PLEVA: the great masquerader

• Main Authors: Abd Rahman, Mohd Aizuddin, Jamani, Nurjasmine Aida, Zainun, Norsafina
• Format: Article
• Language: English
• Published: Faculty of Medicine, UKM 2023
• Subjects: R Medicine (General); RL Dermatology
• Online Access: http://irep.iium.edu.my/109203/1/110119_PLEVA.pdf; http://irep.iium.edu.my/109203/; https://www.medicineandhealthukm.com/article/international-virtual-medical-research-symposium

Introduction: The rare benign dermatosis Pityriasis lichenoides et varioliformis acuta (PLEVA) is mostly found in children and young adults. It has uncertain etiopathogenesis. The largest series of children with pityriasis lichenoides had a median PLEVA onset of 60 months, with a slight male predominance. The hallmark signs of the disease are the presence of acute inflammatory papules and papulovesicles with hemorrhagic or necrotic crusts on the trunk and flexural areas. Recurrence and remittance complicate rash diagnoses, especially in primary care. PLEVA mimics lymphomatoid papulosis, varicella, pityriasis rosea, guttate psoriasis, and secondary syphilis. PLEVA rarely causes febrile ulceronecrotic MuchaHabermann syndrome (FUMHD), which can be fatal. Case report: A 21-year-old man with vitiligo and metabolic syndrome had a one-month vesicular eruption, recurrent erythematous macules, and crusted patches on the trunk, extremities, and scalp. He received systemic and local antibiotics at multiple clinics without improvement. Upon assessment, he appeared well with stable vital signs. There were generalised brownish scales, with patches seen mainly on the trunk and all flexural areas, mixed with newer erythematous macules, papules, and dried-up blisters with necrotic and a few hemorrhagic at the centre of the crust on the skin, especially at the limbs. After a provisional diagnosis of PLEVA, he was referred to a dermatologist for a skin biopsy. Histopathological examination (HPE) of lichenoid dermatitis confirmed PLEVA. The lesions resolved after two months of treatment with oral and topical corticosteroids administered in tapering doses. Conclusion: The lack of consensus on PLEVA’s classification, etiopathogenesis, diagnosis, and treatment makes it a medical challenge. Clinical suspicion and histopathology confirm the diagnosis.