2025 – PAGE 152 – DERMATOLOGY

(DOUBLE TAKE) PHACES SYNDROME

A diagnosis of PHACES syndrome requires a large hemangioma in the face/neck area PLUS one of the following defects:

• Posterior fossa malformation (DANDY WALKER)
• H Often in the distribution of the Facial Nerve. Look for a large segmental hemangioma on the FACE. Segmental refers to what looks like a nerve distribution (segmented by normal skin in between).
• Arterial cerebrovascular anomaly: Including STROKES
• Cardiac anomalies: Especially COARCTATION OF THE AORTA
• Eye anomalies: MICROPHTHALMIA, STRABISMUS
• Sternal defect
• IMAGE: www.pbrlinks.com/PHACES1

(DOUBLE TAKE) KASABACH-MERRITT SYNDROME

In Kasabach-Merritt syndrome, there are large, congenital vascular tumors. They are not true hemangiomas but can cause a severe CONSUMPTIVE COAGULOPATHY (in the form of thrombocytopenia and the consumption of coagulation factors) and death. It is most common in infants.

IMAGE: www.pbrlinks.com/KASABACH1
IMAGE: www.pbrlinks.com/KASABACH2

PEARL: Look at the above images closely. Make sure you look closely at images so that you do not get this vascular tumor confused with hemihypertrophy.

MNEMONIC:

• >—< is used by many of us when recording CBC results.
  

NEVUS SIMPLEX

A nevus simplex is a Salmon colored lesion often called a Stork bite or Salmon patch. They blanch on pressure and tend to be on the midline or symmetrical (e.g. on both eyelids). These fade with time and are benign. Do not get this term confused with Nevus FLAMMEUS (AKA PORT WINE STAIN).

PEARL: These BLANCH with pressure.

IMAGE: www.pbrlinks.com/NevusSimplex1
IMAGE: www.pbrlinks.com/NevusSimplex2