The skin disease pityriasis lichenoides et varioliformis acuta (PLEVA) is a progressive form of pityriasis lichenoides. In this disease, small-spotted papules form, especially in the trunk area, which are associated with itching or burning in individual cases. Local treatment is with anti-inflammatory creams, such as glucocorticoid creams.
What is pityriasis lichenoides et varioliformis acuta?
Skin lesions can take several forms. One form of skin lesion is the papule. This is a round or oval nodule that is less than five millimeters in diameter. Papules are raised above the skin surface, in some cases have peduncles, and may be plateau-shaped. Both the color and consistency of the nodules can vary. This type of skin lesion is classified as a primary efflorescence, which usually corresponds to the direct sequela of a specific primary disease. For example, in the case of papules, the primary disease may be pityriasis lichenoides. Different forms of this disease exist. In addition to an acute form, a chronic form may be present. The transition between acute and chronic form is called Pityriasis lichenoides et varioliformis acuta, in short PLEVA. 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 descriptors. In their honor, this form of the disease 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. The male sex is more frequently affected than the female.
Causes
The exact etiology for pityriasis lichenoides has not yet been determined. Thus, the exact causes of the disease are also unclear. For the inflammatory course of pityriasis lichenoides et varioliformis acuta, an infectious-allergic genesis is suspected. Both PLC and PLEVA belong to the lymphoproliferative T-cell disorders. An immunologic response is therefore involved. For this reason, all courses of pityriasis lichenoides are thought to be infectious-allergic diseases, and different triggers may be involved in their development. Apparently, bacteria are possible factors, especially hemolytic streptococci. On the other hand, the disease may also be rooted in drug-allergic reactions or be related to the influence of viruses. In the case of viral genesis, the herpes zoster virus probably plays a role. In addition, the Epstein-Barr virus is apparently relevant. Meanwhile, scientists also discuss a proximity of the skin disease to T-cell lymphomas, since a monoclonal rearrangement of T-cell receptors can be detected in half of all patients.
Symptoms, complaints, and signs
Patients with PLEVA suffer from skin lesions that are localized primarily in the trunk. More commonly, the lesions form within the first two decades of life. Infants and young children are rarely affected. The average age of manifestation is around eight years of age. The skin lesions correspond to polymorphous pruritic or burning exanthemas with acute onset and a size between 0.2 and 0.4 centimeters. Papules also form, as well as erosions, ulcers, and hemorrhagic vesicles. After the lesions heal, varioliform scars remain. In rare cases, affected individuals suffer from fever with severe general symptoms during disease manifestation. In addition, disseminated crusting ulcerations occur in isolated cases. Complications of the disease occur primarily in adulthood, but are rare. If complications do occur, a lethal course is possible. A subtype of PLEVA is highly febrile ulceronecrotic pityriasis lichenoides with hyperthermia and ulcers, also known as PLUH. This variant is associated with more severe disease.
Diagnosis and disease course
The dermatologist makes the diagnosis of pityriasis lichenoides et varioliformis acuta by histology. Different diseases must be differentially diagnosed. These diseases include varicella, drug exanthema, tuberculid, 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 course is considered an extremely rare phenomenon. Young patients in particular are usually cured. Individual relapses usually last no more than two weeks. However, scars may remain after healing.
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 sometimes 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 thanks to 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?
Because pityriasis lichenoides et varioliformis acuta is an acute condition 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 affected person suffers from skin complaints. There may be the appearance of papules and pustules on the skin, which are associated with severe itching. The affected person does not feel well and often suffers from fever or general fatigue. Similarly, general symptoms of flu together with the skin complaints may indicate pityriasis lichenoides et varioliformis acuta. Therefore, if these symptoms are prolonged, a physician should be consulted in any case. Pityriasis lichenoides et varioliformis acuta can be diagnosed by a dermatologist or by a general practitioner. The disease does not negatively affect the life expectancy of the affected person and can usually be treated relatively well.
Treatment and therapy
Patients with PLEVA are treated with lotio alba or glucocorticoid creams during the acute phase. Phototherapeutic measures can be pursued beyond this local treatment. In addition to UVB therapy, UVA1 therapy has also proven effective in the context of the disease. In principle, phototherapeutic PUVA therapy can also be used for treatment. If recurrences occur, internal therapy is usually given. Patients with bacterial infection receive broad-spectrum antibiotics in combination with glucocorticoid administration. Severe itching is treated with systemic antihistamines. Overall, local treatment of the condition 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 antimicrobials to kill potentially causative bacteria. Thus, PLEVA is treated causally as well as symptomatically. Almost all treatment approaches are conservative drug approaches. Patients should also avoid contact with substances that could cause skin irritation as a supportive measure. In addition, special hygiene measures should be followed during an acute episode.
Outlook and prognosis
The prognosis of pityriasis lichenoides et varioliformis acuta is described as favorable. Normally, if medical and drug therapy is used, recovery is possible within a few weeks. If there are no other diseases of the skin or intolerance to ingredients of the prescribed drugs, a significant alleviation of symptoms is achieved within a few days. The prerequisite for this is a fundamentally healthy general condition and the immediate professional treatment of the symptoms.In case of allergic reactions to the prescribed drugs, healing delays are to be expected. In addition, the course of the disease is more difficult in patients with pre-existing conditions. Old and weakened people show in some cases a chronic course of the skin disease. In addition, scars are documented in the case of a delayed start of treatment or a very intensive disease. These can be treated in individual cases in a further therapy by laser. If no medical care is used, a spreading of the complaints is to be expected. In addition, psychological irregularities and fatigue become apparent. The risk of scarring is also significantly increased. If psychological complications occur, the overall prognosis is worse. Often, the course of recovery in these patients spans several months or years. Nevertheless, recovery is possible. If a new onset of disease occurs after a cure in the further course of development, the prognosis generally remains favorable.
Prevention
The causes of pityriasis lichenoides et varioliformis acuta have not yet been conclusively determined. Various correlations are under discussion. Preventive measures can only be available to the extent that the pathogenesis can be understood. Therefore, no promising preventive measures exist for PLEVA to date.
Follow-up
Affected individuals have few options for direct follow-up in pityriasis lichenoides et varioliformis acuta. At the same time, the patient should see a doctor as early as possible in this disease to avoid further complications or other complaints. The earlier a doctor is consulted and treatment is initiated, the better the further course of the disease usually is. The treatment of pityriasis lichenoides et varioliformis acuta is usually carried out with various medications, creams or ointments. The affected person should use these medications regularly and dose them correctly. In case of questions or uncertainties, a doctor should always be consulted first to avoid side effects or other complications. Likewise, the affected person should regularly visit a dermatologist and have the symptoms checked so that changes on the skin can be detected and treated at an early stage. In general, high hygiene measures should be observed in pityriasis lichenoides et varioliformis acuta so that inflammation and infection can be prevented. Pityriasis lichenoides et varioliformis acuta does not usually reduce the life expectancy of the affected person.
What you can do yourself
Pityriasis lichenoides et varioliformis acuta can be treated specifically with ointments and creams after medical diagnosis. This improves the skin symptoms, which are often accompanied by a reduction in quality of life. The pustules and papules on the skin make young patients in particular feel uncomfortable. In addition, there may 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. 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 sufferers the feeling that they are not alone. In addition to creams for the damaged skin, there are other medications against the typical physical symptoms. Furthermore, patients should not expose themselves to too much stress. This can aggravate the symptoms. In any case, increased fatigue is to be expected, even if no complications occur during the treatment itself.
