Ash leaf macules are seen in –
Correct Answer: Boumeville's disease
Description: Ans. is 'b' i.e., Bourneville's disease o Ash-leaf shaped hypopigmented macules are characteristic of Tuberous sclerosis (Bourneville's disease).Tuberous sclerosis (Bourneville's disease)o Tuberous sclerosis is characterized by : -1. Cutaneous lesions - Adenoma sebaceum, ash-leaf shaped hypopigmented macules, shagreen patches, depigmented nevi.2. Seizures3. Mental retardationo Other features - Calcified subependymal nodules, Subependymal giant cell astrocytoma, ependymoma, Rhabdomyomas of the heart, and angiomyomas of the kidney, liver, adrenals and pancreas.Von Hippel - Lindau syndromeo This syndrome consists of Retinal, cerebellar and spinal hemangioblastoma.o Renal cell carcinoma, Pheochromocytoma, benign cyst of the kidney, liver, pancrease and epididymis may also occur.o Polycythemia may occur due to secretion of erythropoietin from hemangioblastoma.
Category:
Pathology
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