RESUMO
Leprosy is a chronic infectious granulomatous disease caused by Mycobacterium leprae, primarily affecting the skin and the peripheral nerves. Variable involvement of internal organs and mucosa has been reported with incidence ranging from 19% to 60%, more commonly seen in lepromatous spectrum of leprosy (LL and BL). We report a case of borderline tuberculoid (BT) leprosy presenting with oral mucosal lesions involving the gingival mucosa and lower lip.
RESUMO
A four-year-old girl was brought to the dermatology outpatient department with scaling all over the body since birth. She had history of episodic vomiting and abdominal distension. A dermatological diagnosis of lamellar ichthyosis was made. Abdominal examination revealed a nontender hepatomegaly, fatty liver on ultrasonography and deranged liver function tests. Peripheral blood smear showed lipid vacuoles in the granulocytes consistent with Jordans' anomaly. Similar lipid vacuoles were seen in the basal layer in skin biopsy. An inflammatory infiltrate, moderate fibrosis in the portal tract and diffuse severe fatty change in hepatocytes were seen in liver biopsy. The patient was diagnosed as a case of Dorfman-Chanarin syndrome.