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Thread: Hydradenitis Suppurativa

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    Question Hydradenitis Suppurativa

    Vt Is This? Its Inflammation Of Apocrine Sweat Glands. Post In Detail Plz

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    Hidradenitis Suppurativa


    Age:

    Hidradenitis suppurative usually begins in the postpubertal age group, when the apocrine glands start developing. It is most common in the third decade of life, but the untreated disease may persist into the seventh decade.

    Sex:

    The condition is more common in women than in men. Submammary, axillary, and inguinal involvement is more common in females, whereas the perineal form is more common in men.

    Site:

    Hidradenitis suppurative is found in the following areas:

    • Skin-bearing apocrine glands
    • Axilla
    • Groin
    • Perineum
    • Perianal region
    • Buttocks
    • Scrotum
    • Submammary region

    Etiology

    The exact cause of hidradenitis suppurativa has not been determined, although the following theories have been proposed:

    • Folliculitis is observed in all patients with hidradenitis suppurativa; whether this is coincidental or causative has not been established.
    • Local frictional trauma has been proposed to be one of the causative factors.



    Infective etiology

    • Streptococci, staphylococci, and Escherichia coli have been identified in the early stages of the disease; however, in the chronic relapsing stages, anaerobic bacteria and Proteus species have more commonly been isolated.
    • Whether the bacteria are the cause or the result of the disease has not been determined.



    • Diabetes, impaired glucose intolerance, and obesity were observed in some patients with hidradenitis suppurativa. Studies have shown that these entities are only incidental findings and not causative.
    • Hormonal theory: Improvement and relapse after pregnancy and contraceptive pill intake suggest that low levels of estrogens cause a predisposition for hidradenitis suppurativa.
    • Immune theory: Immunity in most patients is intact, but some patients demonstrate a defect in the T-cell lymphocytes.
    • Genetic theory: Increased incidence in individuals with HLA-A1 and HLA-B8 has been demonstrated in some patients.
    • Association tetrads: Hidradenitis suppurativa is part of the tetrad of acne conglobata (cystic acne), pilonidal sinus, and perifollicular capitis.
    • Cigarette smoking and lithium therapy have been identified as triggering factors for the disease.


    Pathophysiology




    Clinical

    The onset of disease is usually after puberty, in the second and third decades, when the apocrine sweat glands start developing. It begins in the epithelium containing apocrine and is more common in the neck, the axilla, and the submammary region in females and in the perineal skin in males. The perineal disease tends to be more severe.

    The early lesions are solitary, painful pruritic nodules that may persist for weeks or months without any change. If subcutaneous extension occurs, it may appear as indurated plaques, which, in lax skin, such as the axilla and the groin, manifest as linear bands. Multiple sites may be simultaneously affected.

    The nodules develop into pustules and eventually rupture externally, draining purulent material. Healing occurs with dense fibrosis, and recurrences crop up in and around the original site. This leads to chronic sinus formation, with intermittent release of serous, purulent, or bloodstained discharge. Ulceration sometimes occurs, and the abscesses may burrow and rupture into the neighboring structures. Episodes of acute cellulitis are sometimes a feature and are accompanied by fever and toxicity.

    Regional lymphadenopathy is characteristically absent. Chronic axillary hidradenitis suppurativa usually causes a reduction of the normal axillary odor.

    Severity and course of the disease are variable, but untreated hidradenitis suppurativa is typically a relentless progressive disease with acute exacerbations and remissions that lead to sinus tract formation and marked scarring.

    Differential diagnoses include the following conditions:

    • Infected cystic acne
    • Lymphogranuloma venereum
    • Developmental fistulae
    • Crohn disease
    • Furunculosis
    • Scrofuloderma
    • Actinomyces


    Comedones should be scrutinized; the nonspecific histologic changes may help in diagnosis.

    Lab Studies

    Blood tests

    • A complete blood cell count identifies the underlying anemia associated with the chronic disease.
    • Blood sugar tests identify associated diabetes.
    • Cell-mediated and immune response studies and T- and B-cell assays usually return normal findings.
    • Routine endocrine assays identify the hormonal imbalances.
    • Routine biochemistry for preoperative workup is essential.

    Microbiology

    I
    • n the acute stages, the isolated organisms include the coagulase negative staphylococci, E coli, Streptococcus milleri, and the anaerobic Bacteroides species. Proteus species have been isolated from patients with chronic conditions.
    • Staphylococci have been shown to be the transient bacteria in the acute initial stages, whereas S milleri is the predominant organism in the chronic stages.


    Staging

    The disease can be divided into the following 3 clinical stages:

    • Stage 1: Single or multiple abscesses form, without sinus tracts and cicatrization.
    • Stage 2: Recurrent abscesses form, with tract formation and cicatrization. There may be single or multiple widely separated lesions.
    • Stage 3: Diffuse or near-diffuse involvement or multiple interconnected tracts and abscesses are observed across the entire area.




    Medical therapy


    Treatment depends upon the stage of the disease. Early lesions are usually treated by medical therapy, whereas the patient with long-standing indolent disease requires surgical therapy.

    Nonspecific treatment measures include good hygiene, weight reduction, use of antiseptic detergents, and avoidance of tight-fitting clothes.

    Acute-stage treatment options

    • Antibiotics: A short course of antibiotics for a period of 2 weeks is usually advisable. The antibiotics used include a combination of erythromycin and metronidazole, minocycline, or clindamycin. Cephalosporins and penicillins can also be used.
    • Intralesional steroids: Intralesional injection of steroids (eg, triamcinolone 5-10 mg diluted with water) causes the early lesions to involute within 12-24 hours.



    Chronic relapsing–stage treatment options

    Long-term antibiotics: Long-term administration of erythromycin and tetracycline has been used to treat the chronic stages and is shown to reduce the relapse rate. However, the efficacy of the antibiotics may be lost after long-term use. Efficacy can usually be regained by stopping the drug for a month and restarting it.
    High-dose systemic steroids (eg, prednisolone 60 mg/d) are useful as adjuvants to antibiotics, and they act by reducing the inflammatory process.
    Estrogens: Contraceptive pills (eg, 50 mcg ethynyl estradiol) and the combination of estrogens with 100 mg of cyproterone acetate have been used.
    Retinoids: These have been shown to be effective in the chronic disease. Isotretinoin, at a dose of 1 mg/kg/d, is administered for 4 months. Etretinate, at a dose of 0.5 mg/kg/d for a period of 6 months, is used for patients whose conditions are unresponsive to isotretinoin. Retinoids are teratogenic, and pregnancy is prevented by the use of contraception. Approximately 40% of patients show good response to retinoids.


    Other therapeutic agents that have been used with limited success include cisplatin, methotrexate, 5-alpha reductase inhibitors, and TNF-alpha inhibitors. Infliximab has been used in patients with Crohn disease and associated hidradenitis suppurativa.

    Surgical therapy

    Stage 1 options include incision and drainage, followed by antibiotics.

    Stage 2 and some stage 3 options include minor procedures.

    • Exteriorization and laying open of tracts and electrocoagulation
    • Excision and primary closure (eg, the Pollock procedure)


    Stage 3 options include total wide excision and healing with secondary intention or flaps and grafts.

    Closure of defects is achieved by the following:

    Z-plasty

    Skin grafts

    • Thiersch split-thickness grafts
    • Meshed grafts
    • Wolfe full-thickness grafts


    Flaps

    • Rotation flaps
    • Free flaps


    Biosynthetics agents, like Biobrane and Integra, have also been used.

    Preoperative details

    • Be aware of the possibility of associated systemic abnormalities.
    • Perform routine preoperative assessment for surgery and anesthesia, including cardiac, respiratory, and renal assessments.
    • Order antibiotics to treat the acute exacerbations before surgery.
    • Order prophylactic antibiotics before surgery.
    • Warn patients of the likelihood of a large raw area, which will require prolonged postoperative dressings.


    Intraoperative details

    • Perform a wide excision, with a 1.5-cm margin all around the lesion.
    • Perform intraoperative mapping of the sinus tracts with methyl violet, which reduces recurrence rates.
    • In the genital and perianal area, primary closure is to be avoided, and healing by secondary intention is advocated.
    • Axilla: Total excision with transverse primary closure (ie, the Pollock procedure) is usually possible.



    Postoperative care

    • Regular and prolonged use of postoperative dressings is necessary to aid secondary healing.
    • Healing can be hastened by the use of silastic foam dressings.
    • The use of Betadine and chlorhexidine dressings and compression has been shown to be of benefit.



    Complications of long-standing untreated disease include the following:

    • Fistula formation into the urethra, bladder, rectum, or peritoneum has been reported.
    • Sequelae of chronic infection, such as secondary anemia, hypoproteinemia, amyloidosis, and renal disease, can occur in chronic disease.
    • Peripheral and axial arthropathy is possible.
    • Chronic malaise and depression are observed.
    • Scarring of the tissue can lead to lymphatic obstruction and lymphedema of the limbs and arthropathy.
    • Marjolin ulcer (squamous cell carcinoma) has been reported in long-standing chronic disease.
    • Scrotal elephantiasis has been reported.

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    thats really nice. it was asked in my final exam.

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