Acquired perforating dermatosis is extremely rare and, if it occurs at all, usually occurs in adults between the ages of 30 and 80. Both men and women can be affected, there is no gender-specific accumulation. EPF is associated with chronic kidney disease . Kidney transplant recipients 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 racial and ethnic backgrounds, but African Americans are more commonly affected. The reasons for this are not yet known. It has not yet been researched what triggers the acquired perforating dermatosis. It is thought that a metabolic disorder may be responsible for the buildup of cellular debris that becomes inflamed. However, the causes of this disorder are still unknown.

causes

The risk of developing acquired perforating dermatosis is particularly high in people with chronic kidney disease and 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’s syndrome or prostate carcinoma .

Furthermore, patients with liver diseases or infectious diseases such as scabies or AIDS belong to the risk group. It is believed that frequent scratching can cause an inflammatory or foreign body reaction in the skin. Deposits of uric acid crystals on the skin could also trigger EPD.

Symptoms, Ailments & Signs

Typical symptoms of acquired perforating dermatosis are convex lesions, also called papules , with a keratinized plug in the middle. The diameter of these papules can be between two and eight millimeters. They can also merge into linear structures and occur all over the body, but commonly on the chest, back, arms and thighs, and the face and neck region.

But hairy parts of the body, such as the scalp, can also be affected. The papules appear pink on fair skin, hyperpigmented on dark skin , and are often associated with intense itching . A chronic inflammatory response develops in the lesions over time with the formation of granulomas . It is difficult to say which risk group may be affected by a stronger or weaker form of EPD.

Diagnose

Since a number of skin diseases have symptoms similar to acquired perforating dermatosis, a detailed examination is necessary in order to be able to make a clear diagnosis. Therefore, a comprehensive physical examination is carried out first and the medical history of the patient is examined in detail. The next step is a dermatoscopy, a non-invasive examination method.

The skin is examined with a special microscope, the dermatoscope. Dermatoscopy makes it possible to visualize deeper skin layers. Some of these devices also have polarized light, which further improves the display of the skin layers.

A biopsy of the skin can also be performed for an even more precise diagnosis. Tissue is removed and examined under a microscope. In the case of EPD, this histological examination shows invaginations of the epidermis, which have a keratinized plug containing basophilic cell debris ( hair follicles or uric acid crystals).

complications

Acquired perforating dermatosis is a skin disease that manifests itself in the form of itchy nodules. The symptom appears in diabetics, chronic kidney disease patients, dialysis patients, after a kidney transplant and people with black skin. The cause of the disease can be an endogenous reaction that impairs the regeneration of connective tissue and the 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 person concerned, papules form as a complication. These can grow up to a centimeter in size and connect to each other over a large area. The syndrome causes extremely severe itching and occurs mainly on all hairy parts of the body, the extremities and the face.

Acquired perforating dermatosis appears hyperpigmented on darker skin and more dark pink on lighter skin. If the condition is not treated by a doctor, the syndrome becomes chronic. Granulomas form in the center of the nodule and the skin is scratched by the intense itching. Scars develop and, in the worst case, dermatological necrosis.

Clinical control of the disease is multimodal. The patient is given glucocorticoids, retinoids or high-dose vitamin A. UV-B phototherapy and skin lotions with cortisone and horn-dissolving substances have a supporting effect. The coordinated medical therapy usually runs without complications.

When should you go to the doctor?

A doctor should be consulted if there is an unusual change in the appearance of the skin. If the changes in the body spread or increase in intensity, it is advisable to have them checked out by a doctor. Lesions, swelling or redness are of concern and should be evaluated and treated by a doctor.

If the skin changes occur on the scalp, they should also be examined by a doctor. If itching is present, special care should be taken. If the stimulus is given in, open wounds can develop. Pathogens enter the organism via these and can cause further diseases. In severe cases, there is a risk of blood poisoning.

If the sores 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 mental stress, mood swings or a depressive phase, a doctor should be consulted as soon as they last for several weeks.

Feelings of shame, reduced self-esteem or listlessness should be discussed with a doctor. If the skin changes worsen as a result of 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 full bath that is not too hot.

Even when showering, the water should not be too hot. Cotton clothing, preferably without harsh chemicals, is recommended. Also, if possible, you should not give in to the itching, as this leads to a deterioration. Antipruritic ointments are therefore usually also prescribed. In more severe cases of EPD, treatment of the skin with phototherapy has been shown to be helpful.

Cryotherapy is also used for treatment. The skin is exposed to very low temperatures, which reduces itching. Some doctors prescribe vitamin A. Some studies have also found 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. Due to the fact that the skin condition cannot be treated causally and a large number of therapeutic agents can be considered, there are different statements regarding the prospects.

As a rule, however, it can be assumed that those affected can significantly alleviate or even prevent the suffering if they make use of the ointments, vitamins and medicines according to the therapy plan. However, the healing process is individual in each case.

A few factors can be named that reduce the chance of healing or at least slow down your healing process. These include, for example, strong sunlight, severe scratching, wearing clothing that is too tight, very hot baths and other illnesses. Since the acquired perforating dermatosis is particularly often associated with other diseases, a worsening of these clinical pictures can also have a negative effect on the prospects of recovery.

On the other hand, following the therapy plan and maintaining skin health (e.g. through a healthy diet) have a positive influence on the prognosis.

Scarring after the skin condition has healed is common. It is possible that the disease will flare up again. In some of those affected, it also becomes chronic and its symptoms can only be suppressed permanently.

prevention

Since acquired perforating dermatosis is a chronic skin disease, it can of course lead to permanent skin damage. Frequent scratching can lead to scarring of the skin. This in turn leads to additional emotional stress for the affected patients . Bacterial or fungal infections can also occur. The constant scratching leads to wetting of the skin, an ideal breeding ground for bacteria and fungi.

Since it has not yet been researched what causes acquired perforating dermatosis, there are also no methods of preventing it. The only proven 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 disease. 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, only very limited measures and options for aftercare are available to those affected by this disease. 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.

It is also not possible for this disease to heal itself, so that an examination by a doctor is essential. This disease is usually treated with 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 are in doubt.

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 depend on taking vitamins to alleviate the symptoms. It is also important to ensure that it is taken regularly and that the correct dosage is used.

Since the disease can also have a negative effect on the aesthetics of those affected, it is not uncommon for conversations with friends or family to be very helpful. However, professional psychological support may also be necessary.

You can do that yourself

Patients suffering from an acquired perforating dermatosis are often diabetics and can improve their quality of life in everyday life by paying attention to a healthy diet . Excessive consumption of sugar should be avoided. You should also avoid foods that cause a strong conversion of blood sugar within the digestive process. This includes foods that contain carbohydrates. The consumption or processing of flour, white bread, yoghurt , pasta or legumes should therefore be reduced.

It is important not to give in to itching by scratching or rubbing if it occurs. Relaxation techniques can be used to distract the unpleasant skin reaction . These help to create an inner balance and support stress reduction.

It is also helpful for mental strengthening if the patient talks about his inner life, his experiences with the illness and the daily challenges with everyday life. The exchange can take place with relatives, therapists, friends or in self-help groups. In dealing with the optical changes of the disease, mutual tips and assistance can be given.

When wearing clothing, care should be taken to ensure that if papules form, they are not accidentally damaged by friction during movement. It is also advisable if excessive washing or rubbing on the skin is avoided.