Borrelia burgdorferi and localized scleroderma

Abstract

L yme borreliosis is a multisystemic disorder caused by the spirochete Borrelia burgdorferi and transmitted by the Ixodes tick. The initial manifestations of the disease are the characteristic skin lesion, erythema migrans, and symptoms of general malaise (stage I). This may be followed weeks to months later by acute articular, cardiac, and neurologic manifestations (stage II). Months to years later intermittent or continuous attacks of mono/oligoarthritis may occur (stage III). Cutaneous manifestations occur in all three stages of the disease; erythema migrans in stage I and lymphadenosis benigna cutis in stage II. Stage III is associated with acrodermatitis chronica atrophicans (ACA);lb4 although many other scleroatrophic skin conditions have been reported as possible late manifestations of Lyme borreliosis. These include lichen sclerosus et atrophicus,5*6 morphea,z*3,6-12 linear scleroderma,g disseminated localized scleroderma,13 generalized scleroderma,14 morphea profunda,15 atrophoderma Pasini Pierini,16~17 eosinophilic fasciitis (or Shulman’s syndrome),ls,lg erythema chronicum migrans et atrophicans,20 progressive facial hemiatrophy of Parry-Romberg,21 and porphyria cutanea tarda with sclerodermic alterations.22 In contrast to acrodermatitis chronica atrophicans, however, the relationship of these conditions to Borrelia burgdorferi infection is uncertain. In 1985 Aberer et al.’ suggested for the first time that morphea may be a late manifestation of Lyme borreliosis; however, since then there have been numerous conflicting reports.