Erythropoietic Protoporphyria. A Major Skin Involvement
Journal Title: Clinical Dermatology Open Access Journal - Year 2017, Vol 2, Issue 4
Abstract
Erythropoietic Protoporphyria (EPP) is a relatively rare disease [1], probably less so than the rare acute intermittent porphyria. Virtually all patients with EPP are symptomatic as young children. For example, a 10-year old boy was referred to me, a porphyria specialist, because of cutaneous photosensitivity since infancy. With sun exposure, his skin quickly developed tingling and subsequently swelling, petechiae, burning, stinging, erythema and pain, and rarely potential lethal liver dysfunction. In general, affected patients, including this boy, avoid being outside or with the sun. When outside, they often wear total-body clothes to avoid body exposure to sun and/or heat. Ultimately, they lead, without treatment, to scarring and resorption of the nose, auricles and fingers. The few physicians who specialize in the porphyrias do encounter them relatively frequently. I have seen many youngsters and adults with this syndrome, and have advised and treated them regarding probably preventive EPP therapy.
Authors and Affiliations
Tishler PV
Keywords
Related Articles
- EP ID EP288559
- DOI 10.23880/cdoaj16000121
- Views 109
- Downloads 0
The Foreword
Every skin disease is an enigma. Dermatology is a very vast field in the Medicine. The human skin measures 2.2 m2. Whatever pathology happens in the human organ or system, beit respiratory, hepatic, digestive, renal or t...
Study of the Knowledge, Attitudes and Behavioral Practices of Bangui University Students in the Field of HIV / AIDS, Central African Republic
Introduction: HIV infection mostly affects young people between the ages of 15 and 25. The purpose of this study was to study the knowledge of infection among students and their behavior towards the pandemic. Materials...
A Case Report on Cetirizine Induced Urticaria
Cetirizine, anti-histamine is a widely used OTC medication for the treatment of hypersensitivity reactions like urticaria. Cetirizine induced hypersensitivity reactions are rarely reported. Here is a case of cetirizine i...
Epidemiological and Clinical Studies of the Chronic Leuts of the Legs at the Friendship Hospital of Bangui, Central African Republic
Introduction: The purpose of this work was to describe the epidemiological and clinical aspects of leg ulcers in Bangui, Central African Republic. Material and methods: This was a descriptive cross-sectional study from...
Angiolymphoid Hyperplasia with Eosinophilia: A Study of Two Cases
Objective: Angiolymphoid hyperplasia with eosinophilia (ALHE), is presented by multiple pink or red papules and nodules on the head, neck, preauricular area and anterior hairline. Although the lesions are usually asympto...