June 2013

Fig.4

Fig.5

 

Fig.6

 

 

                                                                       Fig.7

 

 


Diagnosis: Cutaneous manifestation of Lyme disease

Histology: Biopsy taken from the left 3rd finger lesion showed lymphoid hyperplasia with a predominant T-cell population (Figs 4 and 5). The Warthin Starry stain (Fig 6) and Treponemal immunostain (Figs.7) appear to stain rare spiraling organisms suspicious for spirochetes.

Clinical Course: Although there was no known tick bite in our patient, he reported a trip to rural northern New York several months preceding his initial symptom onset.  In New York the incidence of Lyme disease is significantly higher (12.3-29.5 per 100,000 persons incidence rate from 2001-2011 vs 0.1-1.2 in Missouri and 0.4-1.2 in Illinois).  Due to the small size of Ixodes nymphs many patients with Lyme disease do not recall an actual tick bite, but will present with erythema migrans or less commonly flu like symptoms without skin findings.  The patient was given oral doxycycline 100mg twice a day for 2 weeks and his skin lesions have improved markedly.

Discussion: Lyme disease is a multisystem disorder caused by several Borrelia spirochete species most notably B. burgdorferi in the US and B. afzelii and garinii in addition to B. burgdorferi in Europe.  It is transmitted by the Blacklegged tick vector species Ixodes scapularis, pacificus, and ricinus whose natural hosts include white-footed mice and white-tailed deer.  At least 24-72 hours of human-tick contact is required to transmit the bacteria.  Lyme disease is divided into three clinical stages that include early localized, early disseminated, and chronic disease.  Up to 60-90% of Lyme disease patients present with erythema migrans (early localized disease). 

Less commonly it may, in its chronic phase, present as acrodermatitis chronica atrophicans (ACA) or borrelial lymphocytoma both of which are seen almost exclusively in Europe.  ACA is more commonly due to B. afzelii rather than B. burgdorferi or garinii and therefore is extremely rare in the US, but present in up to 10% of European Lyme disease cases.  ACA is a result of long-term persistence of the offending organisms in the skin and presents early as treatment-responsive erythematous to violaceous plaques and nodules on the acral extremities.  The skin may be doughy or swollen.  After a period of weeks to years the skin changes to a treatment-resistant phase that is atrophic with a “cigarette-paper” appearance and prominent blood vessels. 

Borrelial lymphocytoma is a benign lymphoproliferation appearing as a bluish-red nodule or plaque classically on the earlobe or nipple areola.  Only B. afzelii and B. garinii are known to cause lymphocytoma so it is seen only in individuals who have travelled outside the US.

Proper treatment with antibiotics is required to prevent early disseminated and chronic disease that differ slightly depending on the Ixodes species involved.  Possible neurologic impairments include cranial neuritis, Bell’s palsy, headaches, neuropathy, meningitis, encephalopathy, and encephalomyelitis.  Cardiac complications may occur in the form of atrioventricular block, myopericarditis, and dilated cardiomyopathy.  Other possible complications include iritis, conjunctivitis, and hepatitis.

In this case the patient was given oral doxycycline 100mg twice a day for 2 weeks and his skin lesions have subsequently improved.

References:

 

This case is presented by: Eddie Mount, M.D., Susan Bayliss, M.D., and Alejandro Gru, M.D.