The Steven Johnson Syndrome (SJS) is one of the reactionals dermatitis and is a true dermatological emergency, which could even cause the death of the patient.
SJS is an acute inflammatory disease secondary to a hypersensitivity reaction that affects the skin and mucous membranes of the body, and represents one of the main complications of major erythema multiforme, with the other severe complication being toxic epidermal necrolysis.
This syndrome is clinically characterized by having a sudden onset, with the presence of a dermatosis that covers 10% of the body surface, and tends to generalize, mainly affects the face, hands, feet and trunk; and the initial lesion is a pleomorphic papulo erythematosus rash that can progress to phlyctenas (blisters / ampoules), erosive lesions, ulcers, crusts, petechiae and even purples.
Another of the main characteristics is that it affects the oral, nasal, conjunctival (eyes), anal and genital mucosa.
Stomatitis (presence of aphthae throughout the oral mucosa) is a precocious symptom and evolves with the appearance of phlyctenas (blisters) on the lips, tongue and the rest of the oral mucosa; being able to form pseudomembranes and ulcerative lesions that turn the fragile mucosa causing bleeding.
Conjunctival involvement is characterized by bilateral conjunctivitis and there may even be formation of ulcers in the cornea (membrane that covers the iris). There is formation of ulcerative lesions at the level of the nasal mucosa with rhinitis and epistaxis. The more serious cases there is genital involvement manifesting with vaginitis (female) and balanitis (male) erosive that can cause genital bleeding, and progress to urethritis.
Besides from Dermatological manifestations, it is accompanied by general symptoms such as fever (30-40 ° C), headache (headache), general malaise, odynophagia (sore throat). And unlike major erythema multiforme, the symptoms do not self-limiting.
In cases of greater severity, there are complications such as severe dehydration, arrhythmias, pericarditis, superinfection of skin lesions and progression to sepsis, myositis, liver disease and even
seizures are associated.
The hypersensitivity reaction that causes these manifestations can be caused by drugs, mainly NSAIDs (Non-steroidal analgesics) such as acetaminophen and ibuprofen, and anti-epileptic drugs, among others: sulfas, quinolones, antifungals, metrotexate, etc. It can even be caused by viral and bacterial infections, but less frequently.
With regard to treatment, these patients:
They must be hospitalized for a minimum period of 7-10 days. Receive adequate intravenous hydration. They should keep the areas of the skin that are exposed covered to avoid superinfection. It is necessary to use emollients (moisturizing lotions) and to use antiseptics in oral lesions. The main treatment is based on the use of steroid pulses, which in this case is the methylprednisolone, which will be given at a dose of 1 g daily for 3 days and then the treatment with prednisone should continue orally in a dose of decongestant until observed improvement and resolution of skin lesions. In case of severe ophthalmological injuries consult with the ophthalmologist to determine if a different behavior is necessary than medical treatment. The prophylactic use of antibiotics is not recommended unless there is an infected lesion.
I hope you liked the post about this disease that not only affects the aesthetics of the patient, but can also cause the death of it.
