| Abstract: | We present here an exceptional case of cutaneous and vascular sarcoidosis associated with lesions of the liver, spleen and lymph nodes. The disease began when this male patient was 27 years' old and gradually extended over 35 years, despite long-term systemic corticosteroid therapy. Clinically, the initial cutaneous lesions were atrophic, erythematous and squamous, resembling those of erythroderma; they were located on the skin of the right popliteal fossa, the left arm, the neck, the upper part of the chest and around the waist. Subsequently, they spread slowly to involve almost the entire skin, except for the face, right upper limb, left lower limb, hands and feet. The most ancient of these lesions were distinctly black. The skin was paper thin due to complete disappearance of the subcutaneous tissue. Left temporo-parietal alopecia of the cicatricial type developed towards the end of the patient's life. The nails remained normal throughout, and there were no other cutaneous signs of sarcoidosis. Initially, the venous network was clearly visible beneath the atrophic skin, but later on, the veins became grossly dilated and sacculated in those areas which had first been invaded by skin atrophy. Phlebography of the left upper limb confirmed the venous dilatation, while arteriography of the upper limbs showed multiple sacciform aneurysms of the subclavian, axillary and brachial arteries, with distal thrombosis of the latter vessel. Histology showed typical lesions of sarcoidosis in a vein; no arterial biopsy was performed. The muscles had a nodular and sclerous appearance; amyotrophy developed in the last stages of the disease; tendons and joints were normal.(ABSTRACT TRUNCATED AT 250 WORDS) |
