2.2.8 Erythema (Chronicum) Migrans
ICD-11
1C1G.0
Synonyms
Stage 1 Lyme disease; early cutaneous Lyme borreliosis; Erythema migrans.
Epidemiology
In endemic areas in northern, middle and eastern Europe up to 20% of Ixodes ricinus are infected by B.afzelii, B.garinii, B.Spielmanii and Borrelia burgdorferi sensu strictu. In the USA B.burgdorferi sensu strictu is most common. After a tick bite from an infected Ixodes, in 5% a local infection appears and in up to 2%, if not treated, a manifestation of a borreliosis take place. About 100 -200 cases / 100.000 people per year in Germany, in Austria or Slovenia even higher up to 400 cases. Individuals at special risk: farmers, joggers, hikers, dog owners, forest workers.
Definition
Early target like and annular, then centrifugally spreading erythema with a prominent margin.
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Early target like and annular, then centrifugally spreading erythema with a prominent margin either local or disseminated.
Aetiology & Pathogenesis
Ixodes inserts (no bite!) hypostoma into the upper dermis. Factors help stabilizing its position and to open vessels and avoiding blood clotting when starting to suck. Because of osmotic balance saliva with borrelia is deposited in the skin. Borrelia organisms are 20 µm long and 3µm thick, multiple antigenic structures (853). Flagellin (endoflagellins up to 12 different structures) and outer surface lipoproteins OspA - G. The innate immune system reacts i.p. on the local site and the regional lymph-node with a strong B-cell production of antibodies. Serological detection usually occurs by the third week after exposure.
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The transfer of Borrelia spp occurs mostly via the tick sting from Ixodes ricinus. The most prominent reservoirs are birds, mice, hedgehogs and foxes. Patients report a history of a sting in less than 10% of cases. After an Ixodes nymph or adult arthropod has inserted (not a bite!) its hypostoma into the upper dermis it releases some factors to help stabilizing its position and to open vessels and avoiding blood clotting when starting to suck. Because of osmotic balance, saliva with borrelia is first deposited in the skin. The reservoir of borrelia is almost entirely in the intestinal fluid of the Ixodes which is released into the skin in parallel or later. Borrelia organisms are 20µm long and 3µm thick. They have multiple antigenic structures (853) to which the innate immune system reacts. The most important are the flagellin (endoflagellins up to 12 different structures) and outer surface lipoproteins OspA - G. The innate immune system reacts i.p. on the local site and the regional lymph node with a strong B-cell production of antibodies. Serological detection usually occurs by the third week after exposure.
Signs & Symptoms
- Incubation period: 1-4 weeks.
- Skin: sharply bordered erythematous ring with prominent border (sometimes with central hemorrhagic area), spreads peripherally and clears centrally, mostly 10 - 30 cm depending on body site.
- Center variable: pale, erythematous, hemorrhagic, vesicular. Multiple annular erythemas possible. Systemic symptoms can also occur: headache, neck pain, arthralgias, fever.
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Incubation period: 1-4 weeks.
Skin: sharply bordered erythematous ring with prominent border (sometimes with central hemorrhagic area), spreads peripherally and clears centrally, mostly 10 - 30 cm depending on body site.
Center variable: pale, erythematous, hemorrhagic, vesicular. Multiple annular erythemas possible due to multiple stings, then more symptomatic. Sting from the nymphae in early spring often overlooked and no symptoms, later in summer adult Ixodes stinges can make pain, the organism is much bigger.
Systemic symptoms can also occur: headache, neck pain, arthralgias, fever depending on persisting infection and dissemination of organism and immune response.
Localisation
Preferentially: knee, lower leg, inguinal and buttocks, lumbar area, axilla, shoulder, chest, neck, auricle, mamilla.
Classification
Stage 1 early localized, stage 2 early disseminated, stage 3 late disseminated.
In stage 1 ,2 and 3 an overlap can occur. ( see also chapter 2.2.10 Acrodermatitis Chronica Atrophicans; 2.2.9 Pseudolymphomas)
Laboratory & other workups
Serology with IgM and IgG and additional early antigen markers in the immunoblot (p41, flagellin, OspE, Osp C ) after about 3 weeks. Late stage Osp 17, A, B, p43.
Dermatopathology
Biopsy from the margin. Superficial dermal lymphocytic infiltrate with perivascular accumulation. Later more perineural and periglandular infiltrates and plasma cells appear. Initially some eosinophils.
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A biopsy is taken from the margin. Superficial dermal perivascular lymphocytic infiltrate. The longer the infection persists the more perineural and periglandular infiltrates and plasma cells appear. Initially some eosinophils. Warthin-Starry stain and immunohistochemistry can help to detect the microbe in tissue.
Course
See classification. If not treated, dissemination may occur and manifestations in other organ systems take place: joints, muscles, peripheral and central nervous system, heart.
Complications
Meningoradiculitis, Guillain Barré syndrome, arthritis, myocarditis (conduction disturbances), encephalopathy.
Diagnosis
History, clinical features, PCR from lesion: borrelia DNA; borrelia serology initially negative.
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History, clinical features, PCR from lesion: borrelia DNA; borrelia serology initially negative, not earlier than after 3 weeks should be tested.
Differential Diagnosis
Other arthropodes (spider), urticaria, erythema and granuloma annulare, other figurate erythemas incl. fixed drug eruption, tinea, erysipelas and erysipeloid.
Prevention & Therapy
Doxycycline 100 mg b.i.d. for 14 days. Alternatively, Amoxicillin 3 x 500 to 1000 mg/d 2 weeks. Azithomycin 2 x 250 mg / d for 10 days.
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Prevention: After outdoor activities in garden, walking in the forest and lying at a meadow skin body check and early removal of Ixodes. No squeezing, use of special scissors. Parents should check their children’s skin every evening when playing in areas where Ixodes is present.
Dogs should be inspected too.
Therapy: Doxycycline 100 mg b.i.d. for 14 days. Alternatively, Amoxicillin 3 x 500 to 1000 mg/d 2 weeks. Azithomycin 2 x 250 mg/d for 10 days.
Special
None.
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