The skin disease pityriasis lichenoides et varioliformis acuta (PLEVA) is a progressive form of pityriasis lichenoides. In this disease, small patchy papules form, especially in the area of the trunk, which are associated with itching or burning in individual cases. Local treatment is carried out with anti-inflammatory creams, such as glucocorticoid creams.
What is pityriasis lichenoides and varioliformis acuta?
Skin lesions can take different forms. One form of skin lesion is the papule. It is a round or oval nodule less than five millimeters in diameter. Papules are raised above the skin surface, sometimes have peduncles, and may be plateau-like. See definitionexplorer for Testicular Atrophy Definitions.
Both the color and consistency of the nodules can vary. This type of skin lesion is counted among the primary efflorescences, which usually correspond to the direct consequence of a specific primary disease. In the case of papules, the primary disease can be pityriasis lichenoides, for example act. There are different forms of this disease. In addition to an acute form, a chronic form may be present.
The transition between the acute and chronic form is called pityriasis lichenoides et varioliformis acuta, PLEVA for short. This progressive form of the skin disease was first described at the beginning of the 20th century. Mucha and Habermann are considered to be the first to describe it. In their honor, the progressive form is also referred to as Mucha-Habermann syndrome. The disease manifests itself more frequently in the first two decades of life with the exception of infancy. Males are affected more often than females.
Causes
The exact etiology of pityriasis lichenoides has not yet been clarified. The exact causes of the disease are also unclear. An infection-allergic genesis is suspected for the inflammatory progression of pityriasis lichenoides et varioliformis acuta. Both PLC and PLEVA belong to the lymphoproliferative T-cell diseases.
An immunological reaction is therefore involved. For this reason, all forms of pityriasis lichenoides are considered to be infectious-allergic diseases, the development of which can be caused by different factors. Apparently, bacteria are possible factors, most notably hemolytic streptococci. On the other hand, the disease can also be rooted in drug-allergic reactions or related to the influence of viruses.
In the case of viral genesis, the herpes zoster virus in particular presumably plays a role. In addition, the Epstein-Barr virus is apparently relevant. Scientists are now also discussing whether the skin disease is related to T-cell lymphomas, since a monoclonal rearrangement of T-cell receptors can be detected in half of all patients.
Symptoms, Ailments & Signs
Patients with PLEVA suffer from skin lesions, which are mainly localized in the trunk area. The lesions form more frequently within the first two decades of life. Babies and young children are rarely affected. The average age of onset is around eight years of age.
The skin lesions correspond to polymorphic itchy or burning exanthems of acute onset and ranging in size from 0.2 to 0.4 centimeters. In addition, papules, erosions, ulcers and hemorrhagic vesicles form. After the lesions have healed, varioliform scars remain. In rare cases, those affected suffer from fever with severe general symptoms during the manifestation of the disease.
In addition, disseminated, crust-forming ulcerations occur in isolated cases. Complications of the disease occur mainly in adulthood, but are rather rare. If complications arise, it can be fatal. A subtype of PLEVA is the highly febrile ulcer-necrotic pityriasis lichenoides with hyperthermia and ulcers, also known as PLUH. This variant is associated with a more severe feeling of illness.
Diagnosis & course of disease
The dermatologist uses histology to diagnose pityriasis lichenoides et varioliformis acuta. Different diseases must be differentiated in the differential diagnosis. These diseases include varicella, drug eruptions, tuberculide, and acute and subacute eczema. Psoriasis guttata, pityriasis rosea and early syphilis are also considered differential diagnoses.
The prognosis for patients with pityriasis lichenoides et varioliformis acuta depends on the severity of the course. A complete cure is just as conceivable as a lethal course. However, the lethal form is considered an extremely rare phenomenon. Especially young patients are usually cured. Individual flare-ups usually last a maximum of two weeks. However, scars may remain after healing.
Complications
In the case of pityriasis lichenoides et varioliformis acuta, those affected suffer from various skin complaints. These complaints have a very negative effect on the quality of life of those affected and can limit it considerably. In most cases, this also leads to severe psychological problems and inferiority complexes or reduced self-esteem.
Children may also be bullied or teased. The skin itself is covered with papules and pustules and can also be itchy in some cases. Children in particular scratch their skin very often, which can lead to scars or even bleeding. Most patients with pityriasis lichenoides et varioliformis acuta also suffer from a general feeling of illness and sometimes from exhaustion and fatigue.
Pityriasis lichenoides et varioliformis acuta is treated with the help of medication and various creams or ointments. There are no complications. However, it cannot be universally predicted whether the symptoms will disappear completely thanks to the treatment. However, the life expectancy of the patient is not affected by pityriasis lichenoides et varioliformis acuta.
When should you go to the doctor?
Since pityriasis lichenoides et varioliformis acuta is an acute disease that does not heal on its own, a visit to a doctor is always necessary. This is the only way to avoid further complications. The doctor should be consulted if the person concerned suffers from skin problems.
Papules and pustules may appear on the skin, which are associated with severe itching. The affected person no longer feels well and often suffers from fever or general fatigue. Likewise, general symptoms of influenza together with the skin complaints can indicate pityriasis lichenoides et varioliformis acuta. If these symptoms persist for a longer period of time, you should definitely consult a doctor.
Pityriasis lichenoides et varioliformis acuta can be diagnosed by a dermatologist or by a general practitioner. The disease does not have a negative effect on the life expectancy of those affected and can usually be treated relatively well.
Treatment & Therapy
Patients with PLEVA are treated with lotio alba or glucocorticoid creams in the acute phase. In addition to this local treatment, phototherapeutic measures can be pursued. In addition to UVB therapy, UVA1 therapy has also proven itself in the context of the disease. In principle, phototherapeutic PUVA therapy can also be used for treatment.
If recurrences occur, internal therapy is usually carried out. Patients with bacterial infection receive broad -spectrum antibiotics in combination with glucocorticoids. Severe itching is treated with systemic antihistamines. Overall, local treatment of the disease focuses on anti-inflammatory agents to control inflammation in the affected area and thus limit skin damage.
Internal therapies, on the other hand, rely on antimicrobial agents to kill potentially causative bacteria. In this way, PLEVA is treated causally as well as symptomatically. Almost all treatment approaches are conservative drug approaches. Patients should also avoid contact with materials that may cause skin irritation as a supportive measure. In addition, special hygiene measures must be observed during an acute flare-up.
Outlook & Forecast
The prognosis of pityriasis lichenoides et varioliformis acuta is described as favorable. Normally, recovery is possible within a few weeks if medical and drug therapy is used. If there are no other diseases of the skin or intolerance to the ingredients of the prescribed medication, a significant alleviation of the symptoms is achieved within a few days. The prerequisite for this is a fundamentally healthy general condition and the immediate professional care of the symptoms.
Delays in healing are to be expected in the case of allergic reactions to the prescribed medicines. In addition, the course of the disease is more difficult in patients with previous illnesses. In some cases, old and weakened people show a chronic course of the skin disease. Scars that remain after a delayed start of treatment or a very intensive illness are also documented. In individual cases, these can be treated in a further therapy with laser.
If no medical care is used, the symptoms can be expected to spread. In addition, there are mental irregularities and exhaustion. The risk of scarring is also significantly increased. If psychological complications occur, the overall prognosis is worse. The healing process in these patients often takes several months or years. Still, recovery is possible. If, after healing, the disease breaks out again in the further course of development, the prognosis is generally unchanged.
Prevention
The causes of pityriasis lichenoides et varioliformis acuta have not yet been finally clarified. Various connections are up for discussion. Preventive measures can only be available to the extent that the pathogenesis can be understood. Therefore, there are no promising preventive measures for PLEVA to date.
Aftercare
Those affected with pityriasis lichenoides et varioliformis acuta have only a few options for direct aftercare. The patient with this disease should consult a doctor as early as possible so that there are no further complications or other symptoms. The sooner a doctor is consulted and treatment initiated, the better the further progression of the disease.
In most cases, acute pityriasis lichenoides et varioliformis is treated with various medications, creams or ointments. The person concerned should use these medications regularly and dose them correctly. If you have any questions or are unclear, you should always consult a doctor first to avoid side effects or other complications.
The person concerned should also visit a dermatologist regularly and have the symptoms checked so that changes on the skin can be recognized and treated at an early stage. In general, high hygiene measures should be observed in acute pityriasis lichenoides et varioliformis so that inflammation and infection can be prevented. Pityriasis lichenoides et varioliformis acuta usually does not reduce the life expectancy of those affected.
You can do that yourself
Pityriasis lichenoides et varioliformis acuta can be treated with ointments and creams after the medical diagnosis. This improves the skin complaints, which are often associated with a reduction in quality of life. The pustules and papules on the skin make especially young patients uncomfortable.
There may also be some itching. Those affected must not give in to this under any circumstances, otherwise the situation will worsen. This can lead to scars or bleeding spots. It is all the more important to be well informed about the disease and to follow the doctor’s recommendations.
If psychological problems and even inferiority complexes are caused by the skin problems, therapy can help. Above all, contact with other patients gives those affected the feeling that they are not alone. In addition to the creams for damaged skin, there are other medications for the typical physical symptoms. Furthermore, patients should not expose themselves to excessive stress. This can make the symptoms even worse. In any case, increased fatigue is to be expected, even if no complications arise during the treatment itself.
