A 1-year-old female with maple syrup urine disease presenting with erythematous, partially eroded plaques on the trunk, anogenital area, and extremities experienced metabolic crisis. The skin lesions appeared at 11 months of age and was thought to result from amino acid imbalance secondary to erratic supplementation of specialized milk formula devoid of isoleucine, leucine, and valine. Serial urine monitoring showed persistent ketones and elevated serum leucine and valine. The patient was managed with emollients, intralipid 20%, and addition of isoleucine and valine supplements to counter the neurotoxic effect of leucine. After 8 days of proper feeding and continuous emollient application, the lesions improved and skin biopsy revealed superficial perivascular dermatitis. Although a decrease in erythema and desquamation was noted, the patient had persistent cerebral edema and continued to deteriorate.

Maple Syrup Urine Disease ; Isoleucine ; Leucine ; Valine ; Erythema

Background: Pruritus can impair quality of life in patients with atopic dermatitis. There is evidence that acupuncture reduces pruritus and disease severity, and improves quality of life. Objectives: This study aimed to determine the efficacy of acupuncture in reducing pruritus intensity, disease severity, and medication use, and improving quality of life. Methods: This was a patient- and assessor-blinded, randomized, placebo-controlled trial. Patients diagnosed with atopic dermatitis underwent twice-weekly acupuncture for 12 weeks, with an 8-week follow-up period. Baseline and weekly assessment were done using standard data collection forms and validated assessment tools. Results: Thirty patients were randomized and 28 patients were eligible for the efficacy analysis. There were no significant differences in the baseline demographic and clinical characteristics between the True Acupuncture group (TA) (n=16) and Sham Acupuncture group (SA) (n=12). Both groups showed reduction in mean itch intensity (visual analogue scale, VAS) (p=0.024) but TA showed greater reduction (p=0.009) that was sustained after end of treatment. There was also a reduction in medication use in both groups. The comparable efficacy of SA to TA is attributed to similar peripheral receptive fields and stimulation of cutaneous C-fibers which depletes the neurotransmitters mediating pruritus and results in tachyphylaxis. Mild adverse events, such as petechiae and erythema, were noted in both groups and resolved spontaneously. Conclusion: Acupuncture is a promising adjunct treatment in atopic dermatitis with significant reduction in pruritus, disease severity and medication use and a trend towards improved quality of life. Studies with larger sample size and comparison to acupuncture points farther from the true acupuncture points are recommended. Trial Registration Food and Drug Administration Philippine Health Research Registry ID PHRR171012-001696
Acupuncture ; Pruritus ; Dermatitis, Atopic