Bullous skin lesions in a jaundiced infant after phototherapy: a case of congenital erythropoietic porphyria.

Congenital Erythropoietic Porphyria

Congenital erythropoietic porphyria (CEP) is a rare cutaneous pophyria which presents with photosensitivity, complicated by presence of hemolytic anemia. There is no permanent cure and the treatment is supportive. A 3 day-old male baby was admitted with neonatal jaundice for phototherapy. The baby developed bullous lesions over both the feet within minutes of starting phototherapy(Fig.1). These...

Bullous disease of dialysis=ءادلا يعاقفلا جتانلا نع لاحتلا يومدلا Bullous Disease of Dialysis Bullous dermatosis of dialysis is a syndrome of cutaneous fragility and blistering. The skin lesions clinically and histologically resemble those of porphyria cutanea tarda

Congenital Erythropoietic Porphyria: Mutation of the Uroporphyrinogen III Cosynthase Gene in a Vietnamese Patient

Congenital erythropoietic porphyria (CEP) arises from an autosomal recessive inherited disorder of the porphyrin metabolism, which leads to the accumulation of uroporphyrinogen I in bone marrow, skin and several other tissues by a deficiency of uroporphyrinogen III cosynthase (UROS). We studied a Vietnamese patient and her family suffering from severe cutaneous photosensitivity with skin fragil...

Erythropoietic (congenital) porphyria: A rare abnormality of the normoblasts.

ECENT STUDIES” 2, 3 have emphasized that in human beings, photosensitivity occurs as a result of two fundamentally different disturbances of porphyrin metabolism. In porphyria cutanea tarda, in which the excessive porphyrin formation is believed to take place in the liver, symptoms are seldom manifest before adult life.’ Its porphyria erythropoietica (congeisital, photosensitive), where large a...

Report of a Case Report of Kindler Syndrome

A 13 years old boy with progressive poikiloderma, bullous lesions in the extremities and photosensitivity is reported. Physical examinations were otherwise normal, his physical development was normal, no other family member had a similar disease. Routin laboratory exams were in the normal limits.Histopathology exams from poikilodermatous skin showed atrophy of the epidermis, liquification degen...