Stevens Johnson Syndrome
| Classification according to ICD-10 | |
|---|---|
| L51.1 | Bullous erythema multiforme Stevens-Johnson syndrome |
| ICD-10 online (WHO version 2019) | |
The Stevens-Johnson syndrome (synonym: Dermatostomatitis Baader , Fiessinger-Rendu syndrome ) is a drug to infections or allergy-related skin disease . The term erythema exudativum multiforme majus was previously used as a synonym, although this is obsolete due to its different etiology and courses.
causes
The most common cause (approx. 50%) are allergic reactions to drugs ( carbamazepine , chloroquine , sulfonamides , ( codeine ), hydantoins , NSAIDs , allopurinol , NNRTI , moxifloxacin , strontium ranelate etc.). Less common triggers are lymphoma , mycoplasma infections, or other bacterial or viral infections.
Pathomechanism
It is an immunological, T-cell -mediated reaction. It comes to necrosis of keratinocytes . The final pathogenesis , however, is still unclear.
Symptoms
Clinically, the disease manifests itself with an acute onset with severe disorders of the general condition, high temperatures and rhinitis . The mucous membranes are heavily involved, erythema with a rather blurred definition and a darker central area (atypical cockades ) and painful blisters in the mouth, throat and genital area and erosive conjunctivitis (inflammation of the eye conjunctivitis), which requires ophthalmological treatment. Often the patient can no longer open his mouth and food intake is difficult. Lyell's syndrome is the maximum complication .
Diagnosis
Despite the typical clinical picture, a skin biopsy should be taken to confirm the diagnosis. There are no specific laboratory parameters or specific tests for this diagnosis. Histopathology: There are necrotic keratinocytes and a vacuolization of the basement membrane up to the formation of subepidermal clefts.
therapy
The focus should be on eliminating or avoiding the triggering cause (drugs that have been used in the last 2-3 weeks are particularly suspicious). Mycoplasma infections should be treated with tetracyclines ; macrolide antibiotics are preferred in childhood. Therapy with glucocorticoids is controversial and probably associated with increased mortality. Patients with lesions in the mouth and throat seem to more easily succumb to fatal infections of the respiratory tract from treatment with corticosteroids. The administration of immunoglobulins cannot generally be recommended either. In the case of extensive skin detachment, the same treatment rules apply as for burn patients: intensive medical measures with fluid, protein and electrolyte balance, cardiovascular monitoring and strict infection prophylaxis. Parenteral nutrition may be indicated if food intake is difficult . Local therapy is carried out with disinfecting solutions, gauzes and moist compresses on the mucous membranes.
forecast
The mortality rate is 6%, but in the transitional form to toxic epidermal necrolysis (TEN = drug-induced Lyell's syndrome ) it is around 25% and in the case of existing TEN it is around 50%. The skin symptoms usually heal without scarring, and skin pigmentation disorders can sometimes persist for months . Complications are mucosal strictures , particularly the Symblepharon (adhesion of the eyelid to the eyeball ).
See also
literature
- Pschyrembel, Clinical Dictionary . 261st edition. De Gruyter, Berlin 2007, ISBN 978-3-11-018534-8
- Ingrid Moll: Dermatology (dual series). 7th edition. Thieme Verlag, 2010, ISBN 978-3-13-126687-3
Individual evidence
- ↑ blitz-at of November 20, 2007: “Caution! Serious hypersensitivity reactions to strontium ranelate (Protelos) " arznei-telegramm.de
- ↑ Kelemen JJ 3rd, Cioffi WG, McManus WF, Mason AD Jr, Pruitt BA Jr: Burn center care for patients with toxic epidermal necrolysis. In: J Am Coll Surg. 1995, PMID 7874336
