Hidradenitis suppurativa (acne inversa) is inflammatory skin disease with a progressive-chronic course, which affects women slightly more often than men. The peak age for hidradenitis suppurativa is between 20 and 30 years of age.
What is hidradenitis suppurativa?
Hidradenitis suppurativa is a chronic inflammatory disease of the sebaceous glands as well as the terminal hair follicles associated with them, which is accompanied by painful nodule and abscess formation. The skin lesions usually develop under the armpits, on the female breast, in the groin area, and in the perianal and perigenital areas. Initially, the disease manifests itself as giant comedones (large blackheads) and solitary palpable nodules, which later develop into larger pus collections with deeper fistulous ducts and pronounced nodules that coalesce into strands. The disease can cause serious somatic and psychological disorders due to its painful symptoms and foul-smelling secretion. Hidradenitis suppurativa is also associated with an increased risk of skin, oral mucosa, and liver cancers.
Cause
The exact etiology of hidradenitis suppurativa has not been clearly established. What is known is that due to ichthyosis (cornification disorder), horny material is increasingly accumulated in the sebaceous glands and hair roots. In addition, a bacterial infection (usually with Staphylococcus aureus) and purulent inflammation of the sebaceous gland occur in the affected areas. Due to the accumulation of pus, the sebaceous gland cyst ruptures, allowing the inflammation to spread to the adjacent tissue and causing secondary sweat gland inflammation. Painful abscesses develop and, as the disease progresses, fistulas. Bacterial colonization of the blood vessels located in the subcutis 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 favoring factors. In addition, due to familial clustering, a genetic predisposition to hidradenitis suppurativa is discussed.
Symptoms, complaints, and signs
Hidradenitis suppurativa is primarily manifested by inflamed sebaceous glands, which are usually filled with pus or wound fluid. Additional ulcers, abscesses, or fistulas usually form in the affected region. These increase in size in the course of the disease and often also fill with pus. Severe pain occurs when touched. The skin changes considerably restrict the mobility of the affected body region. In most cases, even slight friction causes intense pain or even rupture of the abscesses. The pain often also occurs at night and at rest, causing the affected person to have trouble sleeping. Furthermore, there are disturbances in wound healing. This leads to further infections and inflammations in the affected skin areas, and the general condition of the affected person increasingly deteriorates. Many patients develop psychological complaints as a result of the skin changes, such as depressive moods or inferiority complexes. The affected person usually appears fatigued and tired. Active participation in life no longer takes place. In most cases, the disease leads to withdrawal from social life. The symptoms persist for several days to weeks and subside rapidly with appropriate treatment.
Diagnosis and course
Hidradenitis suppurativa is diagnosed on the basis of clinical symptoms. The diagnosis is confirmed by a biopsy with subsequent histologic examination, which also serves to differentiate it from similar clinical pictures such as Crohn’s disease with skin involvement or tuberculosis cutis colliquativa. In addition, other infection-related diseases with an analogous clinic (including trichophytosis, sporotrichosis, lymphangranuloma inguinale) should be excluded. If a pronounced fistula formation is present, it can be visualized by contrast imaging or by contrast medium in the X-ray (fistulography). If left untreated, hidradenitis suppurativa shows a progressive-chronic course leading to complete destruction of the affected skin and subcutaneous fat tissue.Since the disease rarely shows spontaneous remissions, hidradenitis suppurativa should be treated consistently and adequately in every case to avoid complications and social consequences of the usually recurrent disease.
Complications
Hidradenitis suppurativa results in the formation of ulcers and fistulas. These are not infrequently filled with pus and can cause severe pain. This pain further leads to movement restrictions. It is not uncommon for patients to suffer from depression and other psychological upsets due to movement restrictions. The patient’s entire daily life is also severely restricted by hidradenitis suppurativa and the quality of life is reduced. The affected person appears fatigued and tired and no longer takes an active part in life. In most cases, the disease also leads to social discomfort and not infrequently 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. Treatment mainly involves antibiotics and surgical interventions, although there are usually no particular complications. The symptoms can be relatively well limited. Life expectancy is also usually not reduced by the disease.
When should you see a doctor?
People who do not have the flu or a cold but still suffer from swollen lymph nodes should consult a doctor. The swellings should be clarified as well as examined more closely. It is particularly worrying if the lymph shows changes over several weeks. A visit to the doctor is necessary as soon as the swellings spread or the lump formation increases in size. If further ulcers, abscesses or diffuse lump formation develop on the body, a doctor should be contacted. Persistent or spreading skin abnormalities and discoloration should also be investigated medically. If headaches persist, body temperature is elevated, or there is an unusual sensation of warmth in the body, it is advisable to consult a physician. If psychological abnormalities occur, the behavior of the affected person changes to a considerable degree or mood swings set in, a doctor should be asked for advice and support. A reduced sense of well-being, a loss of quality of life or a decrease in the usual level of performance should be discussed with a physician. If the usual daily duties can no longer be performed, a visit to the doctor is advisable. If the affected person suffers from sleep disturbances, reduced concentration or attention, he or she needs relief. If there is a feeling of illness, persistent malaise or general weakness, a doctor should be consulted to find the cause of the abnormalities.
Treatment and therapy
Hidradenitis suppurativa is treated conservatively and/or surgically as standard of care. For example, as part of conservative therapy, hormonal imbalances can be compensated for medicinally with antiandrogens (women) or 5-alpha reductase inhibitors (men). Antibiotics can stabilize and reduce symptoms resulting from bacterial inflammation. In addition, the use of TNF-alpha antagonists, which have proven effective in Crohn’s disease and inhibit tumor necrosis factor (TNF) involved in inflammatory processes in the organism, is being tested in clinical trials for the treatment of hidradenitis suppurativa. The initial results are promising in this regard. However, since conservative therapeutic measures have so far proved to be insufficiently effective, surgical intervention is indicated in the long term due to the high risk of spreading inflammatory reactions as well as extensive fistula formation. In 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), various surgical procedures are available.While suture flap plasty achieves wound closure after excision by displacing skin tissue from adjacent structures and split-thickness skin transplantation closes the wound with autologous skin grafts (e.g., from the thigh or back of the head), secondary healing strives for open wound healing. In addition, those affected by hidradenitis suppurativa are advised to follow a diet analogous to that used for multiple sclerosis and rheumatic diseases and to avoid tight clothing and obesity.
Outlook and prognosis
Hidradenitis suppurativa represents a very protracted disease that severely affects the quality of life of affected patients. To date, there are no convincing concepts for therapy with complete cure. Without treatment, the prognosis is not good. Based on the existing inflammation, larger abscesses can form in these cases. Further on, phlegmons on the subcutaneous tissue eventually occur. The extensive abscesses often lead to intensive formation of fistulas. Phlegmons in the subcutaneous tissue not infrequently develop into potentially fatal sepsis. The disease is usually long-lasting and recurrent, even with treatment. This in turn causes severe psychological distress for those affected. In addition to the chronic and excruciating pain, the inflammatory skin lesions are perceived as highly stigmatizing. Affected patients often have to take long periods of sick leave during the very protracted therapy, which in turn can lead to loss of employment 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 illness due to social exclusion. To date, there is little experience of successful treatment, as there is as yet no uniform therapy concept. However, success in the use of various laser methods has already been reported from some smaller clinics.
Prevention
Because the etiology has not yet been conclusively determined, hidradenitis suppurativa cannot be prevented. Avoidance of possible triggering factors such as nicotine consumption or obesity may at least favor the course of hidradenitis suppurativa if there is a familial prevalence (clustering).
Follow-up
In most cases of hidradenitis suppurativa, the patient has very few or no options or direct measures of follow-up care. Here, the patient is primarily dependent on rapid and early detection of the disease to prevent further complications or discomfort. The earlier hidradenitis suppurativa is detected and treated, the better the further course of this disease usually is. 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, mainly antibiotics. The affected person should ensure that the medication is taken regularly and in the correct dosage. If there are any uncertainties or questions, a doctor should always be consulted first. In the event of interactions or side effects, patients should also consult a doctor. Since hidradenitis suppurativa can also lead to disturbances in wound healing, injuries should generally be avoided. Wounds must be given special care to prevent infection or inflammation. In most cases, life expectancy is not reduced by the disease.
This is what you can do yourself
Patients with hidradenitis suppurativa are noticeably limited in their everyday life by the unpleasant symptoms. The inflamed pus foci and fistulas cause pain both in a resting position and during movement. As a result, those affected suffer from reduced mobility and, consequently, irritability. To relieve pain, care should be taken to ensure that clothing does not press on or rub against the inflammation, especially during movement. Patients select cosmetics together with a pharmacist or the attending physician to avoid exposing the inflamed areas to additional irritation. Sensitive hygiene of the affected skin areas is also important. The inflamed areas should be kept clean and free of debris, but the fistulas should be protected from further irritation during cleansing.Patients are sometimes no longer able to perform certain sports without difficulty and therefore seek out opportunities for physical activity that are compatible with the disease. To promote treatment success, patients also work to reduce known risk factors for the disease. For example, sufferers stop smoking and reduce obesity. They also avoid additional psychological stress and strain.
