Milroy's disease associated with scrotal lymphangioma circumscriptum.

INTRODUCTION Milroy’s Disease (MD) is characterized by peripheral edema of the lower extremities at birth or in early childhood which is due to complete aplasia of dermal lymphatics. Diagnosis of MD can be made easily by imaging the lymphatic channels with radionuclide lymphoscintigraphy or dynamic magnetic resonance lymphangiography. Lymphangioma circumscriptum (LC) is also a benign disorder of lymphatic vascular channels and considered to be a circumscribed developmental disease of lymphatic tissues in the dermis which is characterized by lymphatic cisterns in the subcutaneous tissue and communicate with dilated dermal lymphatics. LC is generally localized at any anatomic site of human body but more frequently affect the chest, axillary folds, shoulders, neck, buccal mucosa, proximal limbs and rarely hips, groins, and genital area. The pathognomonic appearance of LC is multiple translucent or hemorrhagic vesicular lesions with clear leakage. Association of MD with LC is very rare.(1)