Cutaneovisceral Angiomatosis

Department of Pediatrics, Dr. Sami Ulus Children’s Health and Diseases Training and Research Hospital, Ankara, Turkey. Email: [email protected]
 

A 4 year old girl presented with painful hemorrhagic lesions all over her body that had gradually increased in size and number since birth. Physical examination revealed multiple, various sized lesions localized on her scalp, face, ears, lips, oral cavity, trunk, arms, palms, genital area, legs and feet (Figs. 1 to 3). Her right foot had been amputated in another center at the age of 2 because of multiple giant hemorrhagic hemangiomas. Hemoglobin was 9.3 g/dL and platelet count was 197x109/ mm3. X-ray revealed radiolucent lesion on the diaphysis of the left ulna with multiple small round calcifications in the surrounding soft tissues interpreted as nonspecific lesions (Fig. 4). Abdominal ultrasound and cerebral computed tomography scans were normal.

Fig.1 Frontal view showing multiple, various sized lesions localized on face, ears, lips, trunk, arms, palms, genital area, legs and feet.	Fig. 2 Back view of the patient showing lesions spread all over the body.

 

Fig. 3 Lesions localized on plantar surface of the foot.

Fig. 4 Radiolucent lesion on the diaphysis of the left ulna with multiple small round calcifications in the surrounding soft tissues.

Biopsy of the of cutaneous lesion revealed thin-walled, blood-filled vascular channels lined by bland, sometimes hobnail endothelial cells and endothelial hyperplasia.

Cutaneovisceral angiomatosis is a rare vascular disorder characterized by generalized multiple, red brown to blue, discrete papules, macules, plaques and nodules ranging in size from millimetres to several centimetres involving the trunk and extremities. The lesions are present congenitally and new lesions continually appear throughout childhood. Other sites of involvement include the gastrointestinal tract, lung, bone, liver, spleen, muscle and synovium. It could be complicated by gastrointestinal hemorrhage, even sepsis and death. Benign lymphangioendothelioma and hobnail hemangioma reveal close histological similarity to cutaneovisceral angiomatosis. The most common clinical conditions that should be thought in differential diagnosis are neonatal hemangiomatosis and blue rubber bleb nevus syndrome. There is no standard treatment.