Perforating dermatoses are divided into primary and secondary. The acquired perforating dermatosis is a rare chronic skin disease that is associated with the former group. It usually occurs in adults with chronic kidney disease. Dialysis patients or diabetics can also be affected. Symptoms are very itchy nodules.
What is acquired perforating dermatosis?
Acquired perforating dermatosis is extremely rare and usually occurs, if at all, in adults between the ages of 30 and 80 years. Men as well as women can be affected, there is no gender-specific accumulation. The EPF is related to chronic kidney disease. Kidney transplants can also be affected by the disease, as can diabetics and around ten percent of dialysis patients.
Acquired perforating dermatosis can occur worldwide and affect people of all races and ethnic backgrounds, but it is more common in African Americans. The reasons for this are not yet known. There is still no research into what causes the acquired perforating dermatosis. It is believed that a metabolic disorder may be responsible for the build-up of cell waste, which becomes inflamed. But the causes of this disorder are still unknown.
Causes
The risk of acquired perforating dermatosis is particularly high in people with chronic kidney disease and those on dialysis. Badly controlled diabetes can also promote EPD. In some cases, EPD has also been described for hyperthyroidism or hypothyroidism, herpes zoster, lepromatous leprosy or people with Down syndrome or prostate cancer.
Furthermore, patients with diseases of the liver or infectious diseases such as scabies or AIDS belong to the risk group. It is believed that frequent scratching can trigger an inflammatory or foreign body reaction in the skin. Deposits of uric acid crystals on the skin could also trigger the EPD.
Symptoms, ailments & signs
Typical symptoms of acquired perforating dermatosis are bulging lesions, also called papules, with a keratinized plug in the center. The diameter of these papules can be between two and eight millimeters. They can also merge into linear structures and appear all over the body, but often on the chest, back, arms and thighs, as well as the face and neck region.
But hairy parts of the body, such as the scalp, can also be affected. The papules appear pink on light skin, hyperpigmented on dark skin, and they are often associated with severe itching. Over time, a chronic inflammatory reaction with the formation of granulomas develops in the lesions. It is difficult to say which risk group can be affected by a stronger or weaker form of EPD.
Diagnosis
Since a number of skin diseases have symptoms similar to acquired perforating dermatosis, an in-depth examination is necessary in order to be able to make a clear diagnosis. Therefore, a comprehensive physical examination will first be carried out and the patient’s medical history will be examined in detail. The next step is then a dermatoscopy, a non-invasive examination method.
The skin is examined with a special microscope, the dermatoscope. Dermoscopy also makes it possible to show deeper layers of the skin. Some of these devices also have polarized light, which further improves the appearance of the skin layers.
A skin biopsy can also be performed for an even more precise diagnosis. In this case, tissue removed and examined under a microscope. In the case of an EPD, this histological examination shows invaginations of the epidermis, which have a cornified plug containing basophilic cell debris (hair follicles or uric acid crystals).
Complications
Acquired perforating dermatosis is a skin condition that manifests itself in the form of itchy nodules. The symptom shows up in diabetics as well as people with chronic kidney disease, dialysis patients, who have had a kidney transplant and people with black skin color. The cause of the disease can be found in the body’s own reaction, which impairs the regeneration of connective tissue and the epidermis.
In some cases, uric acid crystals can be microscopically detected as deposits within the skin, which trigger the symptom. If the affected person treats the first skin nodules incorrectly or ignores them, papules form as a complication. These can be up to one centimeter in size and connect to one another over a large area. The syndrome causes extremely severe itching and occurs predominantly on all hairy parts of the body, the extremities and the face.
The acquired perforating dermatosis appears hyperpigmented on dark skin, and tends to be dark pink on light skin. If the condition is not treated by a doctor, the syndrome develops chronically. Granulomas develop in the center of the nodule and the intense itching scratches the skin. Scars develop and, in the worst case, dermatological necrosis.
The clinical fight against the disease is multimodal. Glucocorticoids, retinoids or high-dose vitamin A are administered to the patient. UV-B phototherapy and skin lotions with cortisone and horn-loosening substances have a supportive effect. The coordinated medical therapy usually runs without complications.
When should you go to the doctor?
A doctor should be consulted in the event of an unusual change in the skin’s appearance. If the changes spread to the body or if they increase in intensity, it is advisable to have them checked out by a doctor. Lesions, swellings or redness are considered to be of concern and should be examined and treated by a doctor.
If the skin changes appear on the scalp, they should also be examined by a doctor. Particular caution should be exercised if itching is present. If the stimulus is given in, open wounds can develop. Pathogens enter the organism through this and can cause further diseases. In severe cases there is a risk of blood poisoning.
If the wounds become inflamed and pus forms, it is advisable to consult a doctor. If the person concerned suffers from emotional problems, the help of a doctor is also recommended. In the case of psychological stress, mood swings or a depressive phase, a doctor should be consulted as soon as they persist for several weeks.
If feelings such as shame, decreased self-esteem or lack of drive occur, these should be discussed with a doctor. If the skin changes increase through the use of cosmetic products, the desired articles and the product quality can be optimized in cooperation with a doctor.
Treatment & Therapy
A number of methods can be used to treat acquired perforating dermatosis. In milder cases, caring for the skin with moisturizing ointments can bring relief. Basically, mild cleaning products without additives should be used. Excessive washing of the skin should be avoided, but there is nothing wrong with an occasional, not too hot, full bath.
Even when showering, the water should not be too hot. Cotton clothing, preferably free of harsh chemicals, is recommended. Also, if possible, you should not give in to the itching, as this leads to a worsening. Itch-relieving ointments are therefore usually also prescribed. In more severe cases of EPD, treatment of the skin with phototherapy has been shown to be helpful.
The cryotherapy is used to treat. The skin is exposed to very low temperatures, which reduces itching. Some doctors prescribe vitamin A. Some studies have also shown the use of steroids or drugs to lower uric acid levels to be effective.
Outlook & forecast
The prognosis for acquired perforating dermatosis varies greatly from patient to patient. Because the skin condition cannot be treated as a cause and a multitude of therapeutic agents can be considered, different statements can be made with regard to the prospects.
As a rule, however, it can be assumed that those affected can significantly alleviate or even prevent the suffering if they use the ointments, vitamins and medication according to the therapy plan. However, the healing process is individual in each case.
A few factors can be named that reduce the chances of recovery or at least slow down your healing process. These include, for example, strong sunlight, strong scratching, wearing clothes that are too tight, very hot baths and other illnesses. Since the acquired perforating dermatosis is particularly often associated with other ailments, a worsening of these clinical pictures can also have a negative effect on the chances of recovery.
On the other hand, following the therapy plan and maintaining skin health (for example through a healthy diet) have a positive influence on the prognosis.
Scarring after the skin condition has healed is common. It is possible for the disease to flare up again. In some sufferers it becomes chronic and its symptoms can only be suppressed permanently.
Prevention
Since the acquired perforating dermatosis is a chronic skin condition, it can naturally lead to permanent skin damage. Frequent scratching can scar the skin. This in turn leads to additional emotional stress in the affected patient. Bacterial or fungal infections can also occur. The constant scratching leads to oozing of the skin, an ideal breeding ground for bacteria and fungi.
Since there is still no research into what causes the acquired perforating dermatosis, there are still no methods of preventing it. The only effective method of prevention is to have regular check-ups and to ensure that the diabetes is optimally controlled. It remains to be noted that acquired perforating dermatosis is a very rare condition. It is therefore very likely that only an extremely small proportion of the so-called risk patients will develop it at all.
Aftercare
As a rule, the person affected with this disease has only very limited measures and options for follow-up care available. The affected person is primarily dependent on a direct and rapid diagnosis with subsequent treatment. This is the only way to prevent further complications or worsening of the symptoms.
This disease cannot heal itself either, so an examination by a doctor is essential. The treatment of this disease is mostly done through the use of creams or ointments. The doctor’s instructions must always be followed, and the person concerned should always consult the doctor if they have any questions or doubts.
Regular examinations by a doctor are also very important in order to regularly check the condition of the skin. It is not uncommon for patients to be dependent on taking vitamins to alleviate the symptoms. It is also important to ensure that it is taken regularly and that the dosage is correct.
Since the disease can also have a negative impact on the aesthetics of those affected, discussions with friends or family are often very helpful. However, professional psychological support may also be necessary.
You can do that yourself
People with acquired perforating dermatosis are often diabetics and can improve their quality of life in everyday life by eating a healthy diet. The excessive consumption of sugar should be avoided. Food should also be avoided that cause a strong conversion of blood sugar within the digestive process. These include foods that contain carbohydrates. The consumption or processing of flour, white bread, yoghurt, pasta or legumes should therefore be reduced.
If itching occurs, it is important not to give in to it by scratching or scrubbing, if possible. Relaxation techniques can be used to distract the uncomfortable skin reaction. These help to establish an inner balance and support the reduction of stress.
For mental strengthening, it is also helpful if the patient talks about his or her inner experience, his experiences with the disease and the daily challenges with everyday life. The exchange can take place with relatives, therapists, friends or in self-help groups. Tips and assistance can be given to each other in dealing with the visual changes in the disease.
When wearing the clothing, care must be taken that if papules form, they are not inadvertently damaged by the friction during movement. It is also advisable to avoid excessive washing or rubbing the skin.
