The Stevens-Johnson syndrome (erythema multiforme or exsudativum ectodermosis erosiva pluriorificialis dermatostomatite or erosive or ectodermose pluriorificielle of Fiessinger-Rendu) is an orphan disease severe, sudden onset, and potentially lethal (15.
It is characterized by a plurality of symptoms, affecting or not be associated.
The first symptom is usually a violation of multiple mucous membranes (mouth, throat, nose, eyes, penis, vulva and / or anus).
Quickly appear polymorphic skin lesions called cockade lesions (macule erythemal circular lesions in English target = target lesions) that can cover most of the skin but are concentrated at the palms. These lesions begin as macules and can become papules, vesicles, hives or purpuric plaques.
There are also bullous and erosive lesions, which can bleed and be painful, mucous membranes at some natural orifices such as the nostrils and mouth, genital mucosa and conjunctiva. The bubbles can reach and thus result in a more or less extensive detachment of the skin.
The internal organs are sometimes affected. Pneumonias are frequently associated with the clinical picture by bronchial mucosal involvement.
In the case of Stevens-Johnson syndrome, the surface of the skin involved by disease is less than 10%. The most extreme cases are called toxic epidermal necrolysis syndrome or toxic epidermal necrolysis.
Drugs identified risks are.
This syndrome is also a possible complication of Mycoplasma pneumoniae in lung diseases.
There is currently no cure against this disease.
Nonetheless, certain steps can be taken.
The ocular sequelae, which affects more than half of survivors are due to severe damage of the cornea and conjunctiva and gradually degrade vision, often blindness. They currently represent one of the greatest challenges in ophthalmology. Reconstructive surgery is powerless to date. They result in a severe and painful photophobia (forcing the port of sunglasses) and a pronounced dry eye (instillation requiring regular, sometimes every 5 minutes, artificial tears).
Phobia drugs, understandably, is another sequel of these syndromes, which can seriously hinder the subsequent treatment of diseases. Other effects are.
This syndrome is named after two U.S. pediatricians, Albert Mason Stevens and Frank Chambliss Johnson. Whereas in France this condition was described by Henri Rendu and Fiessinger.
Trombidiose Dermographism Porphyria