RESUMO
Fibrous hamartoma of infancy (FHI) is a rare, benign, soft tissue tumor. The lesion's clinical resemblance to more aggressive soft tissue tumors of childhood makes histologic diagnosis paramount. We report a unique presentation of a FHI associated with localized hypertrichosis and hyperhidrosis.
Assuntos
Dorso , Hamartoma/diagnóstico , Hiperidrose/diagnóstico , Hipertricose/diagnóstico , Neoplasias Cutâneas/diagnóstico , Biópsia , Diagnóstico Diferencial , Feminino , Hamartoma/patologia , Humanos , Hiperidrose/patologia , Hipertricose/patologia , Lactente , Neoplasias Cutâneas/patologiaRESUMO
BACKGROUND: Medical therapies for hidradenitis suppurativa (HS) are often ineffective. Tumor necrosis factor-alpha inhibitors may be a potential treatment for patients with moderate to severe HS. OBJECTIVES: We sought to evaluate the safety and efficacy of etanercept for patients with severe HS. METHODS: We conducted a phase II clinical trial of etanercept (50 mg/wk subcutaneously) in patients with moderate to severe HS. Efficacy was measured using a Physician Global Assessment and several secondary physician- and patient-reported outcome measures. Responders were classified as those achieving at least a 50% reduction on the Physician Global Assessment score at week 12 compared with baseline. RESULTS: Only 3 of the 15 patients who entered the study were classified as responders (response rate of 20%; 95% confidence interval: 4.3-48.1) based on the intention-to-treat analysis. Dermatology Life Quality Index scores improved slightly from a median of 19 to 15 (P = .02). Comparison of baseline with week-12 Physician Global Assessment scores, and secondary outcome measures of lesion counts and patient pain scores, failed to show statistically significant improvement. Etanercept was generally well tolerated; however, two patients discontinued the study as a result of skin infections at the site of hidradenitis lesions requiring oral antibiotics. LIMITATIONS: Lack of a control group and a small number of participants are limitations. CONCLUSIONS: Our study demonstrated minimal evidence of clinically significant efficacy of etanercept (50 mg/wk subcutaneously) in the treatment of hidradenitis. Future studies using higher doses of etanercept are indicated; however, patients need to be carefully monitored for infection and other adverse events. Randomized, controlled trials will be necessary to demonstrate the risk-to-benefit ratio of tumor necrosis factor-alpha inhibitors in the treatment of hidradenitis.
