sirolimus and Erythema

The regeneration or revascularization of blood vessels after pulsed dye laser (PDL) treatment is one of the causes of treatment failures of cutaneous capillary malformations (CM). Recently, topical administration of rapamycin was introduced as a possible adjunctive therapeutic option to minimize postlaser revascularization in facial CM.. We evaluated the effect of combined use of 1% topical rapamycin with PDL compared to PDL alone in cutaneous CM of trunk or extremities and tried to identify the optimal duration of topical rapamycin application.. Three adjacent areas of cutaneous CM that had never been treated before were selected in each patient and underwent the following regimens: (A) PDL + vehicle for 8 weeks post-PDL; (B) PDL + topical rapamycin for 1-week post-PDL and (C) PDL + topical rapamycin for 8 weeks post-PDL. Each test site was treated by PDL for two sessions with 8 weeks interval.. Only one of six patients showed clinical improvement with combined rapamycin treatment. Overall, there was no statistically significant difference in erythema and blanching rate among PDL alone and combined rapamycin regimens.. One percent topical rapamycin does not seem to be effective as a treatment modality for cutaneous CM of trunk or extremities.

Topics: Administration, Topical; Adult; Combined Modality Therapy; Erythema; Female; Humans; Immunosuppressive Agents; Lasers, Dye; Low-Level Light Therapy; Male; Middle Aged; Pilot Projects; Port-Wine Stain; Prospective Studies; Sirolimus; Treatment Outcome; Young Adult

A term newborn presented with widespread cutaneous erythematous to bluish lesions since birth. He had extensive lesions in the gastrointestinal tract, brain, retina, heart, and bones. He also developed an intestinal perforation due to erosion of an intestinal lesion. Due to his critical status and clinical presentation, he was initially diagnosed with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT), and sirolimus treatment was initiated. Sirolimus was given by buccal route in this nonfeeding patient. Therapeutic serum levels were obtained comparable to enteral administration. Buccal mucosa was an effective novel route of sirolimus administration in this patient.

Topics: Drug Administration Routes; Erythema; Humans; Immunosuppressive Agents; Infant, Newborn; Male; Mouth Mucosa; Sirolimus

Recent reports have suggested that the topical formulation of sirolimus is effective in treating facial angiofibromas in tuberous sclerosis complex (TSC). Here, we determined the safety and efficacy of 0.2% topical sirolimus for the treatment of facial angiofibroma and compared its effects based on age.. This was a retrospective study which involved 36 TSC patients with facial angiofibromas who were treated with 0.2% sirolimus ointment. Its effect was evaluated using the Facial Angiofibroma Severity Index (FASI). In order to observe its comparative effect based on patient age, a subgroup analysis was performed, between the adult group (> 18 years old) and the pediatric group (≤18 years old).. The total FASI as well as its subcategories (erythema, size, and extent) showed statistically significant improvements after the topical treatment with 0.2% sirolimus ointment (FASI before treatment: 7.2 ± 1.1, FASI after treatment: 4.4± 1.4, p < 0.001). Among the subcategories of FASI, the erythema was most significantly reduced with the fastest response to the treatment. In a subgroup analysis, the pediatric group showed significantly greater improvements in FASI (improvement of FASI in the pediatric group = 49.7 ± 12.2%, adult group = 27.9 ± 15.6%, p < 0.001). The serial improvement analysis also showed that the pediatric group achieved a consistently greater improvement in FASI at all visits. Its 1-year application in 3 patients demonstrated a continuous maintenance effect. No significant adverse effects were observed.. 0.2% sirolimus ointment is safe and effective for facial angiofibromas. Considering its higher efficacy in younger patients, an early initiation of the treatment is recommended.

Topics: Administration, Cutaneous; Adolescent; Adult; Age Factors; Angiofibroma; Child; Child, Preschool; Erythema; Facial Neoplasms; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Ointments; Retrospective Studies; Severity of Illness Index; Sirolimus; Skin Neoplasms; Tuberous Sclerosis; Tumor Burden; Young Adult

Topics: Administration, Cutaneous; Adult; Angiofibroma; Chemistry, Pharmaceutical; Erythema; Esthetics; Facial Neoplasms; Female; Humans; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Sirolimus; Solutions; Treatment Outcome; Tuberous Sclerosis