A 2-year-old male patient, from Arboletes, Antioquia (Colombia), with no significant family history.
It presents a congenital lesion of 50x40cm in diameter at the level of the posterior thoracic region, lumbosacra region, lateral region of the abdomen and extremities, in a satelital form, gray brown and with borders.
The surface of the lesion presents thick, semi-blended, woody skin with deep grooves that give it a colored appearance, absence of cutaneous annexes and intense pruritus associated.
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The Lund and Browder table used for body surface area balance in affected patients was used as a reference to approximate the body surface area affected by the nephrotic syndrome.
According to this table, the patient had a 25.7% compromise of his body surface.
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Physical examination revealed hepatomegaly, axillary and inguinal lymphadenopathy and associated systolic murmur; no neurological involvement was observed.
The child was recruited from a plastic surgery outpatient clinic at Engativa II Hospital E.S.E (State Social Company), Fundación Cardiorel, Colombia.
Among the diagnostic studies carried out in the Fundación Fundación Infantil are: two skin biopsies, the one obtained from the skin of the back with histopathological report of compromised margins due to congenital intradermal resection.
The second biopsy was taken from a hyperpigmented nodular lesion with caustic consistency located in the left knee, with a report of necrotic arthritis completely composed.
The specimens studied showed a pattern with cellular architecture of predominantly clusters of histological reticular cells (9.10), circumscribed predominantly intra-, hyperpigmented, within a superficial dense dermis.
Seizures are associated with inflammatory cells as a rapid growth response in a fibroblastic stroma.
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Due to the presence of hepatosinusomegaly on physical examination, imaging studies were performed including:
-Abdominal magnetic resonance angiography and MR angiography showed mild inguinal hepatomegaly and adenomegaly, with no findings suggesting vascular involvement in the right posterolateral part of the abdominal wall.
Portal Doppler ultrasound showed a slight increase in resistance indices of the hepatic artery, with no signs of portal hypertension, hepatosplenic enlargement.
Echocardiogram showed mild left ventricular dilation with preserved function.
Brain magnetic resonance within normal limits showed no leptomeningeo involvement.
-Bone scintigraphy showed no evidence of metastatic disease or primary extension.
-Hemogram: microcytic, hypochromic, heterogeneous anemia.
Establishment of evidence of malignant transformation or leptomeningeos involvement; discharge of the patient with an appointment for periodic check-ups;
