Pityriasis rubra pilaris (PRP) is a very rare skin disease that is often confused with psoriasis. Despite similar symptoms, this syndrome is a group of different skin diseases that are unrelated. Pityriasis rubra pilaris usually heals on its own.
What is pityriasis rubra pilaris?
Pityriasis rubra pilaris is a collective term for different skin diseases with the same symptoms. In rare cases, a gene mutation can be the cause. In most cases, however, the cause is unclear. Pityriasis rubra pilaris is also known as prickly lichen or divergie disease. It was first described in 1856 by the Paris dermatologist Marie Guillaume Devergie. See sportingology for Meaning of Lactic Acidosis in English.
The disease is very rare. In the medical literature, it is given with a probability of 1 in 1000,000 or 1 in 500,000. Pityriasis rubra pilaris is not a dangerous disease. But it is characterized by the appearance of itchy skin nodules on the trunk and head. It often starts in the hair follicles. The skin irritations appear patchy, with a smooth transition to a flat skin rash (erythroderma). In most cases, the disease occurs between the ages of 40 and 60.
In most cases, the causes of pityriasis rubra pilaris are not known. Originally, it was assumed that the disease was a uniform disease that was hereditary. It is now known that only about five percent of all cases are clearly due to hereditary causes. Mutations in the CARD14 gene at locus 17q25.3 are said to be responsible for the disease. The inheritance is autosomal dominant. However, sporadic cases also occur. However, this gene mutation causes an atypical juvenile form of the disease.
Furthermore, if this gene is inherited dominantly, the disease should not always break out. Rather, it has been established that there are six different types of pityriasis rubra pilaris:
- the classic adult type
- the atypical adult type
- the classic juvenile type
- the circumscribed juvenile type
- the atypical juvenile type
- the HIV-associated PRP
Most cases of pityriasis rubra pilaris are not hereditary. However, the exact genesis of the disease is not known. The very rare hereditary form is much more difficult to treat than the other forms. In the classic adult type, a connection with skin cancer is suspected.
However, the incidence of skin cancer when this type occurs is also not known. Some research suggests an abnormality in the processing of vitamin A as the cause of pityriasis rubra pilaris. But an incorrect reaction of the immune system is also taken into account.
Symptoms, Ailments & Signs
Pityriasis rubra pilaris is manifested by bright red, pointed nodules on the trunk, head and the extensor sides of the extremities. There is also scaly erythema on the skin. The nodules usually start at the hair follicles. There is normal skin between the knots, so the skin irritation appears patchy. The affected skin areas feel rough and give a feeling of grating. There is also increased callus formation on the skin .
On the palms of the hands, in addition to the increased formation of calluses, there are also fissures in the skin (rhagades). There is constant moderate itching. In the acute phase, a general reddening of the skin can occur. At no time, however, do general symptoms such as fever or fatigue appear. However, the course of the disease depends on the existing type. In the most common classic adult type, the disease begins in adulthood.
The symptoms subside on their own after a few years and only reappear in exceptional cases after a certain period of time. In the atypical adult type, the symptoms also appear in adulthood. However, they can persist for twenty years or more before disappearing. In the classic juvenile type, symptoms appear in adolescence and usually disappear within a year. They can appear here again later.
In the case of the circumscribed juvenile type, isolated symptoms appear on the palms of the hands, elbows, knees and soles of the feet, even in children, and these disappear again during puberty. The atypical juvenile type of the disease is genetic. It begins before birth and lasts indefinitely. HIV -associated PRP is difficult to treat.
Diagnosis & course of disease
Pityriasis rubra pilaris is similar to psoriasis and can usually only be diagnosed based on a skin biopsy. There are already indications of PRP if treatment of the suspected psoriasis is not successful.
In the case of pityriasis rubra pilaris, those affected primarily suffer from various skin complaints. These primarily have a very negative effect on the aesthetics of the patient and can significantly reduce them. In many cases, the patients also suffer from an inferiority complex or from a significantly reduced self-esteem. Bullying and teasing can also occur and have a negative effect on the psyche of the person concerned.
As a rule, those affected also suffer from severe itching on the skin and fever due to pityriasis rubra pilaris. The patient is also tired and exhausted. The symptoms themselves can also go away on their own. The symptoms usually subside during puberty.
Unfortunately, a causal treatment of pityriasis rubra pilaris is not possible. With the help of creams and ointments, however, the symptoms of the disease can be significantly alleviated. Usually there are no special complications. Light therapy is also possible and leads to a positive course of the disease. The life expectancy of those affected is not affected by the disease.
Treatment & Therapy
Pityriasis rubra pilaris cannot be cured with treatment. Most of the time, however, the symptoms go away on their own. However, if skin irritation persists, topical creams and ointments containing urea and lactic acid will help. This keeps the skin moist. Significant improvements in symptoms were sometimes observed when vitamin A was taken orally.
Drugs can also be used to slow down the growth of skin cells. The retinoids acitretin or isotretinoin have proven themselves for this. They are administered orally. UV light therapy with an additional drug treatment also promises good results.
Outlook & Forecast
The further course of pityriasis rubra pilaris is usually very dependent on when the disease is recognized and how severe it is in the person affected. For this reason, the person affected should ideally consult a doctor as early as possible and also have treatment initiated so that other symptoms or complications do not arise.
Pityriasis rubra pilaris cannot usually heal itself, so treatment by a doctor is always necessary. If the disease is not treated at all, there will be no improvement and the symptoms can also spread to other parts of the body and thus significantly reduce the quality of life of those affected.
The treatment of pityriasis rubra pilaris can significantly alleviate and limit the symptoms with the help of medication and various creams or ointments. However, a complete cure is not always possible, so that those affected keep falling ill with the disease. In general, a healthy diet in particular can have a positive effect on the further course of this disease and, in some cases, also prevent its recurrence. The life expectancy of the affected person is not reduced by this disease.
Since the causes of pityriasis rubra pilaris are not known, unfortunately no recommendations can be made for their prevention. In any case, the skin should also be examined for a possible risk of skin cancer after the occurrence of PRP.
In most cases, those affected by pityriasis rubra pilaris have only a few and no special direct aftercare measures available. Those affected should consult a doctor early on so that the disease does not lead to complications or other symptoms as it progresses. The sooner a doctor is contacted, the better the further course of the disease will usually be.
For this reason, those affected should ideally contact a doctor as soon as the first symptoms and signs of the disease appear. In some cases, the symptoms disappear on their own, so that no special aftercare is necessary or possible. However, in some cases, light therapy can alleviate the symptoms and improve the quality of life of those affected.
Various medications can also be taken to relieve the symptoms. Affected people should always pay attention to the correct dosage and also to taking the medication regularly. If you have any questions or are unclear, you should always contact a doctor first. In most cases, pityriasis rubra pilaris does not reduce the life expectancy of those affected and can be cured relatively easily.
You can do that yourself
Various, rarely occurring skin diseases are summarized under the term pityriasis rubra pilaris, but they are harmless in themselves. Nevertheless, those affected often suffer greatly from them. This is mainly due to the pustules, which also appear in visible areas of the body and sometimes severely disfigure the patients. Young patients may be bullied or teased, but older patients with pityriasis rubra pilaris may also lose confidence.
Psychotherapy or behavioral therapy is therefore recommended as an accompanying treatment. Many of those affected also found group therapy helpful because it enabled them to better understand and assess the reactions of those around them. In principle, patients suffering from pityriasis rubra pilaris should regularly care for their skin with the ointments prescribed for them. Oil baths, lotions containing urea and/or lactic acid and gentle peelings can also improve the complexion and reduce itching. Some patients have also had good experiences with UV light therapies.
A healthy diet is always helpful when dealing with skin diseases. Fatty and sugary convenience products and fast food in particular are often associated with skin lesions. It is therefore worth reaching for fresh foods rich in vitamins, as they support the healing process. Since the body also detoxifies through the skin, it is advisable to avoid toxins such as nicotine and alcohol.