7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described...

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7 months old-boy. Several red or blue papular lesions Case 5 SF

Transcript of 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described...

Page 1: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

7 months old-boy. Several red or blue

papular lesions

Case 5 SF

Page 2: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

Blue lesion

Case 5 SF

Page 3: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

Case 5 SF

Page 4: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

Red lesion

Case 5 SF

Page 5: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

Case 5 SF

Page 6: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

What is your diagnosis ?

Case 5 SF

Page 7: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

Veinous malformation

Blue rubber bleb nevus

syndrome

Soft rubbery nodules, blue or purple

Page 8: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

Blue rubber bleb nevus syndrome

Described by Bean (Bean syndrome)

Can be inherited as an autosomal

dominant trait but sporadic cases are

more common

Multiple veinous malformations in

gastro-intestinal tract

Main complication is the possibility of

bleeding of the GI lesions

Page 9: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

Glomu-veinous malformations (GVM)

« glomangiomas »

Page 10: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

Vascular Tumors Vascular Malformations

°Infantile hemangioma Slow- flow Vascular Malformation

° Capillary Malformation (CM)

°Congenital hemangiomas Port-wine stain

(NICH and RICH) Telangiectasia

°Tufted angioma Angiokeratoma

°Kaposiform hemangioendothelioma °Venous Malformation (VM)

°Spindle cell hemangioma Common sporadic VM

°Others, rare (Dabskas’s T..) Bean syndrome Familial cutaneous and mucosal VM

Glomuvenous malformation

Maffuci syndrome

°Lymphatic malformation (LM)

Fast- flow Vascular Malformation

°Arterial Malformation (AM)

°Arteriovenous fistula

°Arteriovenous malformation (AVM)

Complex-combined vascular malformations°CVM, CLM, LVM, CLVM, AVM-LM, CM-AVM

Vascular anomalies in children ISSVA 1996

Page 11: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

SMA

Glomangioma

Page 12: 7 months old-boy. Several red or blue papular lesions · Blue rubber bleb nevus syndrome Described by Bean (Bean syndrome) Can be inherited as an autosomal dominant trait but sporadic

BRBN


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