Segmental ulcerative vasculitis: a cutaneous manifestation of Takayasu's arteritis

Abstract

A 16-year-old girl with pyoderma gangrenosum (PG)-like skin lesions on the extremities, trunk and face developed Takayasu's arteritis (TA; pulseless disease). After 3 years under maintenance cyclosporin A therapy, the patient developed an ischaemic cerebral accident. Severe obstruction of both subclavian and left carotid arteries was found by Doppler sonography, angiography and computerised axial tomography. Evolution of this disease showed some characteristic findings: (a) PG-like lesions as the first cutaneous manifestation of pulseless disease; (b) methotrexate and cyclosporin A giving good results for the cutaneous lesions, but apparently not exerting an influence on the evolution of TA and the fatal outcome. This morphologic pattern may reflect underlying TA or Wegener's arteritis, and should be termed segmental ulcerative vasculitis.

Figure 1

Numerous pustules and large superficial ulcers with crusts and inflamed borders on the right hand and cubital joint (possible pathergy phenomenon after venous injections).

Figure 2

Ulcer on the right side of the soft palate (possible pathergy phenomenon after incision).

Figure 3

Multiple symmetrical large superficial ulcers with crusts and inflamed borders on both legs and in dermatomal distribution on abdominal skin.

Figure 4

Epidermal subcorneal pustule with peripheral spongiosis and moderate perivascular infiltrate of lymphocytes and plasma cells in dermis. Haematoxylin – eosin, original magnificent ×100.

Figure 5

Pyoderma‐like lesions on the face, neck and upper trunk.

Figure 6

Cribriform scars and anetoderma at the under arms and flexural contractures in axillae and cubital joints.

Figure 7

Severe obstruction of both subclavian and left carotid arteries and formation of small vessels of aortic arch syndrome documented by angiography.