RESUMO
Papillon-Lefèvre syndrome (PLS) is a rare inherited palmoplantar keratoderma (PPK) that is associated with progressive gingivitis and recurrent pyodermas. We present a case exhibiting classic features of this autosomal-recessive condition and review the current understanding of its pathophysiology, diagnosis, and treatment. Additionally, a review of pertinent transgredient PPKs is undertaken, with key and distinguishing features of each syndrome highlighted.
Assuntos
Gengivite/etiologia , Doença de Papillon-Lefevre/fisiopatologia , Pioderma/etiologia , Adulto , Catepsina C/genética , Progressão da Doença , Feminino , Gengivite/tratamento farmacológico , Gengivite/patologia , Humanos , Mutação , Doença de Papillon-Lefevre/tratamento farmacológico , Doença de Papillon-Lefevre/genética , Pioderma/patologia , RecidivaRESUMO
We report a neonate who presented at birth with multiple, scattered, white macerated plaques. Punch biopsy confirmed epidermal nevi. At 3 months of age the patient presented with infantile spasms and, after full evaluation, was diagnosed with tuberous sclerosis complex (TSC). We suggest that physicians consider TSC in a neonate with epidermal nevi inconsistent with any described epidermal nevus syndrome.
Assuntos
Neoplasias Cutâneas/etiologia , Neoplasias Cutâneas/patologia , Esclerose Tuberosa/complicações , Esclerose Tuberosa/patologia , Humanos , Recém-Nascido , Masculino , Nevo/etiologia , Nevo/patologiaRESUMO
BACKGROUND: Few published studies have analyzed serum lidocaine levels and individual patient characteristics affecting metabolism after application of compounded topical anesthetics. OBJECTIVE: To measure serum lidocaine levels during and cutaneous side effects after standardized application of 23% lidocaine/7% tetracaine compounded anesthetic to the face of healthy volunteers. METHODS AND MATERIALS: Fifty-two volunteers were enrolled, and compounded 23% lidocaine/7% tetracaine ointment was applied to their faces for 2 hours. Lidocaine levels were determined every 30 minutes during application and for 2 hours after removal. Follow-up telephone calls 3 days later assessed cutaneous side effects. RESULTS: Median peak lidocaine level was 1.15 µg/mL, and the highest peak lidocaine level in an individual was 3.4 µg/mL. Higher serum lidocaine levels were found in men (p < .01), nonwhite volunteers (p = .02), and those with larger facial surface area (p = .04). Age and body mass index did not affect lidocaine levels. Irritant contact dermatitis was common, resulting in hyperpigmentation in some patients. CONCLUSION: Facial surface area, male sex, and nonwhite ethnicity were associated with higher serum lidocaine levels after topical application of lidocaine. Compounded anesthetics containing lidocaine should be used with caution under the direct supervision of a physician.