Proteus syndrome

A 7‐year‐old girl presented with a history of wine‐colored tumoral lesions on her leg, right foot, abdomen, and back, present since birth. They bled easily on touch or on minimal trauma. Soft, skin‐colored tumors were also present on the pectoral and left axillary regions. All the lesions had increased in size gradually. Also, since birth, she had suffered from progressive enlargement of the feet, in the form of edema. The edema was soft and cold and did not decrease with rest. Over the last 5 years, the feet had been painful while walking. The lesions were pruritic on the legs. She had presented with pain, local heat, erythema, and an odiferous secretion on the periungual margins of both feet over the last year. Her mother reported that the patient presented occasional blood streaks in her stools. The patient was born by cesarean section, due to polyhydramnios. Her psychomotor development and school life were normal, except for a delay in walking (2 years). At the age of 2 years, tumors were excised from the right popliteal and axillary regions. She received blood transfusions six times, because of a persistent anemia. There was no family history of similar tumoral lesions. On physical examination at the first evaluation, she had violet–red maculae, with scattered small papules inside, located on the abdomen, left flank, and right leg ( Fig. 1). A 12 × 6 × 4‐cm tumor, which was soft, mobile, and slightly painful on palpation, was located on the right side of the back ( Fig. 2). Another verrucous tumor with an irregular surface was located on the distal third portion of the left leg. Papillomatous and exophytic lesions with an irregular surface were found on the left axilla ( Fig. 3) and perianal region. There were brown plaques with a rough surface distributed in longitudinal bands from the left hand, through the left arm to the left anterior–posterior side of the trunk, and on the left leg ( Fig. 1). A keloid scar was located on the right popliteal region. Multiple skin‐colored papules with a rough surface, were scattered over the thighs. On the fingernails of the left hand, the first four fingers had dystrophic nails, inversion of distal curvature, longitudinal streaks, and grayish pigmentation. On the feet, there was soft, white, cold, and depressible edema, with toes that were hypertrophic and increased in length. The first toes had periungual swollen and painful margins. Diminished muscular trophism prevailed on the arms. She had syndactyly of the se