IMAGES IN CLINICAL PRACTICES children with large vascular tumors and is Treatment is supportive and includes characterized by thrombocytopenia, con- administration of platelet, red cell and plasma sumption coagulopathy and microangiopathic transfusions. Digitalization may be required hemolytic anemia. The clinical presentation for high output cardiac failure. Various drugs can be severe anemia, torrential hemorrhage, such as systemic steroids, recombinant rapid increase in size of the tumor or high interferon alpha, vincristine and cyclo- output cardiac failure due to the arteriovenous phosphamide have been used with variable malformation, with an overall mortality of 20- success. Other modes of management include 30%. The vascular tumor is believed to be a surgical excision, arterial embolisation and tufted angioma or a kaposiform hemangio- radiotherapy. endothelioma and not a true hemangioma. The Sridhar S., vascular lesion can be superficial or visceral Kuruvilla K.A., occurring in thoracic, abdominal, pelvic or Department of Neonatology, intracranial sites. Thrombocytopenia persists Christian Medical College, for a variable period of time lasting from a few Vellore 632 004, months to years. The child may die of Tamil Nadu, India. infection or hemorrhage. E-mail: [email protected]

Systematized back, chest, abdomen, upper and lower limbs Depigmentosus and sparing the scalp and face (Fig. 1). There was no leucotrichia. Systemic examination including musculoskeletal, ophthalmological and neu-rological was normal. Radiological A 2-year-old girl, product of non- examina-tion including chest X-ray, consanguineous marriage, presented with ultrasound of the abdomen and pelvis, X-ray hypopigmented patches over the entire body skull, pelvis, hands and feet were normal. since 5 months of age. New lesions appeared However, X-ray of the cervical and thoraco- over the next eight months, after which they lumbar spine revealed spina bifida of the C5, have been static. There was no history of C6 and C8 vertebrae. A diagnosis of visual or hearing deficit, seizures, develop- systematized nevus depig-mentosis (ND) with ment delay, abnormal sweating or inflam- spina bifida was made. matory changes preceding pigment loss. She had normal scalp hair and teeth. Family ND is a congenital, non familial, well history was not contributory. Dermatological circumscribed, uniformly hypopigmented examination revealed multiple, variable sized, macule, stable in its relative size and oval to irregular, bilaterally distributed hypo- distribution throughout life and involving to depigmented macules, scattered over the predominantly the trunk and proximal

INDIAN PEDIATRICS 1046 VOLUME 42__OCTOBER 17, 2005 IMAGES IN CLINICAL PRACTICES extremities and rarely the head and neck. Both sexes are equally affected and there is no distinct pattern of inheritance. Histologically there are normal to decreased number of with normal size, shape and content of melanosomes, but reduced number and aggregated within vacuoles. Clinical diagnostic criteria for ND include (i) leukoderma present at birth or onset early in life, (ii) no alteration in distribution of leukoderma throughout life (iii) no alteration in texture, or change of sensation, in the affected area and (iv) no hyperpigmented border around the achromic area. Three morphological variants of ND have been described; isolated (circular or rectangular), which is the commonest presentation, segmental and the rare systematized (unilateral whorls or streaks) variety. Systemic manifestations are rare in ND, although systematized ND in association with seizures, mental retardation, limb hemihypertrophy, atopic dermatitis, yellow scalp hair, has been described. Systematized ND is often confused with hypomelanosis of Fig. 1. Systematized . Ito ( achromians). It presents as unilateral or bilateral hypo- of tuberous sclerosis, , pityriasis pigmented streaks and whorls that follow versicolor, of post kala- Blaschko’s lines, the lesions exhibit changes azar dermal leishmaniasis (PKDL) and post in their manifestation over time (initially inflammatory hypopigmentation. progress and then regress), are associated with ocular, musculoskeletal and CNS Sujay Khandpur, abnormalities in 62% to 94% of cases and K. Sumanth M., show familial tendency (autosomal dominant Department of and mode of inheritance in some cases). Other Venereology, differential diagnosis in children who present All India Institute of Medical Sciences, with hypopigmented macules include , New Delhi 110 029, India. , , ash-leaf macules E-mail: [email protected]

INDIAN PEDIATRICS 1047 VOLUME 42__OCTOBER 17, 2005