Pityriasis Rubra Pilaris: Causes, Symptoms & Treatment

Pityriasis rubra pilaris (PRP) is a very rare skin disease, which is often confused with psoriasis. Despite similar symptoms, this syndrome is a group of different skin diseases that have nothing to do with each other. Usually, pityriasis rubra pilaris heals on its own.

What is pityriasis rubra pilaris?

Pityriasis rubra pilaris represents a collective term for different skin diseases with the same symptoms. The cause may be a gene mutation in rare cases. In most cases, however, the cause is unclear. Pityriasis rubra pilaris is also called prickly lichen or Devergie’s disease. It was first described in 1856 by the Parisian dermatologist Marie Guillaume Devergie. The disease is very rare. In medical literature, it is given a probability of 1 in 1000,000 and 1 in 500,000, respectively. 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 originates from the hair follicles. The skin irritations appear patchy, with a smooth transition to a flat rash (erythroderma). In most cases, the disease occurs between the ages of 40 and 60.

Causes

The causes of pityriasis rubra pilaris are unknown in most cases. Originally, the disease was thought to be uniform and hereditary. Today it is known that only about five percent of all cases are clearly due to hereditary causes. Mutations of the CARD14 gene at gene locus 17q25.3 are thought to be responsible for the disease. The mode of inheritance is autosomal dominant. However, sporadic cases also occur. However, this gene mutation causes an atypical juvenile form of the disease. Furthermore, in the case of dominant inheritance of this gene, the disease should not always break out. Rather, it has been found 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 considerably 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 unknown. Some research suggests an abnormality in the processing of vitamin A as the cause of pityriasis rubra pilaris. However, an immune system mismatch has also been considered.

Symptoms, complaints, and signs

Pityriasis rubra pilaris is manifested by bright red, pointed nodules on the trunk, head, and the extensor sides of the extremities. Furthermore, there are scaly erythema on the skin. The nodules usually begin at the hair follicles. There is normal skin between the nodules, so the skin irritations appear patchy. The affected areas of skin feel rough and give a rubbing sensation. The skin also shows increased callus formation. On the palms of the hands, in addition to the increased callus formation, there are also cleft-like tears in the skin (rhagades). There is constant moderate itching. In the acute phase, there may be a general reddening of the skin. 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 does not begin until adulthood. The symptoms subside on their own after a few years and reappear only in exceptional cases after a certain period of time. In the atypical adult type, the symptoms also appear in adulthood. However, they may persist for twenty years or more before disappearing. In the classic juvenile type, symptoms appear in adolescence and usually disappear within a year. Again, they may reappear later. In the circumscribed juvenile type, isolated symptoms on the palms, elbows, knees, and soles of the feet already occur in children and resolve during puberty.The atypical juvenile type of the disease is genetic. It begins before birth and persists indefinitely. HIV-associated PRP is difficult to treat.

Diagnosis and disease progression

Pityriasis rubra pilaris resembles psoriasis and usually can be diagnosed only by skin biopsy. Indications of PRP already arise when treatment of the presumed psoriasis is unsuccessful.

Complications

In 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, patients also suffer from inferiority complexes or a significantly reduced self-esteem. Bullying and teasing can also occur and have a negative effect on the psyche of the affected person. As a rule, those affected also suffer from severe itching on the skin due to pityriasis rubra pilaris and furthermore also from fever. The patient also suffers from fatigue and exhaustion. The symptoms themselves can also disappear on their own. Especially during puberty, the symptoms subside. Unfortunately, a causal treatment of pityriasis rubra pilaris is not possible. However, with the help of creams and ointments, the symptoms of the disease can be significantly alleviated. As a rule, no particular complications occur. Light therapy is also possible and leads to a positive course of the disease. The life expectancy of the affected person is not affected by the disease.

Treatment and therapy

Pityriasis rubra pilaris cannot be cured by treatment. However, the symptoms usually disappear on their own. However, if skin irritation persists, topical creams and ointments containing urea and lactic acid will help. This keeps the skin moist. When vitamin A is taken orally, significant improvements in symptoms have sometimes been observed. Furthermore, drugs can be used to slow down the growth of skin cells. The retinoids acitretin or isotretinoin have proven effective for this purpose. They are administered orally. UV light therapy with additional drug treatment also promises good success.

Outlook and prognosis

The further course of pityriasis rubra pilaris is usually very dependent on when the disease is recognized and how severe it is in the affected person. For this reason, the affected person should ideally see a doctor as early as possible and also have treatment initiated so that other complaints or complications do not arise. As a rule, self-healing cannot occur with pityriasis rubra pilaris, so that treatment by a doctor is always necessary. If the disease is not treated at all, there will be no improvement and the symptoms may spread to other parts of the body, thus considerably reducing the quality of life of the affected person. The treatment of pityriasis rubra pilaris can significantly alleviate and limit the symptoms with the help of medications and various creams or ointments. However, a complete cure is not always possible, so that the affected person will always come down with the disease again. In general, a healthy diet in particular can have a positive effect on the further course of this disease and in some cases even prevent its recurrence. The life expectancy of the affected person is not reduced by this disease.

Prevention

Unfortunately, since the causes of pityriasis rubra pilaris are unknown, no recommendations can be made for its prevention. In any case, after the appearance of PRP, the skin should also be examined for a possible risk of skin cancer.

Aftercare

Affected individuals have few and no special measures of direct aftercare available in most cases of pityriasis rubra pilaris. However, affected individuals should see a doctor early on to prevent complications or other symptoms from developing as the disease progresses. The earlier a doctor is contacted, the better the further course of the disease usually is. For this reason, affected persons should ideally contact a doctor at the first symptoms and signs of the disease.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 the affected person. Various medications can also be taken to alleviate the symptoms. Patients should always ensure that the correct dosage is used and that the medication is taken regularly. In case of questions or uncertainties, a doctor should always be contacted first. Pityriasis rubra pilaris does not reduce the life expectancy of the affected person in most cases and can be cured relatively well again.

What you can do yourself

Various rarely occurring skin diseases are grouped 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 their self-confidence. Therefore, psychotherapy or behavioral therapy is recommended as adjunctive treatment. Many affected people also found group therapy helpful, because it helped them to better understand and assess the reactions of their peers. In principle, patients suffering from pityriasis rubra pilaris should care for their skin regularly with the ointments prescribed for them. Oil baths, lotions containing urea and/or lactic acid, and gentle peels can also improve the skin’s appearance and reduce itching. Some patients have also had good experiences with UV light therapies. A healthy diet is always helpful in skin diseases. Especially fat- and sugar-containing convenience products and fast food are often associated with skin lesions. Therefore, it is worthwhile to reach for vitamin-rich, fresh foods, because they support the healing process. Since the body also detoxifies through the skin, among other things, it is advisable to avoid toxins such as nicotine and alcohol.