​Kembara Xtra - Medicine - Hidradenitis Suppurativa

​Kembara Xtra - Medicine - Hidradenitis Suppurativa 
Recurrent inflammatory nodules, abscesses, sinus tracts, and complex scarring are all symptoms of a chronic inflammatory skin condition. 
The affected areas are sensitive, foul-smelling, and frequently have exudative discharge.
axillae, groin, perianal, perineal, and inframammary skin are frequently afflicted intertriginous skin regions. afflicted systems include skin and psychological.
Synonym(s): apocrinitis, Verneuil disease, hidradenitis axillaris, acne inversa 


Aspects of Geriatrics
:after menopause uncommon
Child Safety Considerations
:Rarely happens before puberty; when it does, it's linked to early adrenarche in kids.
Pregnant women's issues
:No tetracycline or Accutane (isotretinoin) therapy during pregnancy. A disease may get better during pregnancy and go worse after delivery.


Epidemiology 

 Female is more prevalent than male (3:1)
Teenagers: Females outnumber Males (3.8:1) 
American Indians
Incidence
Peak onset occurs in the second and third decades of life.
Prevalence 0.05–4.10%

Pathophysiology and Etiology 
Unknown; once assumed to be an apocrine gland condition, but more recently believed to be caused by a follicular epithelium deficiency. Deregulation of the neighborhood immune system might also be important.
Hair follicle inflammation brought on by follicular blockage in skin with apocrine glands
Mechanical stress on the skin (intertriginous regions) precipitates follicular rupture and immune response; hormonally induced ductal keratinocyte proliferation results in a failure of follicular epithelial shedding, causing follicular occlusion; bacterial involvement is a secondary event; rupture and re epithelialization cause sinus tracts to form.
Genetics: Familial occurrences (autosomal dominant) suggest a single gene transmission, however the disorder could possibly be polygenic.
An estimated 40% of patients have a family member who is suffering.


Risk Factors: Hyperandrogenism, smoking, obesity, and lithium may cause or aggravate this illness.

Prevention 

 If you are overweight or obese, lose weight.

 Quitting smoking
Steer clear of tight clothing, deodorants, shaving, depilation, heat exposure, frictional stress, and synthetic textiles.
Using antibacterial soaps

Accompanying Conditions 

acne vulgaris and acne conglobate; perifolliculitis capitis abscedens et suffodiens (dissecting cellulitis of the scalp); Pilonidal disease; metabolic syndrome/obesity; polycystic ovary syndrome (PCOS) and androgen dysfunction; thyroid disease; arthritis and spondyloarthritis (seronegative); inflammatory bowel disease (Crohn disease and ulcerative colitis

Presenting History Diagnostic standards adopted by the 2nd International Conference on Hidradenitis Suppurativa, 2009 Diagnosis requires the presence of all three standards (morphology, location, progression) - Common lesions include sore nodules, abscesses, sinuses that drain, bridging scars, and "tombstone" double-ended pseudocomedones in secondary lesions. - Typical topography: infra- and intermammary folds, axillae, groins, perineal and perianal region - Chronicity and recurrences, which are frequently resistant to first therapies

clinical assessment 
There are soft nodules with a dome form, ranging in size from 0.5 to 3.0 cm.
Location is consistent with the distribution of terminal hair follicles that are dependent on low androgen concentrations and breast tissue connected to apocrine.
- The following sites are listed in order of how frequently they are affected: axillary, inguinal, perianal and perineal, mammary and inframammary, buttock, pubic area, chest, scalp, retroauricular, and eyelid. – Large lesions might fluctuate often and have comedones.

 Potentially offensive discharge
Hurley clinical staging system: Stage I: nodule/abscess formation without sinus tracts or scarring; Stage II: multiple lesions widely dispersed; Stage III: widespread, multiple linked tracts and abscesses; and Stage III: scarring.
(Points Attributed) Sartorius Clinical Staging System Anatomical site implicated - Amount and type of lesions - Lesion spacing - Presence or absence of healthy skin in between lesions

Differential diagnosis: Acne vulgaris, conglobate; Furunculosis/carbuncles; Infected Bartholin or sebaceous cysts; Lymphadenopathy/lymphadenitis; Cutaneous Langerhans cell histiocytosis; Actinomycosis; Granuloma inguinale; Lymphogranuloma venereum; Apocrine nevus; Crohn disease with an

Laboratory Results 

Initial examinations (lab, imaging)
Skin cultures or boil aspirates are typically negative. When cultures are positive, they frequently contain a variety of microorganisms, including Staphylococcus aureus and Staphylococcus epidermidis.
Lesion biopsy is typically not necessary, however it is helpful to rule out other conditions such squamous cell carcinoma.
May detect leukocytosis, decreased serum iron, normocytic anemia, elevated erythrocyte sedimentation rate (ESR), alterations in the serum electrophoresis pattern, or any of these things.

Tests in the Future & Special Considerations
Due to the increased risk of squamous cell carcinoma, take into account biopsy for lesions that are worrying.
Consider assessing the following if the patient is female, overweight, and/or hirsute: - Sex hormone-binding globulin - Dehydroepiandrosterone sulfate - Total and free testosterone - Progesterone

Other/Diagnostic Procedures
Biopsy, culture, and incision and drainage
When planning an excision to determine the complete extent of sinus tracts, ultrasound may be helpful.
Interpretation of Tests
Inflammatory cells, large cells, sinus tracts, subcutaneous abscesses, and severe fibrosis are seen in the dermis. Hair follicles are also dilated and blocked by keratinized stratified squamous epithelium. 

Management 

Even though this condition is common, the majority of trials have been small and underpowered. As a result, the quality of the evidence is typically low (6)[A]. Therapy objectives: Reduce disease severity, stop the development of new lesions, get rid of chronic illness, and stop the growth of scars.
Warm compresses, sitz baths, topical antiseptics for inflammatory lesions, and non-opioid analgesics are all examples of conservative treatment.
Smoking cessation and weight loss lead to significant improvements.

 Zinc gluconate, corticosteroids, and isotretinoin
In phases I and II, try getting medical help.
Prior to pursuing surgical treatments in stage III, a brief medical trial may be appropriate. Adalimumab is the only treatment for this illness that has FDA approval. There may be other biologics that work. Relapse is almost unavoidable and no treatment can prevent it, but the illness can be managed. Before beginning biologics, other treatments must typically fail. They might be an expensive choice.

General Actions 
Proper hygiene, which includes avoiding skin-shearing stress (light clothing), frequent washing with antibacterial soap, and education and psychosocial support
Eat less dairy and foods with high glycemic loading.
Improve environmental variables that lead to follicular obstruction. Stop smoking. Lose weight. Treat symptoms of acute lesions.


First Line of Medicine
Consider systemic or topical antibiotics for Stage I illness.
- Antibiotics for topical use (clindamycin was investigated in clinical studies).
 Benzoyl peroxide 5-10% solution or clindamycin 0.1% solution BID for 12 weeks 4% solution of chlorhexidine
- Systemic antibiotics (first 7–10 days of treatment)
Augmentin 875 mg every 8 to 12 hours; Tetracycline 500 mg BID; Doxycycline 100 mg q12h; Clindamycin 300 mg BID (7)[B]
Intralesion corticosteroids may reduce pain, erythema, edema, and lesion size, according to limited data (triamcinolone acetonide 10 mg/mL injection, typically 0.2 to 2.0 mL).
Stages II and III disease: Use broad-spectrum coverage to treat the underlying bacterial illness. Consider the location and characteristics of the disease when choosing an antibiotic; the best research supports the use of clindamycin and rifampicin or ertapenem followed by a six-month course of rifampicin, moxifloxacin, and metronidazole.- Minor surgical treatments to treat specific lesions or sinus tracts (punch débridement, local unroofing). Other techniques (infrequently used)
- Hormonal therapy: finasteride (5 mg daily), estrogen/norgestrel oral contraceptives, and cyproterone acetate (which may not be available in the United States).
Next Line
Spironolactone: significantly lower quantity of lesions and pain; Dapsone 50–150 mg daily; Metformin: significantly lower decline in Sartorius score; 
Isotretinoin, an oral retinoid, has a poor therapeutic effect.
TNF-inhibitors: Adalimumab 40 mg once a week (a high dose) statistically differs from placebo in the treatment of patients, but the clinical effect size is minor (Cochrane), and the long-term safety is unknown.
- Infliximab: Most patients in the therapy group experienced a 50% or higher decline in disease, leading to an improvement in quality of life.
- No difference between etanercept and a placebo

Problems to Refer 
A referral for surgical excision or radiation/laser treatment (stage II) is justified in the event that the disease has not responded to treatment, is in stages II or III, or if there is worry about malignancy (squamous cell carcinoma).
If there is considerable psychosocial stress brought on by the illness, get help with stress management or psychiatric assessment.
Hyperandrogenic conditions, such as PCOS, should be investigated or referred for further evaluation.
Referral to a plastic surgeon or reconstructive urologist may be required in the case of severe perianal/perivulvar disease or other very widespread disease.

Surgery is a crucial component of treatment and is required to completely eliminate scarring and tunnels.
Different surgical methods have been employed for stages II and III disease; they could be used in conjunction with antibiotics or as a last resort.
When an abscess is present, incision and drainage are required to address acute flare-ups. Deroofing sinus tracts and skin tissue-sparing excision with electrosurgical peeling (STEEP) enable secondary intended healing.
While still frequent, recurrences are typically less severe than the initial lesions.
- If all sinus tracts are removed with a clear 1- to 2-cm margin, wide full-thickness excision with healing by granulation or flap insertion is the most effective treatment and seldom causes local recurrence. Rates of local recurrence (within 3 to 72 months): submammary (50%) and inguinoperineal (37%), axillary (3%), and perianal (0%).
Hurley stages I and II disease can be treated with neodymium-doped yttrium aluminum garnet (Nd:YAG) laser monthly sessions for three to four months, however there is no agreement on their effectiveness. - CO2 laser ablation with secondary goal of mending
Although they have had varying degrees of success, cryotherapy and photodynamic therapy are not typically advised.
Potential role for combination therapy in advanced patients, including radical resection and biologics

Take Action 
Follow up at least once a month to assess development and offer symptom management support.

Avoid dairy and foods with high glycemic loading to maintain a healthy diet that helps you lose weight.
Supplemental zinc may be beneficial.

The severity of the condition might range from having just two or three papules each year to having vast leaking sinus tracts.
Surgery "cures" performed locally had no impact on recurrence at distant sites.
Smoking cessation and weight loss can dramatically reduce symptoms.

Individual lesions heal gradually in 10 to 30 days, with recurrences perhaps lasting for years.

In cases of severe illness, sinus tracts and relentlessly increasing scarring are likely.
The best chance for recovery is with radical wide-area excision, which involves removing all hair-bearing skin in the afflicted area.

Complications 
● Contracture and stricturing of the skin after extensive
abscess rupture, scarring, and healing; or at sites of
surgical excision
● Lymphatic obstruction, lymphedema
● Psychosocial: anxiety, malaise, depression, self-injury
●Anemia, amyloidosis, and hypoproteinemia (due to
chronic suppuration)
● Lumbosacral epidural abscess,
sacral bacterial osteomyelitis
● Squamous cell carcinoma
may develop in indolent sinus tracts.
● Disseminated infection or septicemia (rare)
● Urethral, rectal, or bladder fistula (rare)

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