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CASE REPORT
Year : 2022  |  Volume : 15  |  Issue : 5  |  Page : 752-755  

Disseminated secondary erythema migrans: Typical and atypical presentation of a rare disease in India


1 Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India
2 Department of Dermatology, Command Hospital Air Force, Bengaluru, Karnataka, India
3 Department of Pathology, Armed Forces Medical College, Pune, Maharashtra, India

Date of Submission02-Sep-2020
Date of Decision18-Dec-2020
Date of Acceptance21-Dec-2020
Date of Web Publication17-May-2022

Correspondence Address:
Dr. Sunmeet Sandhu
Department of Dermatology, Command Hospital Air Force, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_484_20

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  Abstract 


Lyme borreliosis is a multisystem disease transmitted by the bite of vector, Ixodes tick and caused by the strains of spirochete Borrelia burgdorferi sensu lato. The Centers for Disease Control and Prevention has listed it as a reportable disease since 1991. Although the three classical cutaneous manifestations are primary erythema migrans, borrelial lymphocytoma, and acrodermatitis chronica atrophicans, secondary disseminated erythema migrans is a rare presentation. Reported atypical erythema chronicum migrans (ECM) lesions include vesicles, erythematous papules, purpura, and lymphangitic streaks. We report a case of a female patient who was clinically, serologically, and histopathologically diagnosed as a case of Lyme disease (LD) with disseminated secondary erythema migrans with atypical edematous lesions. Only four confirmed cases of ECM have been reported in India before this report. The case highlights this atypical and rare presentation of LD in India.

Keywords: Borrelia, doxycycline, erythema chronicum migrans, Ixodes, Lyme disease


How to cite this article:
Pathania V, Sandhu S, Vashisht D, Sengupta P. Disseminated secondary erythema migrans: Typical and atypical presentation of a rare disease in India. Med J DY Patil Vidyapeeth 2022;15:752-5

How to cite this URL:
Pathania V, Sandhu S, Vashisht D, Sengupta P. Disseminated secondary erythema migrans: Typical and atypical presentation of a rare disease in India. Med J DY Patil Vidyapeeth [serial online] 2022 [cited 2022 Dec 10];15:752-5. Available from: https://www.mjdrdypv.org/text.asp?2022/15/5/752/345405




  Introduction Top


Lyme disease (LD) is a multisystem disease transmitted by the bite of vector, Ixodes tick and caused by the strains of spirochete Borrelia burgdorferi sensu lato.[1] Erythema chronicum migrans (ECM) seen at the site of inoculation is the pathognomonic presentation of early disease. Dissemination with serious late manifestations of neurological, cardiovascular, or rheumatological systems can occur.[2] While predominantly seen in temperate regions of the world, the cases are now being reported in India.[1] We present a case of LD with disseminated secondary erythema migrans with atypical edematous lesions.


  Case Report Top


A 36-year-old female presented with multiple red raised itchy scaly lesions over the body of 1-month duration. She initially noticed a coin-sized red raised scaly itchy rash over the left side of the neck which, over the next 3 weeks, increased to palm size with multiple similar smaller lesions over neck, trunk, and upper extremities associated with low-grade intermittent fever and malaise. Travel history revealed hiking to the Western Ghats 3 months back, when she recalled two similar coin-sized lesions over the left forearm which resolved spontaneously over 3 weeks. She denied any history of insect bite, joint pains, headache, palpitations, redness of eyes, pain abdomen, photosensitivity, prior drug intake, or any previous skin ailment.

General examination revealed multiple nontender, mobile, discrete cervical and left axillary lymph nodes, largest measuring 1.5 cm over the left axilla. Systemic examination was unremarkable. Dermatological examination showed multiple well-defined, discrete, erythematous papules and annular scaly plaques, over the neck (left > right), trunk, and both upper limbs, with largest plaque over left side of neck measuring 20 cm [Figure 1]a and b[Figure 1]]. Some of these plaques were edematous [Figure 2]a. There were two hyperpigmented macules over the flexor aspect of the left forearm, probably a sequelae of primary ECM [Figure 2]b. Provisional diagnosis of granuloma annulare, subcutaneous lupus erythematosus, secondary syphilis, ECM, and erythema annulare centrifugum (EAC) were considered. Skin biopsy from the neck lesion revealed unremarkable epidermis with dermis showing intense lymphocytic inflammatory infiltrate admixed with plasma cells and eosinophils predominantly centered around the vessels [Figure 3]a and [Figure 3]b. No palisading granulomas, mucin, or atypical cells were seen. She had a raised erythrocyte sedimentation rate of 22 mm/h. Borrelia IgM antibody titer of 1.1 U/ml was marginally raised; however, IgG antibody titer was markedly raised with 4.7 U/ml (>1.1), indicating a persisting acute infection. Western blot could not be performed due to nonavailability. All other laboratory parameters were normal including serum antinuclear antibody titer, venereal disease research laboratory titer, and serum Treponema pallidum hemagglutination (TPHA), which were not raised ruling out differential diagnosis considered here. She was hence diagnosed as LD with disseminated secondary ECM and managed with 21 days of capsule doxycycline 100 mg twice a day. All lesions resolved leaving hyperpigmentation over the next 4 weeks. The patient was on follow-up for the next 6 months without any cutaneous or systemic sequelae.
Figure 1: (a and b) Multiple well-defined, discrete, erythematous papules and annular scaly plaques, seen over the neck and upper limb. In the inset is the largest lesion over the left side of the neck measuring 20 cm

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Figure 2: (a) Few of the atypical edematous plaques seen over neck. (b) Two hyperpigmented macules present over flexor aspect of left forearm, suggestive of sequelae of primary erythema chronicum migrans (yellow arrow)

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Figure 3: (a and b) Intense perivascular lymphocytic inflammatory infiltrate admixed with plasma cells and eosinophils seen in the dermis. (a: H and E, ×200, b: H and E, ×400)

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  Discussion Top


The causative agent of LD is pathogenic species of B. burgdorferi sensu lato which are said to be present in the Himalayan region.[3] Cutaneous manifestations of LD are called as “dermatoborreliosis” and include three stages: early localized disease (primary ECM), disseminated disease (secondary ECM and borrelial lymphocytoma), and chronic disease (acrodermatitis chronic atrophicans).[1] Primary ECM is the pathognomonic feature seen in 60%–90% of LD patients that develops at the site of tick bite within 7–15 days after tick detachment. It favors trunk, axilla, groin, and popliteal fossa with various hues of erythema spreading centrifugally with central clearing and tick bite mark at the center, giving a bull's eye appearance.[2] As per case definition by the Centers for Disease Control and Prevention (CDC), a single primary lesion should be ≥5 cm in size across its largest diameter.[4] However, multiple lesions may occur in 20%–25% cases due to multiple tick bites or spirochetemia. ECM can be associated with burning sensation, pain or pruritus, and constitutional symptoms such as fever, myalgia, headache, flu-like symptoms, or lymphadenopathy. Spontaneous resolution is usually seen within 6 weeks.

Prevalence of secondary ECM varies between 17% and 57%.[1] These occur due to hematogenous or lymphatic dissemination of Borrelia, thereby presenting at sites distant from that of tick bite favoring face and extremities. Lesions are typically smaller with less symptoms, edema, erythema, and central clearing when compared to primary ECM. Our patient presented with similar lesions over the neck and upper extremities approximately 8 weeks after resolution of primary ECM. It may be associated with regional lymphadenopathy and low-grade fever as seen in our patient. If left untreated, secondary ECM resolves spontaneously over few months. Clinical differential diagnosis includes tinea corporis, urticaria, fixed drug eruption, erysipelas, figurate erythema-like EAC, and erythema gyratum repens.

Histopathology is not specific but usually shows perivascular dermal lymphoid infiltrate along with plasma cells and eosinophils.[2] Spirochetes may be demonstrated with Warthin–Starry stain in 50% of primary ECM and in 100% of secondary ECM lesions. Patients with secondary ECM are usually seropositive.[1] Polymerase chain reaction analysis can be done to confirm presence of Borrelia within the tissue specimen.[2] The 2017 Revised CDC case definition of LD categorizes cases as “confirmed,” “probable,” and “suspected” based on the presence of ECM and laboratory evidence of infection.[4] Laboratory evidence includes isolation of Borrelia from tissues or detection of anti-Borrelia antibodies by two-test approach involving an initial screening test (usually ELISA) followed by a Western blot for reactive and equivocal samples.[4] Nonavailability of Warthin–Starry stain for spirochaete and western blot limited the definite confirmation of the diagnosis.

Early treatment is highly effective during LD and prevents serious late neurological, cardiovascular, or rheumatological sequelae. Oral doxycycline given as 100 mg twice daily for 14–21 days is the drug of choice in ECM. Azithromycin, amoxicillin, cefuroxime, and erythromycin are other options. Disseminated disease usually requires parenteral treatment with ceftriaxone, cefotaxime, or benzyl penicillin for 14–21 days.[2] Only few cases of LD with ECM have been reported in India till date, with four as confirmed ECM and five cases reported in a print media as summarized in [Table 1].[5],[6],[7],[8],[9] Seroprevalance of borrelial antibodies has been reported to be 13% and 19.9% in the Northeastern states of India and in the forest areas of Nagarahole and Bandipur ranges in South India respectively. This highlight the prevalence of LD in India however, the cases might be asymptomatic to mildly symptomatic in presentation resulting in their possible under reporting.[10],[11] Although no specific species of ticks causing LD in India have been recognized, the Western Ghats in India could be considered a favorable environment for the tick, as they favor spring to autumn microenvironments with more than 85% relative humidity, more so with recent interest in trekking and hiking as recreational activities in this cooler and woodland areas.[12] While this could explain the emergence of a case from this region particularly, there is also a possibility of under reporting of cases as we do not routinely test for LD in India.
Table 1: Summary of cases of Lyme disease presented with erythema chronicum migrans from India

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  Conclusion Top


The present case had disseminated secondary ECM with edematous lesions which is a rare and atypical presentation. It also emphasizes the emerging risk of LD in the Western Ghats region and warrants awareness of this entity among the physicians. Although we do not routinely test for LD in India, it is important to consider its serology in investigation panel, especially in a patient from a population at risk with characteristic symptoms and signs of LD with history of tick exposure.

Acknowledgment

We are thankful to Dr Niranjan Pudasaini, Resident Dermatology, Armed Forces Medical College, Pune, for technical help.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Vasudevan B, Chatterjee M. Lyme borreliosis and skin. Indian J Dermatol 2013;58:167-74.  Back to cited text no. 1
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2.
Summerly R. The figurate erythemas and neoplasia. Br J Dermatol 1964;76:370-3.  Back to cited text no. 2
    
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Geevarghese G, Fernandes S, Kulkarni SM. A checklist of Indian ticks (Acari: Ixodoidea). Indian J Animal Sci 1997;67:17-25.  Back to cited text no. 3
    
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Lyme Disease | 2017 Case Definition. Available from: https://wwwn.cdc.gov/nndss/conditions/lyme-disease/case-definition/2017. [Last accessed on 2020 Apr 05].  Back to cited text no. 4
    
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Sharma A, Guleria S, Sharma R, Sharma A. Lyme disease: A case report with typical and atypical lesions. Indian Dermatol Online J 2017;8:124-7.  Back to cited text no. 5
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Kandhari R, Kandhari S, Jain S. Borrelial lymphocytoma cutis: A diagnostic dilemma. Indian J Dermatol 2014;59:595-7.  Back to cited text no. 6
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Jairath V, Sehrawat M, Jindal N, Jain VK, Aggarwal P. Lyme disease in Haryana, India. Indian J Dermatol Venereol Leprol 2014;80:320-3.  Back to cited text no. 7
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Baveja S, Oberoi B, Vashisht D, Das P. Lyme disease – A report of atypical cutaneous Sequelae. Indian Dermatol Online J 2019;10:336-7.  Back to cited text no. 8
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Lyme Disease Outbreak in Wayanad | Kozhikode News – Times of India. Available from: https://timesofindia.indiatimes.com/city/kozhikode/Lyme-disease-outbreak-in-Wayanad/articleshow/18758675.cms. [Last accessed on 2020 Apr 05].  Back to cited text no. 9
    
10.
Praharaj AK, Jetley S, Kalghatgi AT. Seroprevalence of Borrelia burgdorferi in North Eastern India. Med J Armed Forces India 2008;64:26-8.  Back to cited text no. 10
    
11.
Babu K, Murthy KR, Bhagya M, Murthy PR, Puttamallesh VN, Ravi V. Seroprevalence of Lymes disease in the Nagarahole and Bandipur forest areas of South India. Indian J Ophthalmol 2020;68:100-5.  Back to cited text no. 11
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Izzoli A, Hauffe H, Carpi G, Vourc HG, Neteler M, Rosà R. Lyme borreliosis in Europe. Euro Surveill 2011;16:19906.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]



 

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