Steven Johnson syndrome - the second "speechless" diseases

Steven-Johnson Syndrome (SSJ) is a set of clinical symptoms of mucocutaneous eruption characterized by the triad of skin disorders on vesikulobulosa, orifice and eye mucosa accompanied by severe general symptoms. Synonyms include:-Rendu syndrome de Friessinger, erythema multiform major eksudativum, erythema poliform pemphigoid, Muko-kutaneo syndrome-ocular, dermatostomatitis, etc..

CLINICAL SYMPTOMS

Prodromal symptoms ranged from 1-14 days of fever, malaise, cough, korizal, pain swallowing, chest pain, vomiting, muscle aches and atralgia which vary greatly in degree of weight and combination of these symptoms.

After that will arise in the lesions:

• The skin form of erythema, chapel, vesicle, or bull symmetrically on almost the entire body.

• mucosa in the form of vesicles, bull, erosion, excoriation, bleeding red and leprosy. Bula occur suddenly in 1-14 days prodormal symptoms, appeared on the mucous membrane, membrane of the nose, mouth, anorectal, vulvovaginal region, and the urethral meatus. Ulcerative stomatitis and crusting hemoragis is the main picture.

• Eyes: konjungtivitas kataralis, blefarokonjungtivitis, iritis, iridosiklitis, eyelid edema and difficult to open, in case of severe erosion and perforation of the cornea that can cause blindness.Ocular mucosal injury is the trigger factors that cause ocular cicatricial pemphigoid, a chronic inflammation of the ocular mucosa, which causes blindness. The time required from onset until the occurrence of ocular cicatricial pemphigoid varies from several months to 31 years.

DIAGNOSIS

Diagnose directed against manifestations in accordance with triad abnormalities of skin, mucosa, eye, and its relationship with clinical factors that have shaped the target lesion, or eye sliced beef, abnormalities in the mucosa, fever. Also supported by laboratory examinations including peripheral blood examination, inspection imunologic, culturing the bacteria and test the resistance of the blood and the lesion, and histopathological examination of skin biopsy.Anemia can be found in cases with severe bleeding, leukocytes usually normal or slightly raised, there was an increase of eosinophils. IgG and IgM can be high, C3 and C4, normal or slightly decreased and can be detected circulating immune complexes. Skin biopsy was planned if there is no classic lesions. Imunoflurosesensi director can help the diagnosis of atypical cases.

Differential diagnosis

The main differential diagnosis is a toxic epidermal necrosis (NET) in which similar clinical manifestations but the general state of NET looks worse than SSJ.

Treatment

In general, patients generally a condition SSJ come with heavy, so therapy is usually given are:

• Fluids and electrolytes, as well as parenteral calories and protein.

• broad-spectrum antibiotics, followed by the results of bacterial culture and resistance tests of skin lesions and blood supply.

• Kortikosteroid Parenteral: 1mg/kg BW deksamentason initial bolus dose, then for three days from 0.2 to 0.5 mg / kg body weight every six hours. The use of systemic steroids is still controversial, there is assuming that the use of systemic steroids in children can cause slow healing and significant side effects, but there are also consider steroids beneficial and save lives.

• Antihistamines if necessary. Especially when there is itching Feniramin example hydrogen maleate (Avil. eyes and skin treatments and topical antibiotics.

• Bula in skin treated with wet compresses Burowi solution.

• Not allowed to use topical steroids on skin lesions.

• mouth lesions are given kenalog in orabase.

• Treatment of secondary infections with antibiotics is rarely cause allergies, broad spectrum, is bactericidal, non-nephrotoxic, such as intravenous clindamycin