Borrelial Lymphocytoma Cutis with Septo-Lobular Panniculitis Involving Bilateral Ear Lobes in a Child: A Rare Presentation in a Non-Endemic Region

Dear Editor, Lyme disease (LD) is a tick-borne multisystem illness usually seen in Canada, the United States, and Europe. The causative agent is Borrelia burgdorferi and transmission occurs by ixodid ticks.[1] Borrelial lymphocytoma cutis (BLC) is an uncommon presentation, showing predominantly dermal lymphocytic collection. Dense panniculitis is less commonly reported. We present a case of BLC of both earlobes in a female child residing in a non-endemic region, showing dermal inflammation with dense septo-lobular panniculitis. A 12 years-old-female child presented with plaque-like lesions on both earlobes with redness and edema for 2 months [Figure 1]. The lesions were painless with no history of insect bites. No fever, myalgias, malaise, arthralgias, or flu-like symptoms were noted. There was no history of travel to an area endemic for borreliosis. With a provisional diagnosis of granulomatous dermatitis and lupus panniculitis, a biopsy was taken.Figure 1: (a) Plaque-like lesion on the left ear lobe with redness and edema of the auricle; (b) Lesion on the right ear lobe with swelling and edemaThe dermis showed many hair follicles with moderate inflammatory cell infiltrate composed of lymphocytes and plasma cells. The subcutaneous tissue showed septo-lobular panniculitis comprised of dense polymorphous inflammatory cell infiltrate including lymphocytes, plasma cells, and histiocytes [Figure 2a]. No features of vasculitis, hyaline necrosis of fat, or mucinous edema of septa were noted, thus ruling out lupus panniculitis. No granulomas were seen. Zeihl-Neelsen (ZN) stain for acid-fast bacilli was negative. Focally perineural inflammation was noted [Figure 2b]. Modified ZN stain for lepra bacilli was negative. Immunohistochemistry showed polyclonal lymphoid cells comprised of CD 19 and CD 3 positive cells [Figure 2c and d]. Complete blood count, liver and renal function tests, and a chest radiograph were normal. The Mantoux test was negative. ELISA for Borrelia burgdorferi IgM antibodies was equivocal, while IgG was negative. A diagnosis of BLC with septo-lobular panniculitis was made and the patient was started on amoxicillin. The lesions showed improvement after 1 month and the antibiotics were continued for 2 months [Figure 3].Figure 2: (a) Skin biopsy is composed of epidermis, dermis, and subcutaneous tissue. The dermis shows mild to moderate inflammatory cell infiltrate and the subcutaneous tissue shows dense inflammatory cell infiltrate in nodules and sheets (H and E; 100×); (b) Dense panniculitis by lymphocytes, plasma cells, and histiocytes. Perineural inflammation is also seen (arrow) (H and E; 400×); (c) Immunohistochemistry shows scattered CD 19 positive cells (CD19; 100×); (d) Immunohistochemistry shows scattered CD3 positive cells (CD3; 400×)Figure 3: (a) Decrease in swelling, redness, and discoloration in the left ear lobe lesion after treatment; (b) Right ear lobe with decrease in redness and edemaLD is commonly seen in temperate countries. However, an increased incidence has been noted in other countries due to travel, deforestation, and alteration in the vector habitat. In India, cases of LD have been scantily reported, however, a study by Praharaj et al. reported seroprevalence of 13% in Northeastern states of India.[2] LD can show three main skin manifestations: Acute LD shows erythema migrans, subacute LD shows BLC, and late LD shows acrodermatitis chronica atrophicans.[1] BLC is a B cell pseudolymphoma and constitutes 5% of all skin manifestations of LD. The incubation period varies from weeks to 10 months. Patients present with a solitary soft, non-tender, bluish-red plaque involving the earlobe, breast, scrotum, or axillary fold.[3] On histopathology, a polyclonal lymphocytic infiltrate is seen in the dermis with predominant B lymphocytes and occasionally germinal center formation. In some cases, perineural inflammatory cell infiltrate may be noted, which is characteristic. Immunohistochemistry shows a predominance of CD 19 positive cells mixed with CD 3 positive lymphocytes. Dense septo-lobular panniculitis has been reported in a single case of disseminated Lyme borreliosis, however, it has not been reported earlier in BLC.[4] BLC should be kept as a differential diagnosis for skin lesions of the ear lobe, nipple, and scrotum in India. Other histologic differential diagnoses include peripheral cutaneous T-cell lymphoma, leprosy, and lupus panniculitis. Diagnosis relies on clinical findings, history of tick bite, travel to endemic areas, and serologic tests. IgM antibodies are detected 2–4 weeks after onset of rash, and IgG levels rise by 6 weeks and peak up to 6 months. In the early stages, antibodies to B. burgdorferi may not be developed, leading to false-negative or equivocal results. Specific histologic findings are seen in BLC as described above. Treatment by oral amoxicillin, cefuroxime, or doxycycline, leads to complete recovery in 90% of patients. Remission of signs and symptoms after treatment confirms the diagnosis.[5] The present case had the characteristic lesion on the ear lobes, right followed by left. The involvement of both ear lobes may represent the initial site of transmission or can be an early case of dissemination, as there is a preference for body parts with low body temperature like ear lobes and scrotum.[3] Histopathologically, the dermis showed moderately dense lymphocytic inflammation, and the subcutis showed polyclonal panniculitis. In addition, perineural invasion was seen that can be seen in cases of Borreliosis. IgM for Borrelia was equivocal and the patient responded to antibiotics, thus confirming the diagnosis. Lyme disease should be a clinical suspicion in non-endemic regions. It has non-specific systemic symptoms and serology may be false negative, hence, knowledge regarding its skin manifestations is important. Our case is unique because of the involvement of both ear lobes with septo-lobular panniculitis, which has not been reported in BLC. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Use of artificial intelligence (AI) The preparation of this manuscript was carried out entirely by the author without the use of artificial intelligence technologies.

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