suppurativa include:
1. **Metformin**: Originally used for type 2 diabetes, it has shown anti-inflammatory effects.
2. **Clindamycin and Rifampin**: Initially antibiotics for bacterial infections, they are used in combination for their anti-inflammatory properties.
3. **Adalimumab**: A TNF-alpha inhibitor used for rheumatoid arthritis, shown effective for severe cases of hidradenitis suppurativa.
4. **Spironolactone**: Commonly used for acne and hypertension, it has anti-androgen properties that may benefit patients.
These drugs are explored for their secondary effects on reducing symptoms and managing hidradenitis suppurativa. - Metabolites
- Hidradenitis suppurativa is a chronic inflammatory skin condition that involves painful lumps under the skin. Research into the metabolite profile of patients with this condition is ongoing, but some studies suggest alterations in metabolites related to lipid metabolism, oxidative stress, and inflammation. Specific metabolites that have been noted in relation to hidradenitis suppurativa include increased levels of pro-inflammatory cytokines and markers of oxidative stress. However, more research is needed to fully understand the metabolic changes in this disease.
- Nutraceuticals
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Nutraceuticals for hidradenitis suppurativa have limited scientific evidence supporting their effectiveness. Some patients may benefit from anti-inflammatory supplements such as:
1. **Turmeric (Curcumin)**: Known for its anti-inflammatory properties.
2. **Zinc**: May help with wound healing and reducing inflammation.
3. **Omega-3 Fatty Acids**: Found in fish oil, these can help reduce inflammation.
Consult with a healthcare provider before starting any new supplement regimen. - Peptides
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Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition characterized by painful nodules, abscesses, and sinus tracts. Peptides are short chains of amino acids that can have various biological activities, including anti-inflammatory and antimicrobial effects. Research is ongoing into the potential use of peptides for HS treatment, aiming to reduce inflammation and prevent bacterial infections.
"NaN" typically stands for "not a number," which does not relate directly to hidradenitis suppurativa or its treatments. If you need information about nanotechnology or nanoparticle-based treatments for HS, please clarify.