Hidradenitis suppurativa ( acne inversa ) is an inflammatory skin disease with a progressive-chronic course, which affects women more often than men. The peak age for hidradenitis suppurativa is between the ages of 20 and 30.
What is hidradenitis suppurativa?
Hidradenitis suppurativa is a chronic inflammatory disease of the sebaceous glands and the terminal hair follicles connected to them, which is associated with painful nodule and abscess formation.
The skin changes usually develop under the armpits, the female breast, in the groin area and in the perianal and perigenital areas. The disease initially manifests itself with giant comedones (large blackheads ) and solitary nodules that can be felt, which later progress into larger collections of pus with deeper fistula tracts and pronounced nodules that converge into strands.
The disease can cause serious somatic and psychological disorders as a result of its pain symptoms and the foul-smelling discharge of secretions. Hidradenitis suppurativa is also associated with an increased risk of skin, oral mucosa and liver carcinomas.
root cause
The exact etiology of hidradenitis suppurativa has not yet been clearly clarified. It is known that due to a form of ichthyosis (keratinization disorder), horny material is increasingly accumulated in the sebaceous glands and hair roots.
In addition, there is a bacterial infection (usually with Staphylococcus aureus ) and purulent inflammation of the sebaceous gland in the affected areas. Due to the accumulation of pus, the sebaceous gland cyst ruptures, so that the inflammation can spread to the adjacent tissue and secondary sweat gland inflammation can be caused. Painful abscesses develop and fistulas develop as the disease progresses.
Bacterial colonization of the blood vessels in the subcutaneous tissue can also cause life-threatening sepsis (blood poisoning). Nicotine consumption (about 80 percent of those affected are smokers), obesity, a weakened immune system due to diabetes mellitus, increased sebum production due to male sex hormones and stress are considered to be triggering or promoting factors.
A genetic predisposition to hidradenitis suppurativa is also discussed due to familial frequency.
Symptoms, Ailments & Signs
Hidradenitis suppurativa is primarily manifested by inflamed sebaceous glands, which are usually filled with pus or wound fluid. More ulcers, abscesses or fistulas usually form in the affected area. These increase in size as the disease progresses and often also fill with pus.
When touched, there is severe pain. The skin changes significantly limit the mobility of the affected body region. In most cases, even slight friction causes intense pain or even the abscesses rupture. The pain often occurs at night and at rest and causes the affected person to have trouble sleeping. Wound healing disorders also occur.
As a result, further infections and inflammations develop in the affected skin areas, and the general condition of the person concerned progressively deteriorates. Many patients develop psychological problems as a result of the skin changes, such as depressive moods or inferiority complexes. The affected person usually appears exhausted and tired.
Active participation in life no longer takes place. In most cases, the disease leads to a withdrawal from social life. The symptoms persist for several days to weeks and subside quickly with appropriate treatment.
Diagnosis & History
Hidradenitis suppurativa is diagnosed based on the clinical symptoms. The diagnosis is confirmed by a biopsy followed by histological examination, which also serves to differentiate between similar clinical pictures such as Crohn’s disease with skin involvement or tuberculosis cutis colliquativa.
In addition, other infection-related diseases with analogous symptoms (including trichophytia, sporotrichosis, inguinal lymphogranuloma) should be ruled out. If there is a pronounced fistula formation, this can be made visible by contrast imaging or by contrast media in the X-ray image (fistulography).
If left untreated, hidradenitis suppurativa has a progressive-chronic course that leads to complete destruction of the affected skin and subcutaneous fatty tissue. Since the disease only rarely shows spontaneous remissions, hidradenitis suppurativa should always be treated consistently and adequately in order to avoid complications and social consequences of the usually recurrent disease.
Complications
Hidradenitis suppurativa leads to the formation of ulcers and fistulas. These are often filled with pus and can cause severe pain. This pain continues to limit mobility. It is not uncommon for patients to suffer from depression and other mental disorders as a result of movement restrictions.
The patient’s entire everyday life is also severely restricted by the hidradenitis suppurativa and the quality of life is reduced. The affected person appears exhausted and tired and no longer actively participates in life. In most cases, the disease also leads to social problems and often to exclusion. Nocturnal pain leads to sleep problems and thus to irritability of the patient.
Furthermore, the patient suffers from wound healing disorders, so that infections and inflammations can develop more easily and quickly. The immune system is also weakened. Antibiotics and surgical interventions are mainly used in the treatment, whereby there are usually no special complications. The complaints can be limited relatively well. Life expectancy is also usually not reduced by the disease.
When should you go to the doctor?
People who don’t have the flu or a cold but still suffer from swollen lymph nodes should see a doctor. The swellings must be clarified and examined more closely. It is particularly worrying if the lymph shows changes over several weeks. A doctor’s visit is necessary as soon as the swelling spreads or the lump increases in size. If further ulcers, abscesses or a diffuse lump develop on the body, a doctor should be contacted.
Persistent or spreading skin abnormalities and discolouration must also be examined medically. If you have a persistent headache, an increased body temperature or an unusual feeling of heat in the body, it is advisable to consult a doctor. If psychological abnormalities occur, the behavior of the person affected changes significantly or mood swings set in, a doctor should be asked for advice and support. A reduced well-being, a loss of quality of life or a decrease in the usual level of performance must be discussed with a doctor.
If the usual everyday duties can no longer be fulfilled, a visit to the doctor is advisable. If the person concerned suffers from insomnia, reduced concentration or attention, he needs help. If you feel ill, if you feel unwell or have a general weakness, you should see a doctor to find the cause of the abnormalities.
Treatment & Therapy
Hidradenitis suppurativa is treated conservatively and/or surgically as standard. As part of conservative therapy, for example, hormonal imbalances can be compensated for with medication using antiandrogens (women) or 5-alpha reductase inhibitors (men).
Antibiotics can stabilize and reduce the symptoms resulting from bacterial inflammation. In addition, the use of TNF-alpha antagonists, which have proven themselves in Crohn’s disease and inhibit the tumor necrosis factor (TNF) involved in inflammatory processes in the organism, is being tested in clinical studies for the treatment of hidradenitis suppurativa. The first results are promising in this regard.
However, since the conservative therapy measures have so far proven to be insufficiently effective, surgical intervention is indicated in the long term due to the high risk of the inflammatory reactions spreading and extensive fistula formation. As part of a surgical procedure, all affected skin areas are completely excised.
Depending on the extent of the defect and the individual influencing factors (including any wound healing disorders that may be present ), different surgical procedures are available. While with a local flap after the excision a wound is closed by moving skin tissue from adjacent structures and with a split-thickness skin transplant the wound is closed with autologous skin transplants (e.g. from the thigh or back of the head), with secondary healing an open wound healing is aimed at.
In addition, those affected by hidradenitis suppurativa are recommended to follow a diet analogous to that of multiple sclerosis and rheumatic diseases and to avoid tight clothing and obesity.
Outlook & Forecast
Hidradenitis suppurativa is a very protracted disease that severely impairs the quality of life of the affected patients. To date, there are still no convincing concepts for a therapy with complete healing.
Without treatment, the prognosis is not good. Larger abscesses can develop in these cases on the basis of the existing inflammation. Eventually, phlegmon develops in the subcutaneous tissue. The extensive abscesses often lead to intensive formation of fistulas. It is not uncommon for a potentially fatal sepsis to develop from cellulitis in the subcutaneous tissue.
The disease is usually long-lasting and recurring, even with treatment. This in turn causes severe psychological stress for those affected. In addition to the chronic and excruciating pain, the inflammatory skin changes are perceived as strongly stigmatizing. The affected patients often have to be on sick leave for a long time during the very lengthy therapy, which in turn can lead to job loss and social exclusion. For this reason, this disease has also been recognized as a disability under social law in Germany. In addition to chronic pain, patients often suffer from mental illnesses due to social exclusion.
So far, there has been little experience of successful treatment, as there is still no uniform therapy concept. However, successes in the use of various laser methods have already been reported from some smaller clinics.
Prevention
Since the etiology has not yet been conclusively clarified, hidradenitis suppurativa cannot be prevented. Avoiding possible triggering factors such as nicotine consumption or obesity can at least favor the course of hidradenitis suppurativa if there is a family prevalence (accumulation).
Aftercare
In the case of the disease hidradenitis suppurativa, in most cases the patient has very few or no options or direct measures for follow-up care. The patient is primarily dependent on rapid and early detection of the disease so that there are no further complications or symptoms. The earlier hidradenitis suppurativa is recognized and treated, the better the further course of this disease will usually be.
A doctor should therefore be consulted at the very first signs or symptoms of the disease. In most cases, the disease is treated by taking medication, with antibiotics in particular being used. The person concerned should pay attention to regular intake with the correct dosage. If anything is unclear or if you have any questions, you should always consult a doctor first.
Those affected should also consult a doctor in the event of interactions or side effects. Since hidradenitis suppurativa can also lead to wound healing disorders, injuries should generally be avoided. Wounds need special care to prevent infection or inflammation. Most of the time, life expectancy is not reduced by the disease.
You can do that yourself
Patients with hidradenitis suppurativa are noticeably restricted in their everyday life due to the unpleasant symptoms. The inflamed foci of pus and fistulas cause pain both in a resting position and during movements. As a result, those affected suffer from reduced mobility and, as a result, from irritability. To relieve the pain, make sure that the clothing does not press on or rub against the inflammation, especially when you move. Patients choose cosmetics together with a pharmacist or the attending physician so as not to expose the inflamed areas to additional irritation.
Sensitive hygiene of the diseased skin areas is also important. The sores should be kept clean and free of debris, but the fistulas should be protected from further irritation during cleaning. Some of the patients are no longer able to carry out certain types of sport without difficulty and therefore look for opportunities for physical activity that are compatible with the disease.
Patients are also working to reduce known risk factors for the disease to promote treatment success. For example, those affected stop smoking and reduce excess weight. In addition, they avoid additional psychological burdens and stress.
