Diagnosis: Acrodermatitis chronica atrophicans.
The patient’s photograph of her skin rash taken 3 years ago,
which clearly showed that she had erythema chronicum
migrans (ECM) at that time, immediately prompted the diagnosis
of Lyme disease in the form of acrodermatitis chronica
atrophicans (ACA). The histology of the skin biopsy was compatible
with this diagnosis, which was confirmed with serology
and polymerase chain reaction (PCR). An enzyme-linked immunosorbent
assay showed strongly elevated serum antibodies
(immunoglobulin G [IgG]) to Borrelia burgdorferi (LiaisonDiaSorin;
detection of immunoglobulin against B. burgdorferi,
Borrelia afzelii, and Borrelia garinii; IgG >240 UA/mL). Western
blotting (Biognost, Borrelia Euroline-WB) detected bands
positive against VlsE, p83, p39, p30, and p21 antigens [1]. PCR
on the skin biopsy sample (primer sets targeting 23S rDNA;
TaqMan) was also positive.
The patient was treated for 4 weeks with 100 mg of doxycycline
twice a day. Six months later, she had no more lesions.
Lyme borreliosis is caused by tick-transmitted spirochetes of
the B. burgdorferi sensu lato complex. Although B. burgdorferi
sensu stricto is the only species known to cause human disease
in North America, at least 5 species can cause the disease
in Europe: B. afzelii, B. garinii, B. burgdorferi sensu stricto,
Borrelia spielmanii, and Borrelia bavariensis. The clinical
symptoms vary widely and depend on the species; some have
been described only in Europe [2].
ACA appears to be due only to B. afzelii [3]. This dermatological
entity is a rare tertiary manifestation of Lyme disease,
manifesting as inflammatory and trophic lesions on acral skin.
After an early inflammatory stage with bluish-red discoloration
and doughy swelling of the skin, a late atrophic stage appears a
few weeks or months later. The skin becomes thin, wrinkled,
dry, and transparent because of the loss of epidermal and
dermal structures. Vessels may be easily visible, and telangiectasias
can be observed.
The diagnosis is suggested by dermatologic lesions and a clinical
history of tick bites or other well-defined manifestations of
Lyme borreliosis, such as ECM, shown in our patient’s picture.
Confirmation of the diagnosis is obtained by serological testing
(enzyme immunoassay and Western blotting). These methods
might increase diagnostic accuracy over that of PCR, which has a
sensitivity of about 50%, depending on primer set [4].
Treatment of ACA is usually based on a course of antibiotic
treatment with ceftriaxone [5] or doxycycline [6] for 21–28 days.
Complete disappearance of lesions is normally described [7, 8].
The absence of treatment can lead to fibrotic nodules and/or
patchy or bandlike indurations that may limit joint movement
without treatment.
Clinical Infectious Diseases 2013;57(12):1782
DOI: 10.1093/cid/cit667