Hidradenitis suppurativa

Hidradenitis suppurativa
Hidradenitis suppurativa (Hurley's Staging II) in the left armpit
Classification and external resources
Specialty Dermatology
ICD-10 L73.2
ICD-9-CM 705.83
DiseasesDB 5892
eMedicine emerg/259 med/2717 derm/892
Patient UK Hidradenitis suppurativa
MeSH D017497

Hidradenitis suppurativa (HS), also known as acne inversa, is a chronic skin disease characterized by clusters of abscesses or subcutaneous boil-like "infections" (often free of actual bacteria) that most commonly affects the underarms, under the breasts, inner thighs, groin, and buttocks.[1][2] Outbreaks are painful and may persist for years with interspersed periods of inflammation, often culminating in sudden drainage of pus. This process often forms open wounds that will not heal and frequently leads to significant scarring.

Persistent lesions may lead to the formation of sinus tracts, or tunnels connecting the abscesses or infections under the skin.[3] At this stage, complete healing is usually not possible, and progression is variable, with some experiencing remission for months to years at a time, while others may worsen and require multiple surgeries. Bacterial infections and cellulitis (deep tissue inflammation) are other common complications of HS. Depression and pain are often seen with HS and can be difficult to manage. HS often goes undiagnosed for years due to embarrassment causing delay in seeking treatment.

HS flares may be triggered by emotional/erotic stress, sweating, hormonal changes, heat and humidity, and the condition is exacerbated by friction from clothing. The disease is not contagious. There are indications that it is hereditary among certain ethnic groups and autoimmune in nature. Onset is most common in the late teens and early 20's. For unknown reasons, people with HS develop plugging of their apocrine glands.[4]

There is currently no known cure nor any consistently effective treatment. Incision and drainage procedures may provide symptomatic relief. Carbon dioxide laser surgery may be an effective treatment with a low chance of recurrence.[5] Lukewarm sitz baths can provide relief, gentle antiseptic skin cleansers and hydrogen peroxide assist in keeping affected areas free of bacteria. The number of people affected is not well established, but has been estimated as being between 1:24 (4.1%) and 1:600 (0.2%).

Causes

The cause of HS remains unknown and experts disagree over proposed causes.[6]

Lesions occur in any body areas with hair follicle although areas such as the axilla, groin, and perianal region are more commonly involved. This theory includes most of the following potentials indicators:[7]

The historical understanding of the disease suggests dysfunctional apocrine glands[11] or dysfunctional hair follicles,[12] possibly triggered by a blocked gland, create inflammation, pain, and a swollen lesion.

Triggering factors

There are several triggering factors that should be taken into consideration.

Predisposing factors

Stages

A case of hidradenitis suppurativa

HS presents itself in three stages.[22][11] Due to the large spectrum of clinical severity and the severe impact on quality of life, a reliable method for evaluating HS severity is needed.

Hurley's staging system

This is historically the first classification system proposed, and is still in use for the classification of patients with skin/dermatologic diseases (i.e., psoriasis, HS, acne). Hurley separated patients into three groups based largely on the presence and extent of cicatrization and sinuses. It has been used as a basis for clinical trials in the past and is a useful basis to approach therapy for patients. These three stages are based on Hurley's staging system, which is simple and relies on the subjective extent of the diseased tissue the patient has. Hurley's three stages of hidradenitis suppurativa are as follows:[23]

Stage Characteristics
I Solitary or multiple isolated abscess formation without scarring or sinus tracts. (A few minor sites with rare inflammation; may be mistaken for acne.)
II Recurrent abscesses, single or multiple widely separated lesions, with sinus tract formation. (Frequent inflammation restrict movement and may require minor surgery such as incision and drainage.)
III Diffuse or broad involvement across a regional area with multiple interconnected sinus tracts and abscesses. (Inflammation of sites to the size of golf balls, or sometimes baseballs; scarring develops, including subcutaneous tracts of infection – see fistula. Obviously, patients at this stage may be unable to function.)

Sartorius staging system

The Sartorius staging system is more sophisticated than Hurley's. Sartorius et al. suggested that the Hurley system is not sophisticated enough to assess treatment effects in clinical trials during research. This classification allows for better dynamic monitoring of the disease severity in individual patients. The elements of this staging system are the following:[24]

Points are accumulated in each of the above categories, and added to give both a regional and total score. In addition, the authors recommend adding a visual analog scale for pain or using the dermatology life quality index (DLQI, or the Skindex) when assessing HS.[25]

Treatments

Treatments may vary depending upon presentation and severity of the disease. Due to the poorly studied nature of this disease, the effectiveness of the drugs and therapies listed below is unclear.[26] Nearly a quarter of patients state that nothing relieves their symptoms.[27] Possible treatments include the following:

Lifestyle

Medication

Surgery

When the process becomes chronic, wide surgical excision is the procedure of choice. Wounds in the affected area do not heal by secondary intention, and immediate application of a split thickness skin graft is more appropriate.[7]

Laser hair removal

The 1064 nanometer wavelength laser for hair removal aids in the treatment of HS.[38] A randomized control study has shown improvement in HS lesions with the use of an Nd:YAG laser.[39]

Prognosis

In stage III disease, fistulae left undiscovered, undiagnosed, or untreated, can lead to the development of squamous cell carcinoma, a rare cancer, in the anus or other affected areas.[40][41] Other stage III chronic sequelae may also include anemia, multilocalized infections, amyloidosis, and arthropathy. Stage III complications have been known to lead to death, but clinical data is still uncertain.

Potential complications

History

A surgeon from Paris, Velpeau described an unusual inflammatory process with formation of superficial axillary, sub-mammary and perianal abscesses in 1839. One of his colleagues also located in Paris, named Verneuil, coined the term “hidrosadénite phlegmoneuse” approximately 15 years later. This name for the disease reflects the former pathogenetic model of acne inversa, which is considered inflammation of sweat glands as the primary cause of hidradenitis suppurativa. In 1922 Schiefferdecker suspected a pathogenicassociation between acne inversa and apocrine sweat glands. In 1956 Pillsbury postulated follicular occlusion as the cause of acne inversa, which they grouped together with acne conglobata and perifolliculitis capitis abscendens et suffodiens (dissecting cellulitis of the scalp) as the "acne triad". Plewig and Kligman added another element to their acne triad, pilonidal sinus. Plewig et al. noted that this new "acne tetrad" includes all the elements found in the original "acne triad", in addition to a fourth element, pilonidal sinus. In 1989, Plewig and Steger introduced the term "acne inversa", indicating a follicular source of the disease and replacing older terms such as "Verneuil disease".

Author Year Findings
Velpeau 1839 First description of the hidradenitis suppurativa
Verneuil 1854 "Hidrosadénite phlegmoneuse"
Pillsbury 1956 Acne triad (hidradenitis suppurativa, perifolliculitis capitis abscendens et suffodiens, acne congoblata)
Plewig & Kligman 1975 Acne tetrad (acne triad + pilonidal sinus)
Plewig & Steger 1989 Acne inversa

Other names

Hidradenitis suppurativa has been referred to by multiple names in the literature, as well as in various cultures. Some of these are also used to describe different diseases, or specific instances of this disease.[22]

Histology

Author Year Major Features
Plewig & Steger[53] 1989 Initial hyperkeratosis of the follicular infundibulum. Bacterial super-infection and follicle rupture. Granulomatous inflammatory reaction of the connective tissue. Apocrine and eccrine sweat glands secondarily involved.
Yu & Cook[58] 1990 Cysts and sinus tracts lined with epithelium, in part with hair shafts. Inflammation of apocrine sweat glands only if eccrine sweat glands and hair follicles are also inflamed.
Boer & Weltevreden[59] 1996 Primary inflammation of the follicular infundibulum. Apocrine sweat glands are secondarily involved.

References

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  3. "Mayo Clinic site". Retrieved 2013-12-07.
  4. Mayo Clinic Staff. "Causes". Mayo Clinic. Retrieved 19 September 2012.
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  8. Mayo Clinic Staff (2012). "Definition". Mayo Clinic. Retrieved 19 September 2012.
  9. Clinical trial number NCT00329823 for "Etanercept in Hidradenitis Suppurativa" at ClinicalTrials.gov
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