Erythema multiforme and persistent erythema as early cutaneous manifestations of Lyme disease

Erythema multiforme and persistent erythema as early cutaneous manifestations of Lyme disease

Erythema multiforme and persistent erythema as early cutaneous manifestations of Lyme disease M-L.A. Schuttelaar,^ R. Laeijendecker,^ R. J. Heinhuis,'...

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Erythema multiforme and persistent erythema as early cutaneous manifestations of Lyme disease M-L.A. Schuttelaar,^ R. Laeijendecker,^ R. J. Heinhuis,'^ and Th. Van Joosf^ Dordrecht and Rotterdam, The Netherlands

We report two cases of borreliosis (Lyme disease) with unusual cutaneous manifestations, erythema multiforme, and persistent erythema. The lesions in both of our patients had distinctive histopathologic features. To our knowledge, this is the first report of erythema multiforme and persistent erythema as early cutaneous manifestations of Lyme disease. (J Am Acad Dermatol 1997;37:873-5.)

Lyme borreliosis can be divided into early (localized or disseminated) and late (chronic, persistent) disease.' We describe two patients with Lyme disease in whom erythema multiforme and persistent erythema were observed as early cutaneous manifestations. CASE REPORTS Case 1 A 50-year-old man had erythematous lesions on his trunk, legs, and arms for 3 weeks. Five days before the skin lesions appeared he had slight fever. The patient mentioned that he had been bitten by a tick 4 months earlier. Examination revealed multiple 3 to 6 cm targetoid lesions on his trunk, legs, and arms (Fig. 1). The results of laboratory tests showed an erythrocyte sedimentation rate of 38 mm/hr, and gamma glutamyl transferase was elevated to 79 U/L (normal, < 40 U/L). Antibodies to herpes simplex virus revealed no evidence of a recent infection. Serologic tests for hepatitis B, mononucleosis, syphilis, and cytomegalovirus were negative. The Borrelia burgdorferi titer was elevated to 1:8192 (immunofluorescent antibody test, IFA). A biopsy specimen from a lesion on the trunk showed mild hyperkeratosis and a perivascular and ORTHO This article is made possible through an educational grant from the Dermatological Division, Ortho Pharmaceutical Corporation. From the Departments of Dermatology" and Pathology,'' Merwede Hospital Dordrecht, and the Department of Dermatology, University Hospital Rotterdam/Dijkzigt.^ Reprint requests: M~L.A. Schuttelaar, Department of Dermatology, Merwede Hospital, Alb. Schweitzerplaats 25, 3318 AT Dordrecht, The Netherlands. Copyright © 1997 by the American Academy of Dermatology, Inc. 0190-9622/97/$5.00 + 0 16/4/81657

Fig. 1. Case 1. Multiple annular erythematous maculopapular lesions with livid center on trunk, legs, arms.

periadnexal lymphocytic dermal infiltrate (Fig. 2). No spirochetes could be demonstrated with Steiner stain. The histopathologic findings were compatible with erythema multiforme. The patient was treated with oral doxycycline 200 mg/day for 4 weeks. During the first week of treatment, the erythema multiforme cleared. After treatment, the anti-Borrelia antibody titer decreased to 1:4096 in 6 weeks. Six weeks later the titer was 1:512. Eight months after starting therapy, the titer was 1:64.

Case 2 A 12-year-old girl had a 3-month history of a red lesion on her neck. Her history revealed atopy. Initially, the lesion was treated with topical corticosteroids. The patient mentioned that she may have been bitten by a tick 3 weeks before the neck lesion appeared. Examination revealed an oval erythematous plaque 3 to 8 cm in diam-

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Fig. 2. Case 1. Trank biopsy specimen reveals perivascular, periadnexal lymphocytic infiltrate in dermis.

Fig. 3. Case 2. Oval erythematous plaque on neck.

eter on the neck (Fig. 3). Other cutaneous or mucosal lesions were not observed. The results of laboratory investigations were normal. Two months later tests revealed positive serology for B. burgdorferi IgG and IgM (enzyme-linked immunosorbent assay). The B. burgdorferi titer was 1:512 (IFA). Histopathologic examination of a biopsy specimen from the lesion showed basketweave keratosis in the epidermis and superficial and deep perivascular and interstitial infiltrates of lymphocytes and plasma cells in the dermis (Fig. 4). These findings were compatible with persistent erythema. She was treated with oral doxycycline 200rag/dayfor 1 week. After 1 week, the dose was reduced to 100 mg/day for 3 weeks. The skin lesion disappeared after treatment. The Borrelia antibody titer was negative after 3 months (IFA); B. burgdorferi IgG was positive and IgM was negative (enzyme-linked immunosorbent assay).

Fig. 4. Case 2. Neck biopsy specimen. Superficial and deep perivascular and interstitial infiltrates of lymphocytes and plasma cells in dermis.

DISCUSSION Erythema chronicum migrans (ECM) may be an early localized or early disseminated cutaneous symptom of Lyme borreliosis. Approximately 75% of patients with Lyme disease present with typical lesions of ECM.^ Demonstration of the spirochete in tissue, or culture of the organism from tissue or blood, is the most specific way to diagnose B. burgdorferi infection. Specific humoral and cell-mediated immune responses are important in the pathogenesis of Lyme borreliosis. In patients with ECM, the specific T cell response to B. burgdorferi can develop before a measurable humoral response.^ The inflammatory infiltrate in ECM lesions consists of abundant Langerhans cells and T cells in the dermis and epidermis with a slight preponderance of the helper/inducer subset.-^ In later Lyme disease other mediators may be involved including interleukin-1 (IL-l),"'^ immune complexes,^ and activation of the complement pathway.^ An association of erythema multiforme (EM) with borreliosis has not been reported. In case 1, both clinical and histopathologic features suggested EM. Other causes which could have precipitated the EM lesions were not found. The observed high titer of B. burgdorferi antibodies, the decrease in the Borrelia antibody titer, and the remission of the clinical signs of EM during and shortly after treatment are all highly suggestive that EM in case 1 was triggered by the Borrelia infection. EM is a reaction pattern to many different stimuli. It involves immunologic mechanisms and

Journal of the American Academy of Dermatology Volume 37, Number 5, Part 2

perhaps immune complexes, such as those composed of B. burgdorferi antigens (a type III reaction). It may also be an allergic reaction to B. burgdorferi. In case 2 other possible causes for persistent erythema such as fixed drug eruption, dermatomyositis, sarcoidosis and systemic lupus erythematosus were excluded. The B. burgdorferi serology was positive. Treatment cleared the persistent erythema, and after treatment the B. burgdorferi titer declined significantly. Topical steroids had no significant effect. In this case persistent erythema was an early cutaneous manifestation of Borrelia infection. In conclusion both EM and persistent erythema may be early cutaneous manifestations of Lyme disease.

Schuttelaar et al. 875 REFERENCES 1. Asbrink E, Hovmark A. Early and late cutaneous manifestations in Ixodes-borne borreliosis. (Erythema migrans borreliosis, Lyme borreliosis.) Ann N Y Acad Sci 1988;539:4-15. 2. Abele DC, Anders KH. The many faces and phases of borreliosis, I: Lyme disease. J Am Acad Dermatol 1990;23:16786. 3. Buchner SA, Rufli T. Erythema chronicum migrans: evidence for cellular immune reactions in the skin lesion. Dermatologica 1987;174:144-9. 4. Beck G, Habicht GS, Benach JL, et al. A role for interleukin-I in the pathogenesis of Lyme disease. Zentralbl Bakteriol Mikrobiol Hyg A 1986;263:133-6. 5. Hardin JA, Steere AC, Malawista SE. The pathogenesis of arthritis in Lyme disease: humoral immune responses and the role of intra-articular immune complexes. Yale J Biol Med 1984;57:589-93. 6. Kochi SK, Johnson RC. The role of specific antibody and complement in killing of Borrelia burgdorferi by human serum (abstract). Ann N Y Acad Sci 1988;539:388.