ABSTRACT
BACKGROUND: With an incidence of 1.5-1.8/1 million inhabitants per year, toxic epidermal necrolysis is a rare but life threatening disease. It is almost always drug-induced and its lethality is pronounced with up to 50 %. Several therapeutic options are described in literature; however, there is still lack of a universally accepted and specific therapy of toxic epidermal necrolysis. METHODS: This survey considers 8 cases of toxic epidermal necrolysis diagnosed and treated in our clinic from 2003 to 2007. The epidermal sloughing was > 30 % of the body surface in each case. RESULTS: After immediately discontinuing the drug suspected of being responsible for toxic epidermal necrolysis, we treated with systemic corticosteroids in an initial dose of up to 1.5 mg/kg. Moreover, special emphasis was put on basic measures such as control of vital parameters. With this treatment we reached good results; none of the patients died. conclusions: Immediate beginning of therapy is essential for a successful treatment of toxic epidermal necrolysis. Besides systemic therapy with corticosteroids, certain basic measures such as isolation of patients at adequate room temperature to prevent hypothermia, strict control of circulation, temperature and laboratory parameters, daily smears of skin and mucous membranes and a diet rich in calories due to the catabolic metabolic status are very important for successful outcome.
Subject(s)
Prednisone/therapeutic use , Stevens-Johnson Syndrome/drug therapy , Adult , Aged , Aged, 80 and over , Allopurinol/therapeutic use , Allopurinol/toxicity , Anti-Bacterial Agents/therapeutic use , Anti-Bacterial Agents/toxicity , Anti-Inflammatory Agents/therapeutic use , Anticonvulsants/therapeutic use , Anticonvulsants/toxicity , Bacterial Infections/diagnosis , Bacterial Infections/drug therapy , Bacterial Infections/mortality , Ciprofloxacin/therapeutic use , Ciprofloxacin/toxicity , Drug Combinations , Female , Folic Acid/therapeutic use , Folic Acid/toxicity , Gout Suppressants/therapeutic use , Gout Suppressants/toxicity , Humans , Hydroxocobalamin/therapeutic use , Hydroxocobalamin/toxicity , Lidocaine/therapeutic use , Lidocaine/toxicity , Male , Middle Aged , Phenytoin/therapeutic use , Phenytoin/toxicity , Pyridoxine/therapeutic use , Pyridoxine/toxicity , Stevens-Johnson Syndrome/diagnosis , Stevens-Johnson Syndrome/mortality , Superinfection/diagnosis , Superinfection/drug therapy , Superinfection/mortality , Survival Rate , Trimethoprim, Sulfamethoxazole Drug Combination/therapeutic use , Trimethoprim, Sulfamethoxazole Drug Combination/toxicityABSTRACT
Subcorneal pustulosis (Sneddon-Wilkinson disease) is a rare inflammatory neutrophilic dermatosis. While subcorneal pustulosis is often associated with an IgA gammopathy, the combined lack of IgG/IgM seen in our case is rare. An 83-year-old man with combined lack of IgG/IgM and monoclonal gammopathy type IgA/Kappa presented with subcorneal pustules. Intravenous immunoglobulin therapy led to complete regression and might be another therapeutic option.
