Cases reported "Scleroderma, Localized"

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1/32. lichen sclerosus et atrophicus, bullous morphea, and systemic lupus erythematosus: a case report.

    lichen sclerosus et atrophicus (LSA) rarely coexists with morphea, especially when bullae occur in lesions of morphea. Here we report the case of a 15-year-old girl with this condition, who also fulfilled four out of 11 diagnostic criteria for systemic lupus erythematosus (SLE). Tissue biopsy of different skin lesions showed LSA in the regions of bullous morphea, that has rarely been reported in the literature.
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ranking = 1
keywords = sclerosus
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2/32. 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.
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ranking = 5.901091567919
keywords = lichen sclerosus, sclerosus, lichen
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3/32. Associated localization of morphea and lichen planus of the lip in a patient with vitiligo.

    Morphea, lichen planus and vitiligo are common diseases, but seldom associated. Morphea or lichen planus in the lips have not frequently been reported, and an associated localization of both lesions in the lip mucosa is indeed a rare event. A case of morphea and lichen planus localized in the upper lip mucosa in a patient with vitiligo is reported. immunohistochemistry revealed a clear prevalence of t-lymphocytes in the inflammatory infiltrates of morphea and in the band-like infiltrate of the lichen, a finding consistent with previous reports of morphea, lichen planus, and vitiligo. The inverse ratio found between CD8 and CD4 lymphocytes, compared to other reports, seems to confirm that the pathogenesis of morphea and lichen planus evolves through different stages. The present report, besides presenting a rare case, may prompt to consider a common pathogenesis among morphea, lichen planus and vitiligo.
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ranking = 0.10505790874278
keywords = lichen
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4/32. Chronic borreliosis presenting with morphea- and lichen sclerosus et atrophicus-like cutaneous lesions. a case report.

    We report on a case of chronic cutaneous borreliosis with manifestations clinically compatible with morphea and lichen sclerosus et atrophicus. The histopathologic features of these lesions were those of acrodermatitis chronica atrophicans. Our case illustrates the concept that clinical aspects of morphea and lichen sclerosus et atrophicus pertain to the spectrum of cutaneous borreliosis.
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ranking = 5.0498921562364
keywords = lichen sclerosus, sclerosus, lichen
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5/32. Effects of low dose ultraviolet A-1 phototherapy on morphea.

    AIM: The effects of low dose ultraviolet A-1 (UVA-1) phototherapy on different clinical stages of morphea (localized scleroderma) were analyzed in this case study. Based on these data, the different types of phototherapy described in the literature and currently used for treatment of morphea are compared. methods: Three patients with severe plaque type morphea in different stages were studied: one patient with late-stage lesions having stable sclerotic plaques; another patient with active inflammatory lesions; and a third patient with late-stage lesions associated with overlying lichen sclerosus et atrophicus (LSA). The treatment given was low dose UVA-1 phototherapy with single doses of 20 J/cm2 administered four times a week for 6 weeks, and once a week for another 6 weeks. RESULTS: Following UVA-1 phototherapy, the sclerotic plaques resolved, leaving smooth and soft tanned skin with normal structure, consistency and folding capability. In morphea with overlying LSA the elastic fibers did not completely return to the superficial papillary dermis despite the clinical clearance of both morphea and LSA. These data suggest that low dose UVA-1 phototherapy may improve, but not completely reverse, the histopathological changes of LSA. No side effects were observed during or after treatment. CONCLUSION: Our observations show in three patients that low-dose UVA-1 phototherapy is highly effective for treatment of all stages of morphea, including early inflammatory and late sclerotic lesions, and morphea with overlying lichen sclerosus et atrophicus. Because of its safety and efficacy, low dose UVA-1 phototherapy appears to be the treatment modality of choice.
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ranking = 1.6832973854121
keywords = lichen sclerosus, sclerosus, lichen
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6/32. Bullous lesions in scleroderma.

    BACKGROUND: The occurrence of bullous lesions in localized or systemic scleroderma is rare. Three histologic patterns have been reported: lichen sclerosus et atrophicus-like, lymphangiectatic blisters and autoimmune blistering diseases. OBJECTIVE: To investigate the frequency, clinical, and immunopathologic features of patients with scleroderma and bullous eruptions and to review the literature regarding this rare condition. methods: A retrospective study of 53 cases of scleroderma (localized, generalized, and systemic) in the dermatology and rheumatology clinics at one institution over an 8-year span. Clinical, serologic, and immunopathologic findings were analyzed in four cases. RESULTS: Four of 53 patients exhibited bullous lesions in association with scleroderma. The first case illustrates lymphangioma-like clinical and pathologic presentation. The second case demonstrates bullous lichen sclerosus et atrophicus-like pattern. The other two cases exemplify a superimposed autoimmune skin disease, epidermolysis bullosa acquisita and penicillamine induced pemphigus foliaceus after treatment for systemic scleroderma. CONCLUSIONS: Of the 53 original patients, we have described four cases of bullous scleroderma (7.5%) Illustrating several pathogenetic mechanisms of bulla formation. inflammatory (lichen sclerosus et atrophicus), fibrotic/obstructive (lymphangiomatous), autoimmune (epidermolysis bullosa acquisita), and pemphigus foliaceus. The final case illustrates bullae as a complication of therapy for the underlying scleroderma.
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ranking = 2.5249460781182
keywords = lichen sclerosus, sclerosus, lichen
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7/32. Morphea-like tattoo reaction.

    Tattoo reactions are histologically diverse. In general, dermal changes predominate, although epidermal changes such as acanthosis or spongiosis can also be seen. The chronic inflammatory cell infiltrate can be nodular, lichenoid, or granulomatous. Occasionally, the dermal infiltrate may be so dense as to suggest a diagnosis of cutaneous lymphoma. We report an unusual tattoo reaction that mimicked morphea histologically.
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ranking = 0.0095507189766164
keywords = lichen
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8/32. Bullous scleroderma-like changes in chronic graft-versus-host disease.

    Cutaneous graft-versus-host disease (GVHD) is the most common clinical setting for GVHD after bone marrow transplantation. Chronic cutaneous GVHD is categorized according to the type of skin lesions into lichenoid and sclerodermoid variants, but bullous scleroderma-like changes are exceptional. Recently, we studied a patient with these alterations. This is the second case described in the literature.
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ranking = 0.0095507189766164
keywords = lichen
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9/32. Localized morphoea preceded by a pigmented purpuric dermatosis.

    Localized morphoea uncommonly occurs in a linear distribution and may present following trauma, although most cases are idiopathic. Pigmented purpuric dermatoses such as lichen aureus may also rarely occur in a linear distribution and have been associated with trauma. A middle-aged man is described who initially presented with lesions typical of lichen aureus in a linear distribution at a site exposed to chronic low-grade trauma. This eruption was transient and clinically underwent spontaneous complete resolution. Several months later he developed localized morphoea in an identical distribution. To our knowledge this is the first reported case of a pigmented purpuric dermatosis, presumably precipitated by trauma, evolving into linear morphoea.
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ranking = 0.019101437953233
keywords = lichen
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10/32. Immunohistochemical investigations and introduction of new therapeutic strategies in scleromyxoedema: case report.

    BACKGROUND: Scleromyxoedema is a rare chronic skin disease of obscure origin, which may often be associated with severe internal co-morbidity. Even though different casuistic treatment modalities have been described, to date, curing still seems to be impossible. CASE PRESENTATION: We report a 44-year-old Caucasian female presenting with remarkable circumscribed, erythematous to skin-coloured, indurated skin eruptions at the forehead, arms, shoulders, legs and the gluteal region. Routine histology and alcian blue labelling confirmed a massive deposition of acid mucopolysaccharides. Immunohistochemical investigations revealed proliferating fibroblasts and a discrete lymphocytic infiltration as well as increased dermal expression of MIB-1 and anti-mastcell-tryptase cells. Bone marrow biopsies confirmed a monoclonal gammopathy of undetermined significance without morphological characteristics of plasmocytoma; immunofixation unveiled the presence of IgG-kappa paraproteins. CONCLUSIONS: Taking all data into account, our patient exhibited a complex form of lichen mxyoedematosus, which could most likely be linked a variant of scleromyxoedema. Experimental treatment with methotrexate resulted in a stabilisation of clinical symptoms but no improvement after five months of therapy. A subsequent therapeutic attempt by the use of medium-dose ultraviolet A1 cold-light photomonotherapy led to a further stabilisation of clinical symptoms, but could not induce a sustained amelioration of skin condition.
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ranking = 0.0095507189766164
keywords = lichen
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