Superficial migratory thrombophlebitis. Thromboangiitis obliterans (Buerger's disease)

ARTICLE

Histopathology showed a thrombosed vein at the dermal-subdermal junction (Fig. 3, arrows). An inflammatory infiltrate of lymphocytes, histiocytes, neutrophils and giant cells was present both in the thrombus and in the vessel walls (Fig. 3). Arteriography evidenced the existence of a unique tibial posterior trunk, which was distally occluded. In the ankle there was a rich collateral circulation (Fig. 4) with "corkscrew" images (Fig. 4, arrows).

Evolution: When the patient came for follow up two months later these skin lesions had spontaneously vanished and new painful nodular lesions had developed on the base of the left big toe with linear configuration. The patient gave up smoking and since then he has remained asymptomatic.

Discussion

It is a case of superficial migratory thrombophlebitis which led us to make a diagnosis of thromboangiitis obliterans or Buerger's disease. Superficial migratory thrombophlebitis is an entity characterized by painful and infiltrated skin lesions which spontaneously vanish within 1-2 weeks and subsequently appear on other locations (hence the denomination "migratory"). Its histopathological picture (inflammation of venular wall with occlusion of the lumen by a thrombus) is indistinguishable from that of other superficial thrombophlebitis. The causes of migratory thrombophlebitis are the following: hypercoagulable states, varicose veins, thromboangiitis obliterans, Behçet's disease, chronic bacterial infections, pregnancy, neoplasms, and traumatisms [1, 2]. Migratory thrombophlebitis occurs in 38% of cases of thromboangiitis obliterans [3, 4].

Thromboangiitis obliterans or Buerger's disease is a segmental inflammatory vascular disease involving small and medium-sized arteries and veins with formation of thrombi that occlude the vessels. It generally affects young male smokers. The etiology and pathogenesis are unknown, several factors being involved [5]: tobacco (which would act as a direct toxin or by vasoconstriction), genetic factors (in Spain it has been associated with HLA-B35 and HLA-B40) [4], and autoimmune factors (anticollagen antibodies). Furthermore, there are cases associated with deficiencies of coagulation cascade factors (proteins C and S) [6].

The diagnosis of Bueger's disease is basically made on clinical grounds. Several series of diagnostic criteria have been proposed. Mills et al. [7] pointed out as major criteria 1) distal ischemia starting before 45 years of age, 2) tobacco abuse, 3) exclusion of other causes of ischemia (atherosclerosis, autoimmune disease, embolism, hypercoagulable state), 4) undiseased arteries proximal to popliteal or distal brachial level, and 5) objective documentation of distal occlusive disease by arteriography, plethysmography, or histopathology; and as minor criteria 1) migratory superficial thrombophlebitis, 2) Raynaud's syndrome, 3) upper extremity involvement and 4) instep claudication. The major criteria are essential to establish the diagnosis, while the minor criteria support it. Although the diagnosis is basically clinical, both histopathology and arteriography may suggest it.

Histopathology

Features described as very characteristic of thromboangiitis obliterans, but not pathognomonic, are inflammation and thrombosis of small and medium-sized arteries and veins, with giant cells and microabscesses within the thrombi. These findings can be seen in lesions of superficial migratory thrombophlebitis [8].

Arteriography

The involvement of distal arteries with distal occlusions and the occurrence of collaterals which can adopt "corkscrew" configurations, is characteristic. Proximal arteries of limbs must not be affected, as their involvement points to a diagnosis of atherosclerosis [5, 7].

CONCLUSION

In conclusion, cutaneous signs are fundamental for suspecting the diagnosis of Buerger's disease and can be its first manifestation. Acral ischemic lesions and episodes of thrombophlebitis in a young smoker must make us think of this entity.

Article accepted on 5/3/00

REFERENCES

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