Incontinentia pigmenti, also known as Bloch-Sulzberger syndrome, is a rare X- linked dominant multisystem disease involving ectodermal structures namely cutaneous, ocular, dental, neurological and skeletal systems1. Mutation of the nuclear factor kappa B essential modulator (NEMO) gene in chromosome Xq28 is determined to cause this rare genodermatosis2. The cutaneous manifestations are the most characteristic features of this disorder3. We would like to report 3 cases of incontinentia pigmenti seen in the skin clinic, Sarawak General Hospital.

Introduction: Infantile haemangioma (IH) is a common benign vascular tumour which is characterised by rapid proliferation in early infancy period followed by slow involution for years. The initial rapid growth and the location of haemangioma may result in significant morbidity. The introduction of oral Propranolol for the treatment of IH in recent years has seen many promising results though with potential risks. Objective: To review the demography, clinical pattern, management and outcome of patients with IH treated with propranolol in a paediatric dermatology referral centre. Methodology: A retrospective observational study of medical records of patients with IH treated with oral propranolol (maximum dose 2mg/kg/day) from May 2009 to May 2011 in Paediatric Institute, Kuala Lumpur Hospital. Results: A total of 17 patients (14 girls, 3 boys) were studied. They were 12 Malays (70.6%), 2 Chinese(11.8%), 2 Indians(11.8%) and 1 Indonesian (5.9%). Mostly term babies (88%). The age of presentation is from birth to 2 months old. The location of haemangiomas involve periocular (35.3%), perioral (17.6%), facial segmental (17.6%), nasal (5.9%), multiple (17.6%) and spinal (5.9%). They were started on Propranolol at a mean age of 5 months old except one at 2 years old. Five patients had concurrent adjunctive therapies. 70.6% had significant reduction of the size and colour of haemangiomas, 11.8% not significant, 11.8% unclear, and one patient defaulted follow up. Some patients developed wheezing episodes (29.4%), diarrhoea (17.6%), regurgitation (5.9%); but no serious consequences. Conclusion: Low dose oral propranolol is a safe and effective treatment with minor side effects from our experience.