Rothmund-Thomson syndrome in a Filipino child: Report of a rare case

Rothmund-Thomson syndrome (RTS) is a rare, autosomal recessive disorder characterized by early onset of progressive poikiloderma, cutaneous and extracutaneous findings including sparse hair, juvenile cataracts, short stature, skeletal defects, hypogonadism, dystrophic teeth and nails.This is a case of a 4-year-old boy who developed at 2 months of age, reticulated erythematous macules and patches over predominantly sun-exposed areas such as the malar areas, ears, neck, extensor surfaces of the forearms and legs. This later on progressed into poikilodermatous lesions associated with sparse scalp hair, eyebrows, eyelashes and short stature. This is a report on the first documented Filipino patient diagnosed with RTS.

Asymptomatic neurocutaneous melanosis in a Filipino baby girl

Neurocutaneous melanosis (NCM) is a rare congenital syndrome characterized by the development of congenital melanocytic nevi and benign or malignant melanocytic tumors of the central nervous system. Neurologic symptoms usually manifest during the first two years of life with a high mortality rate.This is a case of a 3-year-old girl presenting with generalized large and multiple hyperpigmented macules and patches, some with hair, over the scalp, face, trunk, and extremities, present since birth. Histologic findings of a nodule within a nevus include nested melanocytes at the epidermis, cords and strands of melanocytes extending to the deep reticular dermis showing maturation with descent and lack of mitosis consistent with melanocytic nevi. The cranial magnetic resonance imaging contrast study revealed melanocytic deposits on the cerebellum and amygdala. She has regular follow-up with her dermatologist, pediatrician, and neurologist. The patient has remained asymptomatic of neurological symptoms despite the melanocytic deposits in the central nervous system.

Tolerance of baby cleansers in infants: a randomized controlled trial.

This randomized controlled trial was conducted to evaluate the tolerability of a new baby cleanser formulation Johnsons Top-to-Toe cleanser (JTT) on infantile skin. 180 healthy infants (60 in each group) were enrolled and JTT Sebamed Baby Liquid cleanser (SM), and lukewarm tap water were used on the skin of the subjects as whole body cleansers twice a week for 2 weeks. Assessment was done at baseline, 1 week and 2 weeks clinically by a dermatologist, instrumentally, and by the parents. Clinical assessment (erythema, edema, dryness and scaling); skin moisture content; skin surface pH; trans-epidermal water loss; skin oxyhemoglobin and deoxyhemoglobin; and consumer satisfaction were the outcome measures. There was no significant erythema, edema, dryness, or scaling elicited by any of the three test components. Parents did not report any side-effects. All the three studied interventions used as whole body cleansers were efficacious and well tolerated by infants.

A study on the knowledge, attitudes and practices of Southeast Asian dermatologists in the management of atopic dermatitis.

INTRODUCTION: This study evaluated the knowledge, attitudes and practices of Southeast Asian dermatologists in the management of atopic dermatitis (AD). MATERIALS AND METHODS: A questionnaire survey of 255 dermatologists in Indonesia, Malaysia, the Philippines, Singapore, Thailand and Vietnam. RESULTS: Familiarity with diagnostic criteria varied considerably. The usage of moisturisers by the respondents from Vietnam and Indonesia was significantly less frequent than the other countries. Most respondents (91% to 100%) used topical corticosteroids in children with mild-to-moderately severe dermatitis. Some respondents in the Philippines (17% to 19%) and Vietnam (11% to 25%) only used topical corticosteroids for severe disease. For infected eczema, most respondents would prescribe systemic antibiotics for mild-to-moderate infection. A minority in the Philippines (14%) and Vietnam (11%) did so only for severe infection. The top 4 systemic antibiotics prescribed most frequently were: erythromycin, cloxacillin, cephalosporin and amoxicillin/clavulanic acid. In Indonesia, a large proportion of the respondents (47%) prescribed amoxicillin most frequently. The majority of respondents (60% to 100%) prescribed both sedating and non-sedating oral antihistamines. Most respondents used oral corticosteroids to treat severe AD. Some in Malaysia, Singapore and Vietnam used cyclosporin (7% to 58%), azathioprine (5% to 31%) and methotrexate (5% to 14%). With the exception of those in Singapore, the majority of respondents (71% to 97%) did not use phototherapy. CONCLUSION: Familiarity with diagnostic criteria, the early and judicious use of moisturisers and topical corticosteroids, as well as the treatment of Staphylococcus aureus superinfection with penicillinase-stable antibiotics should be emphasised in this region.