We report the case of 17-year-old female diagnosed with Costello syndrome. Genetic testing provided a proof with G12S mutation in the HRAS gene since 3 years of age with a presentation of severe nodulocystic acne on her face. After 2 months of oral isotretinoin treatment, improvement in her acne was observed. Interestingly, an unexpected significant improvement of acanthosis nigricans on her neck and dorsum of her hands was found as well. We present this case as a successful treatment option by using oral isotretinoin for the treatment of acanthosis nigricans in Costello syndrome patients.
Background: Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening skin conditions with an etiology of drug exposure or infections. Objectives: To determine the cause, treatments, complications, and outcomes of SJS/TEN in children admitted to Srinagrind Hospital during 1992−2012. Methods: Retrospective chart review. A diagnosis of SJS and TEN was confirmed by pediatric dermatologists. Results: A total of 38 patients was recorded. They consisted 31 (82%) SJS patients and 7 (18%) TEN patients. Mean age 6.6 years (range 1 to 14 years). Male to female was 1.1:1. Most cases (30 or 79%) were caused by drug exposure. Three cases (8%) by infection, and 5 cases (13%) were of unknown cause. The antiepileptic drug group was the most common cause. Systemic corticosteroids were used in 33 cases (87%). Intravenous immunoglobulin was used in one TEN patient (3%). There were 18 cases (47%) with acute complications. Ocular complications (7 cases, 39%), septicemia (4 cases, 22%), and secondary skin infections (3 cases, 17%) were the most common. Mean difference in length of hospital stay between those with and without acute complications was 12.3 days (P < 0.01, 95% CI 5.9-18.6). Ocular complications were the only long-term complications at 1-year follow up, and included symblepharon, corneal pannus, and dry eyes. Two patients (5%), both having cases of TEN, died. Conclusions: Antiepileptic drugs were the most common causes of SJS/TEN in our study. Good ophthalmologic care of the prevalent acute eye complications in these patients is needed to prevent long-term ophthalmic complications.
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