Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta of the eyelid: a rare presentation
Author: Shantanu Bajaj
Base Hospital / Institution: Hull University Teaching Hospitals
ePoster presentation
Abstract ID: 25-507
Purpose
to describe a rare presentation of Ulceronecrotic Pityriasis Lichenoides et Varioliformis Acuta affecting left upper eyelid in a patient with a history of previously undiagnosed recurrent necrotic ulcerating skin lesions on limbs and torso, and review of current treatments.
Methods
65 year old patient referred to a tertiary Oculoplastics centre with an acute history of a painful, ulcerating and necrotic upper eyelid. There was no history of trauma and he was systemically well. Initially treated with systemic antibiotics, but review of past medical history revealed recurrent episodic ulcerating necrotic lesions in digits, limbs and torso.
Results
a 3mm eyelid biopsy was perform but didn’t demonstrate anything other than non specific inflammation. However a much more sizeable biopsy from a digit during a subsequent attack clenched the diagnosis. The extensive necrotic appearance healed without loss of tissue or significant contracture, be it with resulting madarosis. He was started on long-term Methotrexate which has so far prevented any further lesions from recurring.
Conclusion
Ulceronecrotic pityriasis lichenoides et Varioliformis, also known as ulceronecrotic Mucha-Habermann disease is a rare and sometimes severe condition. It is characterised by rapid development of macules and papules that swiftly transform into ulcerated and necrotic lesions. The diagnosis is primarily clinical, but a skin biopsy can be helpful to confirming the condition, athough skip lesions often exist and a large sample size is likely to yield more positive results, obtained during an acute stage of presentation. Owing to its rarity and variability of manifestation it can be particularly challenging to diagnose, hence a multidisciplinary approach with dermatology input is cardinal. There is no standard accepted treatment but, antibiotics and systemic steroids are used acutely, with systemic immunosuppressants for long-term management. Surgery should be avoided, aside from the initial biopsy, as necrotic lesions often heals well with medical treatment alone, and surgical trauma will negatively affect the clinical course.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Egle | Rostron | Hull University Teaching Hospitals |
| Yahya | Khedr | Hull University Teaching Hospitals |
| James | Britton | Hull University Teaching Hospitals |
| Colin | Vize | Hull University Teaching Hospitals |
