Clinical and Experimental Rheumatology 2014; 32: 121-122. CASE REPORT A boy with tight skin: ABSTRACT Physical examination was normal. borrelia-associated We present a case of a 16-year-old A severe limitation of movement in boy with caused by Borre- the shoulders, knees, ankles, elbows, early-onset morphea lia burgdorferi. We re-emphasise an wrists and finger joints was seen with immunohistochemical method, focus circumscribed ivory sclerotic plaques R.M. Verberkt1, M. Janssen2, floating microscopy (FFM), to detect around these joints, surrounded by J. Wesseling1 Borrelia burgdorferi spirochetes in tis- areas of hyperpigmentation. We sus- 1Department of Paediatrics; sue sections. Focus floating microscopy pected morphea. The skin around his 2Department of Rheumatology, (FFM) proved to be more sensitive than feet, knees and elbows was tight (Fig. Rijnstate, Arnhem, the Netherlands. polymerase chain reaction (PCR) and 1), but the facial skin was normal. The Roos M. Verberkt, MD nearly equally specific. capillaries in the cuticles were normal Matthijs Janssen, MD and there was no sclerodactyly. There Judit Wesseling, MD, PhD Introduction were no digital pitting or ulcera. Please address correspondence to: Morphea, or localised , is His blood pressure was normal. Judit Wesseling, MD, PhD, an inflammatory connective tissue dis- Laboratory studies showed no infec- Department of Paediatrics, ease that affects the skin and leads to tious parameters besides a slightly Rijnstate, a pronounced sclerosis with increased elevated erythrocyte sedimentation Wagnerlaan 55, 6815 AD Arnhem, The Netherlands. collagen deposition and of the rate (16 mm/hour). There was a mild E-mail: [email protected] skin. It displays a wide clinical spec- elevation of alkalic phosphatase (157 Received on January 26, 2013; accepted in trum ranging from single localised U/L). X-rays of the joints were all nor- revised form on April 30, 2013. patches to disseminated devastating mal. However, testing for anti-Borrelia © Copyright Clinical and forms. Lesions usually present as ivory burgdorferi IgG was extremely positive Experimental Rheumatology 2014. sclerotic patches surrounded by an in- (>400 U/ml). Immunoblot tested posi- flamed lilac ring. tive for p39, p41, p58, p100, OspC and Key words: morphea, localised Involvement of subcutaneous fat and VlsE. IgM was negative. A treponemal scleroderma, Borrelia burgdorferi, underlying muscles may result in pro- screening test was negative. Antinucle- rheumatology found atrophy. In children this may lead ar antibodies (ANA) tested negative. A to growth retardation of the affected skin biopsy showed extensive lympho- limb or anatomic region. The possible cytoplasmic infiltration in the (epi)der- involvement of Borrelia burgdorferi mis and subcutis, no spirochetes were as a causative agent in morphea shows found. A polymerase chain reaction conflicting results, obtained by differ- (PCR) for Borrelia burgdorferi was ent studies using serological, immuno- negative in the skin biopsy material. histochemical, culture and polymerase The positive Borrelia serology suggest- chain reaction (PCR) approaches (1). ed a causative role in the presence of We present a patient with morphea morphea and oral Doxycyclin (200 mg caused by Borrelia burgdorferi. for 30 days) was started. Three months later, a deep skin biopsy (i.e. skin, Case report muscle and fascia) showed a lymphop- Herein, we report the case of a 16-year- lasmocytic infiltration with increased old, previously healthy, boy presented fibrosis in the dermis, subcutis, fascia to the department of paediatric rheuma- as well as in striated muscle tissue. He tology in November 2009, with severe was started on treatment with metho- progressive limitation of flexion and trexate and steroids. extension in shoulders, knees, ankles, Eisendle et al. used focus-floating mi- elbows, wrists and fingers over a period croscopy (FFM) for detection of Bor- of six months. He also noticed a brown- relia. This is a modified immunohis- ish discoloration of the skin around tochemical technique that combines the large joints. There were no signs several strategies to detect minuscule of arthritis and the movements were organisms in tissue sections. Spiro- not painful. The patient was extremely chetes can be shown by staining with tired. Our patient did not recall a tick polyclonal rabbit antibodies to Borre- bite or erythema migrans over the pre- lia burgdorferi (2, 3). FFM proved to vious months. There was no Raynaud’s be more sensitive than PCR (96.0% phenomenon. Inflammatory autoim- vs. 45.2%) and nearly equally specific mune diseases were unknown to his (99.4% vs. 100%). Furthermore, FFM Competing interests: none declared. family. rejected the diagnosis Borrelia infec-

121 CASE REPORT Borrelia-associated early-onset morphea / R.M. Verberkt et al.

Fig. 2. Focus floating microscopy detected spirochetes in the skin biopsy material.

Fig. 1. Morphea on the patient’s right elbow. tion with more consistency: negative are well known skin manifestations of 2. EISENDLE K, GRABNER T, ZELGER B: Mor- predictive value is 97.1% vs. 66% (3). Lyme disease. This case history shows phoea: a manifestation of infection with Borrelia species? Br J Dermatol 2007; 157: Focus floating microscopy in our pa- that morphea should also be considered 1189-98. tient detected spirochetes in the skin bi- as a skin manifestation of Lyme disease 3. EISENDLE K, GRABNER T, ZELGER B: Focus opsy material (Fig. 2) ultimately prov- in young patients. It had to be added to floating microscopy: “gold standard” for cu- ing Lyme disease. The second biopsy the differential diagnosis of scleroder- taneous borreliosis? Am J Clin Pathol 2007; 127: 213-22. material, after treatment for Borrelia ma-like diseases presenting to paediat- 4. O’CONNELL S: Lyme borreliosis: current infection tested negative for spirochetes ric rheumatology practices. issues in diagnosis and management. Curr by FFM indicative for an effective anti- Opin Infect Dis 2010; 23: 231-5. biotic treatment. References Lyme borreliosis is the most com- 1. PRINZ J, KUTASI Z, WEISENSEEL P et al.: mon vector-borne bacterial infection “Borrelia-associated early-onset morphea”: A particular type of scleroderma in child- in temperate areas of the Northern hood and adolescence with high titer antinu- hemisphere (4). Erythema migrans and clear antibodies? J Am Acad Dermatol 2009; acrodermatitis chronica atrophicans 60: 248-55.

122