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Hidradenitis Suppurativa

Updated : August 24, 2023





Background

An inflammatory responses skin disorder known as hidradenitis suppurativa (HS), commonly known as acne inversus, causes lesions such as deep-seated abscesses and nodules, draining passages, & fibrotic lesions. The intertriginous and apocrine gland-rich regions are where these lesions most frequently develop.

The axillary, perineal, groin, perianal and inframammary regions are among the most typical. This disorder may have a detrimental psychosocial effect due to the pain, odor, sensitive areas, drainage, and scars that are connected with it. The interprofessional team’s role in the treatment of hidradenitis suppurativa is highlighted in this activity, which also discusses the condition’s presentation, cause, and consequences.

 

Epidemiology

Prevalence estimates range from 1% – 4%. Due to inadequate reporting & misdiagnosis, these figures need to be more accurate. The illness often first manifests between puberty & age 40, most commonly between the ages of 21 and 29. About three to one, women are more likely than men to be afflicted. Evidence of a pattern of ethnic or racial preference is lacking.

 

Anatomy

 

 

Pathophysiology

A damaged hair follicle that has been blocked and ruptured releasing its contents, including germs and keratin, into the nearby dermis, is the first stage of the pathologic process that leads to hidradenitis suppurativa. The pilosebaceous component and also other nearby structures may get infected as a result of a chemotactic inflammatory reaction by the neutrophils & lymphocytes in the area.

Other potential pathology-causing factors include aberrant antimicrobial peptides, inappropriate apocrine gland secretion, irregular epidermal invaginations that result in tract development, and insufficient sebaceous gland production. Additionally, immunological anomalies have been noted.

Increased amounts of inflammatory factors, such as tumor necrosis factor-alpha and different interleukins, have been found in the hidradenitis suppurativa lesions and may serve as potential targets for future therapies. It doesn’t seem like bacteria are the cause. A sterile culture is often produced while aspirating from a non-ruptured lesion. However, further worsening of hidradenitis suppurativa might result from pathogenic bacteria & colonization throughout the process.

 

Etiology

The genesis of hidradenitis suppurativa appears to be influenced by factors in the behavior and environment. The first-degree relation with hidradenitis suppurativa is reported by 33–40% of those who have the condition, which raises the possibility of an underlying genetic factor with an autosomal dominant propagation pattern. Researchers have discovered an abnormality of the gamma-secretase Notch signaling pathway in a small proportion of families affected.

Additionally, behavioral and environmental factors play a role. People with HS are much more likely to be overweight and obese. Obesity contributes to HS by increasing the intertriginous contact area & skin friction, increasing sweat production & retention, and altering hormones that result in relative hyperandrogenism. Since metabolic syndrome is more prevalent in obese people, it is also more common in HS.

Smoking is also widespread among people with HS. Although the exact cause is unknown, smoking may lead to more follicular plugging. The progression of the disease & severity is worse in smokers, similar to obesity. Hormones can be seen to have an impact on HS. More females than males are affected, and the age of predominant occurrence is often between puberty & menopause. Menstrual periods & exogenous hormonal substances also cause changes in the intensity of acute symptomatic events.

 

Genetics

 

 

Prognostic Factors

The prognosis varies. For this illness, there is no treatment. The prognosis deteriorates if early disease identification and therapy are delayed and if concomitant problems like obesity and smoking (if present) are not treated & improved.

 

Clinical History

Hidradenitis suppurativa typically affects people who are otherwise healthy, while it extremely infrequently might start before adolescence. The condition develops slowly, with erythema being the first symptom. Later, the lesions start to hurt.

Asymmetric pauciarticular arthritis, symmetric polyarthritis, polyarthralgia syndrome, and spondyloarthropathy are just a few of the possible clinical characteristics of hidradenitis suppurativa-related arthritis. Furthermore, epidemiological evidence points to a connection between metabolic syndrome and hidradenitis suppurativa.

 

Physical Examination

The average time it takes to make an accurate diagnosis is seven years because the early stages of hidradenitis suppurativa are frequently misdiagnosed as other illnesses. Recognition of the morphology (deep, inflammatory, painful lumps, sinus tubes, scars), location (intertriginous regions, apocrine gland-containing regions), & duration of the illness process is necessary for clinical diagnosis (extended course with remission & activity intervals).

Up to 48 hours prior to the onset of a lesion, up to 50% of people will experience a prodromal syndrome that includes blistering, stinging, discomfort, pruritus, warmth, and hyperhidrosis in the affected area. Menstrual cycle, excess weight, anxiety, hormone levels, extreme heat, & perspiration are a few triggers that might occur. Unless there is a latent infection or an advanced condition, people usually look healthy and are afebrile when they come.

Relevant past experiences include lesions that started during puberty and continued throughout young adulthood, as well as recurrent lesions that occasionally improved or disappeared. Chronic hidradenitis suppurativa is a disease. Establishing the correct diagnosis also benefits from knowing if there is a family history of a related ailment.

The Hurley staging method can be used to categorize the case after the history and thorough physical examination has been completed.

Hurley Stage I: Formation of an abscess without tracts or scarring

Hurley Stage II: Recurrent abscesses, scarring, and sinus tracts; lesions may be solitary or widely spaced

Hurley Stage III: Little to no uninvolved skin remains after diffuse involvement; numerous interconnected sinus systems and abscesses cover a large area.

The follicular occlusion tetrad, which also includes pilonidal sinus, dissecting cellulitis of the scalp, and acne conglobata, includes HS as one of its four components. When someone is given the diagnosis of having hidradenitis suppurativa, it is important to check for these other diagnoses. Additionally, when conducting a medical history & physical examination, clinicians should look for signs of spondyloarthropathy, metabolic disease, & bowel inflammation.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Differential Diagnoses

Granuloma Inguinale

Cat Scratch Disease

Blastomycosis

Erysipelas

Symptoms of Lymphogranuloma Venereum in the Skin

Dermatologic Aspects of Actinomycosis

Laboratory Studies

 

 

Imaging Studies

 

 

Procedures

 

 

Histologic Findings

 

 

Staging

 

 

Treatment Paradigm

The main objectives are to stop the progression of the disease, lessen the incidence of recurrence, & treat current lesions to reduce pain & drainage. Most therapy algorithms are based on consensus among experts because there isn’t much research that compares different treatment plans. Topical antibiotics are the first-line therapy for early, simple illnesses. The most successful treatment has been dermal clindamycin. Intralesional corticosteroids can lessen inflammatory reactions, and punch debridement of specific lesions can speed up the healing process.

Oral antibiotics are used as a therapy for Hurley Stage Two & resistant Hurley Stage one. The most successful antibiotics have been those in the tetracycline group. Combination therapy using oral clindamycin and rifampin is advised if treatment failure continues. Additionally, anti-androgenic hormone treatments, which include cyproterone acetate, spironolactone, finasteride, and oral contraceptives, can be beneficial. Retinoids used orally have varied responses.

Acitretin seems to work better in HS, where isotretinoin is most beneficial for acne. For some people, systemic steroids are efficacious. TNF-alpha blockers are suggested for Hurley stage III & lower resistant phases. The sole FDA-approved treatment for HS is adalimumab. At this point, surgery is frequently necessary, and it entails a broad excision that removes the tumors, tracts, & scars of the entire area affected. The best course of action is frequently a combination of medicinal treatment & surgical intervention. Pulsed light therapy & targeted laser therapy are two other treatment alternatives that work to reduce inflammation.

The management of pain is also essential. Both non-inflammatory and inflammatory pain are seen with HS. Scarring (which can result in tensile pain), abscesses, open ulcerations, keloids, sinus tracts, lymphedema, anal fissures, frictional discomfort, & arthritis are some possible sources of pain. Topical medications (like anti-inflammatories & lidocaine), acetaminophen, systemic nonsteroidal anti-inflammatories, atypical anticonvulsants like pregabalin or gabapentin, & serotonin-norepinephrine reuptake inhibitors may be helpful depending on the severity of the disease as well as the kind of pain.

If there is concurrent depression, duloxetine is extremely beneficial. The care of comorbidities that lead to the onset or progression of the disease process should be a part of all treatments, regardless of the disease’s stage. Counseling and assistance with losing weight & cessation of smoking are crucial parts of treatment for people who are overweight or who smoke because they have a more serious progression of the disease.

Avoiding skin damage is another component of treatment. Avoiding harsh chemicals or cleaning instruments (loofahs, brushes, washcloths), as well as wearing loose, synthetic clothing, can be helpful. To stop draining lesions from becoming more irritated, use soft coverings with transparent petroleum jelly and non-occlusive coverings. The psychosocial component of the condition is a crucial but frequently disregarded aspect of treatment.

Because of the discomfort, odor, drainage & sensitive afflicted areas that are also a part of this disorder, quality of life is reduced in those who have it. Patients may experience high sexual and relationship dysfunction, social isolation, and career issues due to lost days of work when flares happen. It can be comforting to know that this disease is not communicable or the result of inadequate hygiene. The addition of counseling and support groups to treatment strategies is frequently useful.

 

by Stage

 

 

by Modality

 

 

Chemotherapy

 

 

Radiation Therapy

 

 

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

 

adalimumab-aacf 


Initial dose: 160mg subcutaneously as four injections in 40mg each on day 1 or 80mg subcutaneously two weeks later
Maintenance dose:2 weeks later-40mg subcutaneously every two weeks



 
 

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK534867/

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Hidradenitis Suppurativa

Updated : August 24, 2023




An inflammatory responses skin disorder known as hidradenitis suppurativa (HS), commonly known as acne inversus, causes lesions such as deep-seated abscesses and nodules, draining passages, & fibrotic lesions. The intertriginous and apocrine gland-rich regions are where these lesions most frequently develop.

The axillary, perineal, groin, perianal and inframammary regions are among the most typical. This disorder may have a detrimental psychosocial effect due to the pain, odor, sensitive areas, drainage, and scars that are connected with it. The interprofessional team’s role in the treatment of hidradenitis suppurativa is highlighted in this activity, which also discusses the condition’s presentation, cause, and consequences.

 

Prevalence estimates range from 1% – 4%. Due to inadequate reporting & misdiagnosis, these figures need to be more accurate. The illness often first manifests between puberty & age 40, most commonly between the ages of 21 and 29. About three to one, women are more likely than men to be afflicted. Evidence of a pattern of ethnic or racial preference is lacking.

 

 

 

A damaged hair follicle that has been blocked and ruptured releasing its contents, including germs and keratin, into the nearby dermis, is the first stage of the pathologic process that leads to hidradenitis suppurativa. The pilosebaceous component and also other nearby structures may get infected as a result of a chemotactic inflammatory reaction by the neutrophils & lymphocytes in the area.

Other potential pathology-causing factors include aberrant antimicrobial peptides, inappropriate apocrine gland secretion, irregular epidermal invaginations that result in tract development, and insufficient sebaceous gland production. Additionally, immunological anomalies have been noted.

Increased amounts of inflammatory factors, such as tumor necrosis factor-alpha and different interleukins, have been found in the hidradenitis suppurativa lesions and may serve as potential targets for future therapies. It doesn’t seem like bacteria are the cause. A sterile culture is often produced while aspirating from a non-ruptured lesion. However, further worsening of hidradenitis suppurativa might result from pathogenic bacteria & colonization throughout the process.

 

The genesis of hidradenitis suppurativa appears to be influenced by factors in the behavior and environment. The first-degree relation with hidradenitis suppurativa is reported by 33–40% of those who have the condition, which raises the possibility of an underlying genetic factor with an autosomal dominant propagation pattern. Researchers have discovered an abnormality of the gamma-secretase Notch signaling pathway in a small proportion of families affected.

Additionally, behavioral and environmental factors play a role. People with HS are much more likely to be overweight and obese. Obesity contributes to HS by increasing the intertriginous contact area & skin friction, increasing sweat production & retention, and altering hormones that result in relative hyperandrogenism. Since metabolic syndrome is more prevalent in obese people, it is also more common in HS.

Smoking is also widespread among people with HS. Although the exact cause is unknown, smoking may lead to more follicular plugging. The progression of the disease & severity is worse in smokers, similar to obesity. Hormones can be seen to have an impact on HS. More females than males are affected, and the age of predominant occurrence is often between puberty & menopause. Menstrual periods & exogenous hormonal substances also cause changes in the intensity of acute symptomatic events.

 

 

 

The prognosis varies. For this illness, there is no treatment. The prognosis deteriorates if early disease identification and therapy are delayed and if concomitant problems like obesity and smoking (if present) are not treated & improved.

 

Hidradenitis suppurativa typically affects people who are otherwise healthy, while it extremely infrequently might start before adolescence. The condition develops slowly, with erythema being the first symptom. Later, the lesions start to hurt.

Asymmetric pauciarticular arthritis, symmetric polyarthritis, polyarthralgia syndrome, and spondyloarthropathy are just a few of the possible clinical characteristics of hidradenitis suppurativa-related arthritis. Furthermore, epidemiological evidence points to a connection between metabolic syndrome and hidradenitis suppurativa.

 

The average time it takes to make an accurate diagnosis is seven years because the early stages of hidradenitis suppurativa are frequently misdiagnosed as other illnesses. Recognition of the morphology (deep, inflammatory, painful lumps, sinus tubes, scars), location (intertriginous regions, apocrine gland-containing regions), & duration of the illness process is necessary for clinical diagnosis (extended course with remission & activity intervals).

Up to 48 hours prior to the onset of a lesion, up to 50% of people will experience a prodromal syndrome that includes blistering, stinging, discomfort, pruritus, warmth, and hyperhidrosis in the affected area. Menstrual cycle, excess weight, anxiety, hormone levels, extreme heat, & perspiration are a few triggers that might occur. Unless there is a latent infection or an advanced condition, people usually look healthy and are afebrile when they come.

Relevant past experiences include lesions that started during puberty and continued throughout young adulthood, as well as recurrent lesions that occasionally improved or disappeared. Chronic hidradenitis suppurativa is a disease. Establishing the correct diagnosis also benefits from knowing if there is a family history of a related ailment.

The Hurley staging method can be used to categorize the case after the history and thorough physical examination has been completed.

Hurley Stage I: Formation of an abscess without tracts or scarring

Hurley Stage II: Recurrent abscesses, scarring, and sinus tracts; lesions may be solitary or widely spaced

Hurley Stage III: Little to no uninvolved skin remains after diffuse involvement; numerous interconnected sinus systems and abscesses cover a large area.

The follicular occlusion tetrad, which also includes pilonidal sinus, dissecting cellulitis of the scalp, and acne conglobata, includes HS as one of its four components. When someone is given the diagnosis of having hidradenitis suppurativa, it is important to check for these other diagnoses. Additionally, when conducting a medical history & physical examination, clinicians should look for signs of spondyloarthropathy, metabolic disease, & bowel inflammation.

Differential Diagnoses

Granuloma Inguinale

Cat Scratch Disease

Blastomycosis

Erysipelas

Symptoms of Lymphogranuloma Venereum in the Skin

Dermatologic Aspects of Actinomycosis

 

 

 

 

 

 

 

 

 

 

The main objectives are to stop the progression of the disease, lessen the incidence of recurrence, & treat current lesions to reduce pain & drainage. Most therapy algorithms are based on consensus among experts because there isn’t much research that compares different treatment plans. Topical antibiotics are the first-line therapy for early, simple illnesses. The most successful treatment has been dermal clindamycin. Intralesional corticosteroids can lessen inflammatory reactions, and punch debridement of specific lesions can speed up the healing process.

Oral antibiotics are used as a therapy for Hurley Stage Two & resistant Hurley Stage one. The most successful antibiotics have been those in the tetracycline group. Combination therapy using oral clindamycin and rifampin is advised if treatment failure continues. Additionally, anti-androgenic hormone treatments, which include cyproterone acetate, spironolactone, finasteride, and oral contraceptives, can be beneficial. Retinoids used orally have varied responses.

Acitretin seems to work better in HS, where isotretinoin is most beneficial for acne. For some people, systemic steroids are efficacious. TNF-alpha blockers are suggested for Hurley stage III & lower resistant phases. The sole FDA-approved treatment for HS is adalimumab. At this point, surgery is frequently necessary, and it entails a broad excision that removes the tumors, tracts, & scars of the entire area affected. The best course of action is frequently a combination of medicinal treatment & surgical intervention. Pulsed light therapy & targeted laser therapy are two other treatment alternatives that work to reduce inflammation.

The management of pain is also essential. Both non-inflammatory and inflammatory pain are seen with HS. Scarring (which can result in tensile pain), abscesses, open ulcerations, keloids, sinus tracts, lymphedema, anal fissures, frictional discomfort, & arthritis are some possible sources of pain. Topical medications (like anti-inflammatories & lidocaine), acetaminophen, systemic nonsteroidal anti-inflammatories, atypical anticonvulsants like pregabalin or gabapentin, & serotonin-norepinephrine reuptake inhibitors may be helpful depending on the severity of the disease as well as the kind of pain.

If there is concurrent depression, duloxetine is extremely beneficial. The care of comorbidities that lead to the onset or progression of the disease process should be a part of all treatments, regardless of the disease’s stage. Counseling and assistance with losing weight & cessation of smoking are crucial parts of treatment for people who are overweight or who smoke because they have a more serious progression of the disease.

Avoiding skin damage is another component of treatment. Avoiding harsh chemicals or cleaning instruments (loofahs, brushes, washcloths), as well as wearing loose, synthetic clothing, can be helpful. To stop draining lesions from becoming more irritated, use soft coverings with transparent petroleum jelly and non-occlusive coverings. The psychosocial component of the condition is a crucial but frequently disregarded aspect of treatment.

Because of the discomfort, odor, drainage & sensitive afflicted areas that are also a part of this disorder, quality of life is reduced in those who have it. Patients may experience high sexual and relationship dysfunction, social isolation, and career issues due to lost days of work when flares happen. It can be comforting to know that this disease is not communicable or the result of inadequate hygiene. The addition of counseling and support groups to treatment strategies is frequently useful.

 

 

 

 

 

 

 

 

 

adalimumab-aacf 


Initial dose: 160mg subcutaneously as four injections in 40mg each on day 1 or 80mg subcutaneously two weeks later
Maintenance dose:2 weeks later-40mg subcutaneously every two weeks



https://www.ncbi.nlm.nih.gov/books/NBK534867/

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