Ocular lichen planus treated with lifitegrast

arrested growth pattern (IHMAG). The diagnosis, especially in the latter, could be challenging in the newborn period because of their telangiectatic appearance and can be confused with capillary malformations. The association of large segmental hemangioma on the head and neck region with underlying developmental anomalies is well recognized as PHACE(S) syndrome (posterior fossa anomalies, hemangioma, arterial lesions, cardiac abnormalities, coarctation of the aorta, eye anomalies, sternal anomalies, and supraumbilical raphe). In a similar manner, segmental IH of the lower body regions may be associated with regional developmental defects. In 2006, Girard et al. proposed the acronym PELVIS (perineal, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tags) to denote the clinical presentation of large perineal IH associated with regional visceral malformations, also including bony deformities and arterial anomalies. LUMBAR and SACRAL are other acronyms used to describe this association of findings. Anomalies of the chest and abdominal midline are frequently described in PHACE(S) syndrome. Sternal defects including, sternal pit, sternal cleft, or supraumbilical raphe are major criteria for diagnosis of the syndrome. Midline anomalies are also found in PELVIS syndrome, although infraumbilical raphe has not been previously described in either PELVIS or PHACE(S). The histopathology of associated-midline anomalies is very heterogeneous. However, anomaly of migration or organization of mesenchymal cells is implicated in most of these defects, including umbilical raphe. The high incidence of midline defects in PELVIS and PHACE(S) highlights the similarities between these syndromes and raises the question of the key role of mesenchymal cell in their possible mutual pathogenesis. We found only one case of an infraumbilical raphe previously published. It was associated with a supraumbilical raphe and a pectus excavatum. This clinical presentation highlights the risk for other ventral developmental defects associated with umbilical raphe. These ventral defects, occurring between 6 and 8 weeks of gestational age, are thus frequently associated. This clinical case reports median infraumbilical raphe as a new clinical feature associated with PELVIS. The presence of supraumbilical raphe in PHACE(S) highlights the similarities and shared pathogenesis with PELVIS. These findings support that these two syndromes should be considered as similar entities affecting the upper or the lower half of the body respectively, and share a common pathogenesis.