Cutaneous collagenous vasculopathy is a rare acquired idiopathic microangiopathy
characterized by progressive development of diffuse asymptomatic telangiectasias
and histologically by accumulation of collagen type IV around the affected
vessels. It is diagnosed by its clinical history, confirmed by light microscopy
with collagen-specific immunostaining. We report a case of a patient with
extensive acquired telangiectasias on the left arm, clinically resembling
unilateral nevoid telangiectasia. Dilated blood vessels with thickened walls
were observed in the dermis. Immunohistochemistry with collagen IV antibodies
revealed marked collagen deposition around the vessels, confirming the
diagnosis. Transmission electron microscopy observed duplicate and triplicate
vascular basal membrane associated with deposition of amorphous material around
the membranes.
The Osler-Weber-Rendu syndrome or Hereditary Hemorrhagic Telangiectasia (HHT) is a
systemic fibrovascular dysplasia characterized by defects in the elastic and vascular
walls of blood vessels, making them varicose and prone to disruptions. Lesions occur
in different organs and can lead to hemorrhage in the lungs, digestive tract and
brain. We describe the case of a patient with cutaneous manifestations and severe
impairment of the digestive tract. It is important for the dermatologist to recognize
this syndrome, since the cutaneous lesions may play a key role in diagnosis.
We report a case of erythropoietic protoporphyria (EPP) in a young male adult, who was admitted to the hospital due to abdominal pain and jaundice. Complementary exams showed elevation of liver and canalicular enzymes and choledocholithiasis. The patient also had pronounced photodamaged skin and a history of significant photosensitivity since childhood. The dermatology team, based on the clinical and histological findings, gave the diagnosis of EPP and treatment with cholestyramine was instituted.
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