CLAPO syndrome: Effective response to treatment with oral rapamycin

Dermatol Ther. 2019 Jul;32(4):e12991. doi: 10.1111/dth.12991. Epub 2019 Jun 20.

Abstract

CLAPO syndrome (Capillary vascular malformation of the lower lip, Lymphatic malformations of the head and neck, Asymmetry and Partial or generalized Overgrowth) is a nonfrequent pathology. This syndrome is characterized by the capillary malformation (CM) of the lower lip, a very important clinical sign when diagnosing CLAPO. The aim of our report is to demonstrate that rapamycin could be a reliable and safe targeted therapy in lymphatic malformations (LMs). This drug is useful in reducing the LM's size before final surgical treatment. The clinical and radiological evolution of a patient with CLAPO syndrome is reported in this article, before and after the treatment with rapamycin.

Keywords: overgrowth; rapamycin; vascular malformation.

Publication types

  • Case Reports

MeSH terms

  • Administration, Oral
  • Arteriovenous Malformations / drug therapy*
  • Arteriovenous Malformations / physiopathology
  • Humans
  • Immunosuppressive Agents / administration & dosage*
  • Immunosuppressive Agents / adverse effects
  • Infant, Newborn
  • Lymphatic Diseases / drug therapy*
  • Lymphatic Diseases / physiopathology
  • Male
  • Sirolimus / administration & dosage*
  • Sirolimus / adverse effects
  • Treatment Outcome

Substances

  • Immunosuppressive Agents
  • Sirolimus

Supplementary concepts

  • CLAPO Syndrome