Pityriasis lichenoides is one of the erythematosquamous skin diseases that can present in an acute or chronic form. It is an autoimmune disease with an unknown cause. Symptomatic treatment of skin papules is by anti-inflammatory local treatment.
What is pityriasis lichenoides?
The medical literature also refers to skin diseases as dermatoses. These diseases usually manifest not only on the skin, but also in its appendages. These include the hair, nails, sebaceous glands and sweat glands. Different subcategories are included under dermatoses. One of them is that of erythemato-squamous skin diseases. Pityriasis lichenoides is an erythemato-squamous skin disease first described in 1899. Juliusberg is considered to be the first describer. Brocq described the disease three years after the initial description as one of several parapsoriasis disorders. The speculation of multiple psoriasis-like diseases cannot be sustained based on recent research findings. Meanwhile, medicine assumes clinically different manifestations of pityriasis lichenoides, which are to be understood as variants of one and the same disease. Clinically, four forms are distinguished from each other so far, which are characterized by different acuity. The acute form of the disease is also known as Mucha-Habermann disease.
Causes
Pityriasis lichenoides is a rather rare disease. In most cases, male adolescents or adult males are affected by the dermatosis. The cause of the skin disease has not yet been clarified. Also speculations about causative factors are so far rather thinly sown, which probably has to do with the rare occurrence of the dermatosis. It is presumably an autoimmune disease. T-cells of the immune system cause inflammations in the skin area during the course of the disease. The immunological cells thus falsely recognize the body’s own tissue as an exogenous substance or pathogen to be fought. The primary cause of such immune system misprogramming could be a virus. In addition, vaccines and environmental factors such as periods of stress have been discussed as primary causes of pityriasis lichenoides. Why males suffer more frequently from the disease remains unclear to date. For the causal relationship, gender seems to play at least hardly any role.
Symptoms, complaints, and signs
An acute is distinguished from a chronic form of pityriasis lichenoides. In principle, both forms can also occur side by side in the same patient. The acute form (pityriasis lichenoides et varioliformis acuta or PLEVA) is the rarer variant and manifests as single or multiple papules that begin to necrotize hemorrhagically. Pain or an uncomfortable feeling of tightness accompanies the tissue-destructive necrosis. Exanthema is characteristic of the chronic form of the disease (pityriasis lichenoides chronica or PLC). These are large areas of uniform skin rash. In chronic pityriasis lichenoides, these rashes usually persist for several months and are accompanied by the formation of round or oval sharply demarcated papules. Most patients suffer only minor itching. On the other hand, in many cases their skin is covered by a parakeratotic cap of scales. Parakeratosis is a keratinization disorder of the squamous epithelium that leaves nuclei of epithelial cells in the uppermost cell layer.
Diagnosis and course of the disease
The diagnosis of pityriasis lichenoides is made on the basis of clinical symptoms. For a fine diagnosis and determination of the form of the disease, follow-up is indicated in addition to histopathology. In terms of differential diagnosis, the chronic form of pityriasis lichenoides in particular must be differentiated from psoriasis guttata and lues II. In the acute form, there is some similarity to chickenpox as well as drug exanthema and allergic vasculitis. Because the disease often first appears in early childhood, patients are most often misdiagnosed with chickenpox during their first acute attack. There is a rather favorable prognosis for those affected. Although the acute form presents clinically with a more severe picture, it usually goes into remission on its own and regresses completely.
Complications
In pityriasis lichenoides et varioliformis acuta, affected individuals suffer from various skin complaints. These complaints have a very negative effect on the quality of life of the affected person and can limit it considerably. In most cases, there are also strong psychological complaints and inferiority complexes or a reduced self-esteem. Children may also be bullied or teased. The skin itself is covered with papules and pustules and may itch in some cases. Children in particular often scratch their skin, which can lead to scarring or even bleeding. Most patients with pityriasis lichenoides et varioliformis acuta also suffer from a general feeling of illness and further also from fatigue and tiredness. Pityriasis lichenoides et varioliformis acuta is treated with the help of medications and various creams or ointments. No complications occur. However, whether the symptoms disappear completely as a result of the treatment cannot be universally predicted. However, the patient’s life expectancy is not affected by pityriasis lichenoides et varioliformis acuta.
When should you see a doctor?
If unusual skin changes occur that do not resolve on their own within a few days, medical help is needed. At the latest, when symptoms such as itching or inflammation are added, pityriasis lichenoides must be taken to the doctor. The diagnosis enables immediate treatment with medication, which should cause the pityriasis lichenoides to recede within a few days to weeks. Individuals suffering from chronic skin diseases or autoimmune disorders are particularly at risk. Likewise, patients of skin fungi such as Malassezia furfur belong to the risk groups and should have the mentioned symptoms clarified promptly. Also who stays in humid-warm climate, sweats strongly or has basic diseases like diabetes mellitus, must inform the family doctor. Further contact points are the dermatologist or a specialist in internal medicine. Treatment usually takes place on an outpatient basis. In the case of severe pityriasis lichenoides, inpatient therapy in a specialized clinic may be necessary. The patient may also need therapeutic support, as the disease can be a great emotional burden.
Treatment and therapy
A causal treatment option does not yet exist for patients with pityriasis lichenoides. Because the primary cause of the disease has not yet been determined, causal treatment approaches cannot be developed. For this reason, the chronic form of the dermatosis in particular is considered an incurable disease. Symptomatic treatment options are available. The treatment approaches used to date range from exposure to UV radiation to conservative drug therapies. The PUVA method, for example, resorts to UV. This is the combination of psoralenes with long-wave UV irradiation, which is used as a therapy in dermatology for various indications. Among the drug therapy routes, the use of antibiotics has been shown to be effective for patients with pityriasis lichenoides. The same is true for the provision of steroids. Systemic glucocorticoids are the most common therapeutic method. Another treatment option is anti-inflammatory local therapy of individual papules. In the acute form of the disease, the lesions disappear on their own in most cases. Depending on the individual course, this regression may take some time. In the chronic form, remissions are also common. In this variant, however, the symptoms disappear only for a certain period of time and then recur.
Outlook and prognosis
As a rule, the further course in pityriasis lichenoides is very much dependent on its exact cause and also on the severity of the disease, so that a general prediction cannot usually be made in this case. However, an early diagnosis by a physician has a very positive effect on the further course of this disease and can also prevent the occurrence of further complications or complaints. If pityriasis lichenoides is not treated at all, the symptoms do not disappear on their own and can also intensify in many cases.Therefore, treatment should always be sought for this disease. As a rule, various creams or ointments are used to alleviate the symptoms. Regular application of these medications can permanently and sustainably reduce the symptoms so that they do not recur. However, the duration of the healing process can vary greatly and also depends strongly on how well the medication works for the respective patient. The affected person should avoid scratching the affected areas in any case, so that bleeding or scars do not occur. Likewise, a high standard of hygiene can alleviate the discomfort and also prevent the recurrence of the disease.
Prevention
Preventive steps hardly exist for pityriasis lichenoides. As long as the primary cause of the disease is not clarified, no promising preventive measures can be developed. Since extreme stress situations appear to be a causative factor, the application of stress management strategies may play a role in prevention. Nonetheless, it is not a sure prevention strategy.
Follow-up
Affected individuals have very few and limited aftercare measures available in most cases of pityriasis lichenoides. As a rule, a physician should be contacted at an early stage to prevent further complications as well as other medical conditions. An early diagnosis usually has a very positive effect on the further course of the disease and can prevent a worsening of the symptoms. Therefore, the patient should see a doctor at the first symptoms and signs of the disease. Most of those affected by pityriasis lichenoides are therefore dependent on taking various medications. Here, it is always important to pay attention to a correct dosage with the regular intake in order to properly alleviate the symptoms. If antibiotics are taken, they should not be taken together with alcohol. Furthermore, those affected with pityriasis lichenoides should protect their skin particularly well against light and also not spend time in the sun without protection. Regular checks by a doctor help to detect and treat damage at an early stage.
What you can do yourself
This rather rare disease often affects men. Since no cause of the disease has yet been determined, it can only be treated symptomatically. Here, the treating dermatologist will suggest suitable therapies. However, it is possible that pityriasis lichenoides is an autoimmune disease, the course of which worsens depending on stress and environmental influences. Therefore, a healthy lifestyle is advised, which includes several components. Firstly, patients should not ingest any additional toxins, as they are also eliminated through the skin, which can aggravate the disease. Thus, they should not smoke, consume only moderate to no alcohol, and avoid stress. Exercise is important, because on the one hand it stimulates the metabolism and on the other hand it supports the immune system in the fight against pityriasis lichenoides. It also improves body awareness, which is particularly beneficial for patients who suffer from the visually disfiguring blisters. A careful diet with fresh foods rich in vitamins and low in fat and sugar is also part of a healthy lifestyle. Patients are advised to drink plenty of water to flush out any toxins. Detoxification measures can also strengthen the immune system. For this purpose, sauna visits are combined with naturopathic or homeopathic remedies. Naturopathic physicians as well as alternative practitioners are happy to advise here.
