Southern tick-associated rash illness
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Synopsis

Southern tick-associated rash illness (STARI) was first described in the mid-1980s in Missouri and the Southeastern United States. It is also known as Southern Lyme disease, lone star tick-vectored Lyme-like illness, and Masters' disease.
STARI is a tickborne syndrome, vectored by the lone star tick (Amblyomma americanum). One case report described the spirochete Borrelia lonestari as the causative agent of STARI, but after extensive research, it has not been reproduced. Therefore, the etiology of STARI has not been confirmed, and this is merely speculation.
The disease is most commonly seen in the Southeastern and South Central United States, where A americanum ticks frequently bite humans. Cases are now being reported in several other regions. This disease should be considered an emerging health threat.
The estimated mean incubation period varies from 6.1 days to 9.2 days. The syndrome is characterized by an erythematous skin lesion with a central clearing, similar to erythema migrans, at the site of a lone star tick bite. These lesions may be single or multiple. Other symptoms include headache, fatigue, and myalgias.
CBC, basic metabolic panel, and liver function tests are usually unremarkable.
Treatment with antibiotics is recommended, and all symptoms resolve with tetracycline or amoxicillin.
Long-term sequelae or life-threatening complications have not been identified to date, but long-term follow-up studies evaluating the outcome following recovery from acute illness are lacking. Bites from A americanum have been associated with the subsequent development of allergies to mammalian meat (eg, beef, pork) in a small number of patients. It is thought that the allergy is mediated by induced immunoglobulin E (IgE) antibodies to alpha-gal (galactose-alpha-1,3-galactose), a mammalian oligosaccharide. Individuals with elevated IgE titers to alpha-gal have experienced urticaria, angioedema, and anaphylaxis symptoms either immediately or 3-6 hours (delayed onset) after ingesting mammalian meat (alpha-gal syndrome). Exactly how the tick bite leads to development of this allergy is unclear. Implicated tick bites have been noted to itch for 2 or more weeks. A blood test for these IgE antibodies exists.
STARI is a tickborne syndrome, vectored by the lone star tick (Amblyomma americanum). One case report described the spirochete Borrelia lonestari as the causative agent of STARI, but after extensive research, it has not been reproduced. Therefore, the etiology of STARI has not been confirmed, and this is merely speculation.
The disease is most commonly seen in the Southeastern and South Central United States, where A americanum ticks frequently bite humans. Cases are now being reported in several other regions. This disease should be considered an emerging health threat.
The estimated mean incubation period varies from 6.1 days to 9.2 days. The syndrome is characterized by an erythematous skin lesion with a central clearing, similar to erythema migrans, at the site of a lone star tick bite. These lesions may be single or multiple. Other symptoms include headache, fatigue, and myalgias.
CBC, basic metabolic panel, and liver function tests are usually unremarkable.
Treatment with antibiotics is recommended, and all symptoms resolve with tetracycline or amoxicillin.
Long-term sequelae or life-threatening complications have not been identified to date, but long-term follow-up studies evaluating the outcome following recovery from acute illness are lacking. Bites from A americanum have been associated with the subsequent development of allergies to mammalian meat (eg, beef, pork) in a small number of patients. It is thought that the allergy is mediated by induced immunoglobulin E (IgE) antibodies to alpha-gal (galactose-alpha-1,3-galactose), a mammalian oligosaccharide. Individuals with elevated IgE titers to alpha-gal have experienced urticaria, angioedema, and anaphylaxis symptoms either immediately or 3-6 hours (delayed onset) after ingesting mammalian meat (alpha-gal syndrome). Exactly how the tick bite leads to development of this allergy is unclear. Implicated tick bites have been noted to itch for 2 or more weeks. A blood test for these IgE antibodies exists.
Codes
ICD10CM:
A69.8 – Other specified spirochetal infections
SNOMEDCT:
444100007 – Southern tick-associated rash illness
A69.8 – Other specified spirochetal infections
SNOMEDCT:
444100007 – Southern tick-associated rash illness
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Differential Diagnosis & Pitfalls
Lyme disease can also present with erythema migrans (the bull's-eye skin lesion). However, the geographic location and vector of the 2 illnesses are different. The tick vectors for Lyme disease are Ixodes scapularis and Ixodes pacificus, which are widely distributed in the Northeast, Midwest, and Pacific Northwest of the United States. Unlike Lyme disease, STARI is most commonly seen in the Southeastern and South Central United States.
The skin rash in Lyme disease is classically more common on the extremities, and the target-like lesion has a mean diameter of 7 cm, whereas in STARI the skin lesions are often on the trunk and tend to be smaller, with a mean diameter of 4.5 cm.
In STARI, leukopenia, thrombocytopenia, anemia, and transaminitis are typically absent, while these abnormal labs are common findings in patients with Lyme disease.
Histology of the skin lesion in Lyme disease tends to show abundant plasma cell infiltrates, while in STARI lymphocytic infiltration predominates.
The diagnosis of Lyme disease by methods such as ELISA, immunofluorescent antibody test (IFA), and Western blot is useful and excludes STARI, which remains a clinical diagnosis.
The skin rash in Lyme disease is classically more common on the extremities, and the target-like lesion has a mean diameter of 7 cm, whereas in STARI the skin lesions are often on the trunk and tend to be smaller, with a mean diameter of 4.5 cm.
In STARI, leukopenia, thrombocytopenia, anemia, and transaminitis are typically absent, while these abnormal labs are common findings in patients with Lyme disease.
Histology of the skin lesion in Lyme disease tends to show abundant plasma cell infiltrates, while in STARI lymphocytic infiltration predominates.
The diagnosis of Lyme disease by methods such as ELISA, immunofluorescent antibody test (IFA), and Western blot is useful and excludes STARI, which remains a clinical diagnosis.
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Last Updated:05/16/2023