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1.
Journal of the Philippine Dermatological Society ; : 58-60, 2011.
Article in English | WPRIM | ID: wpr-633072

ABSTRACT

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.


Subject(s)
Humans , Male , Atrophy , Cataract , Connective Tissue Diseases , Dwarfism , Eyebrows , Eyelashes , Forearm , Hypogonadism , Leg , Nails , Rothmund-Thomson Syndrome , Scalp , Skin Diseases
2.
Journal of the Philippine Dermatological Society ; : 52-54, 2011.
Article in English | WPRIM | ID: wpr-633070

ABSTRACT

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.


Subject(s)
Humans , Female , Child , Amygdala , Cerebellum , Dermatologists , Dermis , Epidermis , Hair , Melanocytes , Melanosis , Mitosis , Neurocutaneous Syndromes , Neurologists , Nevus , Nevus, Pigmented , Scalp , Skin Neoplasms
3.
Indian Pediatr ; 2010 Nov; 47(11): 959-963
Article in English | IMSEAR | ID: sea-168704

ABSTRACT

This randomized controlled trial was conducted to evaluate the tolerability of a new baby cleanser formulation - Johnson’s 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 dermato-logist, 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 consu-mer 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.

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