Cutis marmorata telangiectatica congenita is characterized by the presence of a bluish-purple reticulated cutaneous vascular network on the skin intermixed with telangiectasia and occasionally prominent veins at birth. Areas of the skin within the reticulated cutaneous vascular network may be normal, erythematous, atrophic, and, at times, ulcerated. Areas of ulcerations and focal cutaneous and subcutaneous atrophy occasionally occur resulting in body asymmetry. On the other hand, cutaneous and subcutaneous atrophy, extensive and severe enough leading to hemiatrophy, of the entire limb is rare. A search of the English literature revealed only eight documented cases to which we are adding two more cases.
Cutaneous vasculitis presents with a variety of clinical morphologies and causes significant morbidity. A total of 85 patients with cutaneous vasculitis at Hospital Kuala Lumpur were retrospectively reviewed. Palpable purpura was seen in 49.4% and frequently involved the lower limbs (50.6%). Identifiable causes include drugs (28.2%), infections (20.0%) and connective tissue disorders (16.5%). Non steroidal antiinflammatory were the commonest group of drugs responsible for 25% of cases while B-haemolytic streptococci was the leading infectious cause (64.7%).
A 28-year old Malay man with evidence of an upper motor neuron cord lesion was diagnosed to have a C7 to T2 spinal arterio-venous malformation and associated cutaneous vascular lesion. He finally agreed for treatment after 5 years of progressive spastic right lower limb weakness leading to inability to mobilize. A two staged intravascular procedure was done followed by surgery with recovery of ASIA impairment scale grade B.