ABSTRACT
Linear and whorled nevoid hypermelanosis(LWNH) is a congenital disorder characterized by hyperpigmented macules in a linear and whorled pattern along Blaschko's lines, and develops a few weeks after birth. Skin manifestations closely resemble hypomelanosis of Ito with inverse pigmentation. There is no preceding inflammatory event and no pigmentary incontinence histologically. It may be associated with various congenital abnormalities. We report a case of linear and whorled nevoid hypermelanosis associated with congenital hemihypertrophy of left extremities in a 10-year old boy.
Subject(s)
Child , Humans , Male , Congenital Abnormalities , Congenital, Hereditary, and Neonatal Diseases and Abnormalities , Extremities , Hyperpigmentation , Hypopigmentation , Parturition , Pigmentation , Skin ManifestationsABSTRACT
Congenital hemihypertrophy is a rare idiopathic condition, first described by Meckel in 1822 and represents enlargement of a part or entire side of the body without associated vascular or neurologic disease. Wagner recorded the first case in the 1839. Hemihypertrophy is to be differentiated from hemiatrophy (which involves unilateral subnormal development, muscle weakness, or neurologic deficit) and the syndromes of hemidystrophy. Approximately 25 - 50% of the reported cases of hemihypertrophy have been associated with hamartomas or congenital defects, especially genitourinary anomalies. The early detection of asymptomatic intraabdominal tumor in patients with congenital hemihypertrophy is important We report 3 cases of congenital hemihypertrophy with discussion of the clinical manifestations and associated anomalies, which were rarely reported in domestic literatures.