1/7. A case of generalized morphea with a high titer of anti-borrelia burgdorferi antibodies.A 69-year-old male had noticed pruritus on the back for the previous 3-4 years and cutaneous sclerosis with swelling of the dorsum of the neck had developed in the last one and a half years. However, he had never complained of Raynaud's phenomenon of the fingers, dry mouth, or dry eyes. At this first visit to our hospital, he complained of erythematous cutaneous sclerosis with swelling of the dorsum of the neck. Histopathological findings biopsied from the neck showed epidermal hyperplasia with elongation of rete ridges and homogeneous and fibromatous changes of the dermis with dense perivascular cell infiltration consisting of mononuclear cells or lymphocytes with several nests of incontinentia pigmenti. However, there were no sclerotic changes in blood vessels in the upper dermis biopsied from the forearm skin, although slightly homogeneous and fibromatous changes of the dermis were seen. In the clinical course, the cutaneous sclerotic change enlarged to extend to the bottom of the cheek, forearm, and lower legs. These clinical features and histopathological findings led to the diagnosis of generalized morphea. Hematologic examination showed positive anti-borrelia burgdorferi IgM antibodies, although there were no positive anti-borrelia burgdorferi IgG antibodies. These results revealed that there can be a close association of localized scleroderma with borrelia burgdorferi and that generalized morphea may also represent a Borrelia infection.- - - - - - - - - - ranking = 1keywords = burgdorferi (Clic here for more details about this article) |
2/7. Genital lichen sclerosus associated with morphoea or systemic sclerosis: clinical and HLA characteristics.Although patients with both morphoea and lichen sclerosus have been reported previously, in the majority of these reports the lichen sclerosus has been extragenital. We report nine patients in whom genital lichen sclerosus coexisted with scleroderma spectrum disorders including seven with morphoea, one with morphoea and lichen planus, and one with systemic sclerosis. The clinical features, associated autoimmune disease, autoantibodies and HLA type are reported. Antibodies to borrelia burgdorferi were not detected in any of the patients. The coexistence of these diseases raises a number of intriguing questions about the relationship between them.- - - - - - - - - - ranking = 0.14285714285714keywords = burgdorferi (Clic here for more details about this article) |
3/7. Case study: periodic follow-up is necessary in morphea profunda to identify systemic evolution.The term "morphea" includes a wide spectrum of clinical entities, varying from localized plaques of only cosmetic importance to deep lesions resulting in considerable morbidity for the patient. In fact, although survival rates are no different from that of the general population, localized scleroderma may be associated with development of substantial disability, as occurs in deep morphea and in pediatric patients (disabling pansclerotic morphea of children). We report a case of morphea profunda affecting a young man with severe, rapidly progressive, widespread skin involvement and focus on the eventual systemic evolution of such cases. A 40-year-old man was admitted in 2002 for progressive subcutaneous indurations, preferentially involving the right side of the trunk. His health was altogether good, with the exception of a beginning chronic obstructive bronchopneumopathy. There was no family or personal history of dysmetabolic, cardiovascular, neoplastic, or cutaneous disease. Three years earlier, the patient had noted the appearance of two infiltrated, intensely red lesions on the right laterocervical and paraumbilical regions. These had been interpreted as subcutaneous lipomatosis on the basis of an ultrasound scan. The lesions had become progressively larger, while their surface had assumed a scleroatrophic appearance. Thereafter, other lesions had developed on his chest and lower limbs, mostly distributed on the right side of the body. Clinical examination revealed well demarcated, depressed sclerotic plaques with ivory-colored centers and erythematous borders ("lilac ring") localized on the neck, chest, and lower abdomen and limbs (Figure 1). They were bound to the deeper structures and arranged in a band-like linear distribution on the right side of the chest and abdomen where they extended horizontally for more than 10 cm in diameter. These lesions were totally asymptomatic. In addition, arborizing telangiectasias were evident on the neck and upper chest (Figure 2). Laboratory investigations provided normal range of erythrocyte sedimentation rat and C reactive protein levels and other inflammation markers. Antinuclear antibody, antidouble-strand dna, antimitochondrial, anti-extractable antigens (anti-centromere, anti-Scl-70, anti-U1RNP), and anti-borrelia burgdorferi antibodies were negative. Circulating immunocomplexes binding C1q were substantially increased. Oesophageal x-rays and lower limb electromyography were within normal limits; ventilatory function testing revealed a mild obstruction consistent with the beginning of chronic obstructive pulmonary disease. Although nailfold capillaroscopy documented nonspecific findings of connective tissue disease (mega-capillaries, segmentary dilatation and destruction), the laser-Doppler flussimetry revealed few signs of microcirculatory abnormalities, in absence of Raynaud's phenomenon. An abdominal wall ultrasonography, performed on a sclerotic plaque, documented thinning of the subcutaneous tissue, with increase of the fibrous component and lower fascia and muscle retraction. The biopsy specimen from the abdominal region included fascia and the subcutaneous tissue (previously obtained from the lower abdomen) with epidermal atrophy, a thickening and homogenization of collagen bundles in the deep dermis and hair reduction. A perivascular lympho-monocytic and plasmacellular infiltration with a dermo-epidermal distribution was present. Moreover, septal fibrosis with a perivascular lymphoplasmacellular inflammatory infiltrate was documented within the abdominal rectus muscle. The diagnosis of morphea profunda was made on the basis of clinical and histopathological findings. A therapeutic regimen based on amino benzoic potassium (Potaba; Glenwood, LLC, Glenwood, NJ), oral prednisone, and topical clobetasol was started. After several months of follow-up, the patient had obtained only moderate improvement of the clinical findings.- - - - - - - - - - ranking = 0.14285714285714keywords = burgdorferi (Clic here for more details about this article) |
4/7. Antibodies to the borrelia burgdorferi flagellum in patients with scleroderma, granuloma annulare and porphyria cutanea tarda.It is generally accepted that cutaneous Lyme borreliosis comprises erythema chronicum migrans, lymphadenosis benigna cutis, and acrodermatitis chronica atrophicans. In recent years the tick-borne spirochete borrelia burgdorferi has been associated with a number of other cutaneous disorders. We therefore investigated sera from 175 patients with localized scleroderma (morphea) (n = 64), systemic sclerosis (n = 74), granuloma annulare (n = 16) and porphyria cutanea tarda (n = 21) with the new, highly sensitive and specific borrelia burgdorferi flagellum ELISA assay. As controls (n = 297) served normal healthy volunteers and patients with other skin diseases. It was found that the distribution of individual antibody values and the median antibody levels were identical in controls and in patients with scleroderma, granuloma annulare and porphyria cutanea tarda. These data do not support the hypothesis of an etiological association between borrelia burgdorferi infection and scleroderma, granuloma annulare or porphyria cutanea tarda.- - - - - - - - - - ranking = 1keywords = burgdorferi (Clic here for more details about this article) |
5/7. The relevance of elevated borrelia burgdorferi titres in localized scleroderma.A 46-year-old man with a 2-year history of localized scleroderma of his right upper arm and elevated borrelia burgdorferi titres is described. The association of borrelia burgdorferi infection and localized scleroderma is discussed.- - - - - - - - - - ranking = 0.85714285714286keywords = burgdorferi (Clic here for more details about this article) |
6/7. Atrophoderma of Pasini and Pierini. Clinical and histopathologic findings and antibodies to borrelia burgdorferi in thirty-four patients.BACKGROUND: Idiopathic atrophoderma of Pasini and Pierini (IAPP) is a distinctive form of dermal atrophy, usually appearing as one or more sharply demarcated depressed areas. Little is known about the clinical variants of IAPP, and limited data are available on antibiotic therapy for this condition. OBJECTIVE: Our purpose was to define the various types of IAPP clinically and histologically and to investigate a possible association with borrelia burgdorferi infection. methods: The records of 34 patients with IAPP were reviewed. Skin biopsy specimens for routine histologic examination were obtained from 17 patients. serum from 26 patients was analyzed for antibodies against B. burgdorferi. RESULTS: Of the 34 patients (21 female, 13 male, 7 to 66 years of age), 23 had well-circumscribed brown, depressed plaques. The back was most frequently involved (82%). Eleven patients had a superficial variant of IAPP, characterized by slightly atrophic brown macules forming large hyperpigmented patches with an irregular border. Secondary areas of induration developed in 7 of 34 patients. Ten of 26 patients (38%) had elevated serum antibodies to B. burgdorferi. Twenty of the 25 patients treated with oral antibiotics had clinical improvement with no evidence of new active lesions. CONCLUSION: IAPP is an abortive, primarily atrophic variant of morphea. The clinical appearance of IAPP may be variable according to the stage of dermal atrophy and distribution of the lesions.- - - - - - - - - - ranking = 1keywords = burgdorferi (Clic here for more details about this article) |
7/7. Localized scleroderma associated with borrelia burgdorferi infection. Clinical, histologic, and immunohistochemical observations.BACKGROUND: Recent reports have implicated borrelia burgdorferi infection as a possible cause of localized scleroderma (LS). OBJECTIVE: Our purpose was to describe the clinical, histologic, and immunopathologic features of patients with LS who had serum antibodies to B. burgdorferi. methods: Ten patients were examined clinically and by routine microscopy. biopsy specimens from seven patients were studied immunohistochemically with monoclonal antibodies. The proliferative response of peripheral blood mononuclear cells to B. burgdorferi was investigated in seven patients by lymphocyte proliferation assay. RESULTS: Seven patients had plaque-type morphea, and three patients had linear scleroderma. Two patients had a history of previous erythema migrans. One patient had coexistent acrodermatitis chronica atrophicans, and in two patients lichen sclerosus et atrophicus was observed. Histologically, a prominent inflammatory phase with sclerosis of the connective tissue was shown in all patients. Immunohistochemical studies revealed that the inflammatory infiltrates consisted of both B and T lymphocytes, predominantly of the CD4 subset. All 10 patients had strongly elevated serum antibodies to B. burgdorferi. patients with LS showed significantly elevated lymphoproliferative responses to B. burgdorferi when compared with healthy control subjects. CONCLUSION: Our findings suggest that some cases of LS are linked to Borrelia infection.- - - - - - - - - - ranking = 1.2857142857143keywords = burgdorferi (Clic here for more details about this article) |