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What is the history of Pityriasis lichenoides et varioliformis acuta?

When was Pityriasis lichenoides et varioliformis acuta discovered? What is the story of this discovery? Was it coincidence or not?

History of Pityriasis lichenoides et varioliformis acuta

Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare skin disorder characterized by the development of small, red, scaly papules that may progress to form vesicles or pustules. The condition was first described by French dermatologist Louis-Anne-Jean Brocq in 1901. PLEVA is also known as Mucha-Habermann disease, named after the dermatologists who further contributed to its understanding.



Early Observations:



The history of PLEVA dates back to the late 19th century when Brocq initially observed the skin lesions in a young boy. He described the condition as "lichen ruber acuminatus" due to its resemblance to lichen planus. However, it was later recognized as a distinct entity.



Further Research and Classification:



In 1903, Mucha and Habermann independently reported cases similar to Brocq's observations. They emphasized the acute nature of the disease and its association with fever. Their contributions led to the alternative name "Mucha-Habermann disease."



Over the years, researchers have made significant efforts to understand PLEVA and its classification. In 1953, Gougerot and Burnier proposed a classification system that divided PLEVA into two forms: PLEVA and its more severe variant, pityriasis lichenoides chronica (PLC). This classification was based on the duration and clinical course of the disease.



Etiology and Pathogenesis:



The exact cause of PLEVA remains unknown. Various theories have been proposed, including infectious agents, immunological factors, and genetic predisposition. Some studies suggest a possible association with viral infections, such as Epstein-Barr virus and human herpesvirus 7, but no definitive link has been established.



The pathogenesis of PLEVA involves an abnormal immune response. It is believed that activated T-cells play a crucial role in the development of skin lesions. These T-cells release cytokines and cause inflammation, leading to the characteristic papules and vesicles.



Clinical Presentation and Diagnosis:



PLEVA typically affects children and young adults, with a peak incidence between 5 and 15 years of age. The condition presents as crops of red, scaly papules that may evolve into vesicles or pustules. The lesions are usually distributed symmetrically on the trunk and extremities.



Diagnosing PLEVA can be challenging due to its similarity to other skin conditions. A skin biopsy is often necessary to confirm the diagnosis. Histopathological examination reveals a dense infiltrate of lymphocytes and histiocytes in the upper dermis.



Treatment and Prognosis:



PLEVA is a self-limiting condition, with most cases resolving spontaneously within months to years. However, treatment may be required to alleviate symptoms and prevent complications. The choice of treatment depends on the severity of the disease and individual patient factors.



Topical corticosteroids, phototherapy, and systemic antibiotics are commonly used treatment options. Severe cases may require systemic corticosteroids or immunosuppressive agents. Regular follow-up is essential to monitor the progression of the disease and adjust treatment accordingly.



Conclusion:



Pityriasis lichenoides et varioliformis acuta, or Mucha-Habermann disease, is a rare skin disorder characterized by the development of red, scaly papules that may progress to vesicles or pustules. Although the exact cause remains unknown, it is believed to involve an abnormal immune response. The disease typically affects children and young adults and is self-limiting in most cases. Treatment aims to alleviate symptoms and prevent complications. Further research is needed to fully understand the etiology and pathogenesis of PLEVA.


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