Perforating dermatoses are divided into primary and secondary. Acquired perforating dermatosis is a rare chronic skin disease classified in the former group. It usually occurs in adults suffering from chronic kidney disease. It may also affect dialysis patients or diabetics. The symptoms are severely 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. It can affect men as well as women; there is no gender-specific clustering. EPF is associated with chronic kidney disease. Kidney transplant patients can also be affected by the disease, as can diabetics and about ten percent of dialysis patients. Acquired penetrating dermatosis can occur worldwide and affect members of all races and ethnic backgrounds, but African Americans are more commonly affected. The reasons for this are not yet known. It is also not yet understood what causes acquired perforating dermatosis. It is suspected that a metabolic disorder may be responsible for the deposition of cellular debris that becomes inflamed. However, the causes of this disorder are also unknown as of yet.
Causes
The risk of developing acquired perforating dermatosis is particularly high in people with chronic kidney disease as well as patients requiring dialysis. Poorly controlled diabetes can also promote EPD. In some cases, EPD has also been described in hyperthyroidism or hypothyroidism, herpes zoster, lepromatous leprosy, or people with Down syndrome or prostate cancer. Furthermore, patients with liver diseases or infectious diseases such as scabies or AIDS belong to the risk group. It is assumed that frequent scratching can cause an inflammatory or foreign body reaction of the skin. Deposits of uric acid crystals on the skin could also be triggers of EPD.
Symptoms, complaints, and signs
Typical symptoms of acquired perforating dermatosis are domed lesions, also called papules, with a keratinized plug in the center. The diameter of these papules can range from two to 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. However, hairy areas of the body, such as the scalp, may also be affected. The papules appear pinkish on light skin, hyperpigmented on dark skin, and they are often accompanied by intense itching. Over time, a chronic inflammatory reaction develops in the lesions with the formation of granulomas. It is difficult to say which risk group may be affected by a more severe or weaker form of EPD.
Diagnosis
Because a number of skin diseases have symptoms similar to those of acquired penetrating dermatosis, a thorough examination is necessary to make a definitive diagnosis. Therefore, a comprehensive physical examination is first performed, and the patient’s medical history is reviewed in detail. The next step is then a dermoscopy, a non-invasive examination method. This involves examining the skin with a special microscope called a dermatoscope. Dermatoscopy makes it possible to visualize deeper layers of the skin. Some of these devices also have polarized light, which further improves the visualization of the skin layers. For an even more precise diagnosis, a biopsy of the skin can also be performed. Tissue is removed and examined under a microscope. In the case of EPD, this histologic examination reveals invaginations of the epidermis that have a keratinized plug containing basophilic cell debris (hair follicles or even uric acid crystals).
Complications
Acquired perforating dermatosis is a skin disease that manifests as itchy nodules. The symptom is seen in diabetics as well as chronic kidney disease patients, dialysis patients, after kidney transplantation and people with black skin color. The cause of the disease may be an endogenous reaction that impairs the regeneration of connective tissue and epidermis.In some cases, uric acid crystals can be detected microscopically as deposits within the skin, which trigger the symptom. If the first skin nodules are treated incorrectly or ignored by the affected person, papules form as a complication. These can grow up to one centimeter in size and join together over a large area. The syndrome causes extremely intense itching and occurs predominantly on all hairy parts of the body, the extremities and the face. On dark skin, acquired perforating dermatosis appears hyperpigmented, while on light skin it tends to be dark pink. If the condition is not treated medically, the syndrome develops chronically. Granulomas develop in the nodal center and the skin is scratched due to the intense itching. Scars develop and in the worst case dermatological necrosis. Clinical control of the disease is multimodal. The patient is administered glucocorticoids, retinoids, or high-dose vitamin A. UV-B phototherapy as well as skin lotions with cortisone and horn-dissolving substances have a supportive effect. The coordinated medical therapy usually proceeds without complications.
When should one go to the doctor?
If there is an unusual change in the appearance of the skin, a doctor should be consulted. If the changes spread over the body or increase in intensity, it is advisable to have them clarified by a doctor. Lesions, swellings or redness are considered worrisome and should be examined and treated by a doctor. If the skin lesions occur on the scalp, they should also be evaluated by a physician. If itching is present, special care should be taken. If the irritation is indulged, open wounds may develop. Via these, pathogens enter the organism and can cause further illnesses. In severe cases, there is a risk of blood poisoning. If the wounds become infected and pus forms, it is advisable to consult a doctor. If the affected person suffers from emotional problems, the help of a doctor is also recommended. In the case of psychological stress, fluctuations in mood or a depressive phase, a doctor should be consulted as soon as they persist for several weeks. If feelings of shame, reduced self-esteem or listlessness occur, this should be discussed with a doctor. If the skin changes intensify due to the use of cosmetic products, an optimization of the desired articles as well as the product quality can take place in cooperation with a medical doctor.
Treatment and therapy
A number of methods can be used to treat acquired perforating dermatosis. In milder cases, care of the skin with lipid-replenishing ointments may provide relief. In general, mild cleansing 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 without aggressive chemicals, is recommended. Also, itching should not be indulged if possible, as this will lead to worsening. Anti-itching ointments are therefore usually prescribed as well. In more severe cases of EPD, treatment of the skin with phototherapy has been shown to be helpful. Cryotherapy is also used for treatment. This involves exposing the skin to very low temperatures, which reduces itching. Some doctors prescribe vitamin A. In some studies, the use of steroids or medications to lower uric acid levels has also been shown to be effective.
Outlook and prognosis
The prognosis for acquired perforating dermatosis varies greatly from patient to patient. Due to the fact that the skin condition cannot be treated causally and a variety of therapeutic agents can be considered, there are different statements regarding the outlook. As a rule, however, it can be assumed that those affected will be able to significantly alleviate or even eliminate the condition if they make use of the ointments, vitamins and medications in accordance with the therapy plan. However, the healing process is individual in each case. Some factors can be named, which reduce the healing prospect or at least slow down your healing process. Among them are, for example, strong sunlight, strong scratching, wearing too tight clothes, very hot baths and other diseases.Since Acquired Perforating Dermatosis is particularly often associated with other conditions, a worsening of these conditions can also have a negative impact on the prospects of cure. On the other hand, following the treatment plan and maintaining skin health (for example, through a healthy diet) have a positive influence on the prognosis. Scarring after healing of the skin condition is common. It is possible for the disease to flare up again. In some sufferers, it also becomes chronic and its symptomatology can only be permanently suppressed.
Prevention
Because acquired perforating dermatosis is a chronic skin disease, it can naturally lead to permanent skin damage. Frequent scratching can lead to scarring of the skin. This, in turn, leads to additional emotional stress in affected patients. Bacterial or fungal infections can also occur. Constant scratching leads to oozing of the skin, an ideal breeding ground for bacteria and fungi. Since it is not yet known what causes acquired perforating dermatosis, there are no methods to prevent it. The only proven method of prevention is to attend regular check-ups and to pay attention to optimal diabetes control. It should be noted that acquired perforating dermatosis is a very rare disease. Therefore, it is very likely that only an extremely small proportion of so-called high-risk patients will develop it at all.
Follow-up
As a rule, only very limited measures and options for aftercare are available to the affected person with this disease. The affected person is primarily dependent here on a direct and rapid diagnosis with subsequent treatment. This is the only way to prevent further complications or a worsening of the symptoms. It is also not possible for this disease to heal itself, so that an examination by a physician is indispensable. The treatment of this disease is mostly done by using creams or ointments. The doctor’s instructions should always be followed, and the affected person should always consult the doctor if there are any questions or doubts. Likewise, regular examinations by a doctor are very important to check the condition of the skin regularly. It is not uncommon for patients to be dependent on taking vitamins to alleviate the symptoms. In this case, regular intake and also the correct dosage must also be ensured. Since the disease can also have a negative effect on the aesthetics of the person affected, it is not uncommon for discussions with friends or one’s own family to be very helpful. However, professional psychological support may also be necessary.
What you can do yourself
Sufferers of acquired perforating dermatosis are often diabetic and can achieve an improvement in their quality of daily life by paying attention to a healthy diet. Excessive consumption of sugar should be avoided. Likewise, foods that produce a strong conversion of blood sugar within the digestive process should be avoided. These include foods that contain carbohydrates. The consumption or processing of flour, white bread, yogurt, pasta or legumes should therefore be reduced. If itching occurs, it is important not to give in to it by scratching or rubbing, if possible. Relaxation techniques can be used to deflect the unpleasant skin reaction. These help to establish an inner balance and support stress reduction. For mental strengthening, it is also helpful if the patient talks with everyday life about his inner experience, his experiences with the disease as well as the daily challenges. The exchange can take place with relatives, therapists, friends or in self-help groups. In dealing with the visual changes of the disease, mutual tips as well as assistance can be given. When wearing clothing, care should be taken to ensure that if papules form, they are not unwillingly damaged by friction when moving around. It is also advisable to avoid excessive washing or rubbing the skin.
