1/9. Localized scleroderma associated with progressing ischemic stroke.We present a 73 year-old Japanese woman with localized scleroderma involving the right side of the scalp accompanied by continuous tingling pain, who developed insidiously progressive left hemiparesis. In magnetic resonance imaging of the brain, an infarct first appeared in the watershed region of the right middle cerebral artery territory and subsequently extended to deep white matter accompanied by scattered hemorrhages. Focal stenosis in the M2 portion of the right middle cerebral artery was revealed on magnetic resonance angiography, and the distal vessels were only shown faintly. A biopsy specimen from the sclerotic scalp lesion showed obvious thickening of vessel walls and mild mononuclear cell infiltration. We believe that the progressing ischemic stroke was caused by hemodynamic disturbances from localized sclerotic obstruction of the middle cerebral artery, with an autoimmune pathogenesis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/9. 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 = 0.5keywords = vessel (Clic here for more details about this article) |
3/9. Necrotizing vasculitis in a patient with generalized morphea.Generalized morphea is rarely associated with systemic overlap. We report an unusual case with generalized morphea involving cutaneous large vessel vasculitis, mononeuritis multiplex, and lupus anticoagulant without any evidence of the coexistent systemic lupus erythematosus.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
4/9. Development of histologic features of scleroderma in congenital lesions.BACKGROUND: Localized scleroderma has been reported after radiation therapy, but has never been reported to occur at the site of a congenital lesion. CASE REPORT: We present two patients, both with family histories of autoimmune disease, who reported unilateral hypopigmented areas on the trunk since birth. The areas remained asymptomatic and grew with the patients until adulthood when the areas became indurated then firm and showed hyperpigmentation. histology: Histologically, both lesions showed features of localized scleroderma with diffuse sclerosis of collagen and loss of periadnexal fat. There was a perivascular lymphoplasmocytic infiltrate with occasional eosinophils extending into the subcutaneous fat predominantly along fat septae, and diffuse loss of CD34 stromal cell populations within the lesions. CONCLUSION: We propose that somatic mutations affecting vessels may predispose to increased endothelial cell apoptosis. This could lead to the development of an autoimmune response in some individuals, and the areas of localized scleroderma may be markers of susceptibility to autoimmune disease.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
5/9. En coup de sabre.A man born in 1961 was apparently well until his marriage in May 1986. In August of that year, his attention was drawn to the presence of a reddish patch over the right forehead. It was initially asymptomatic. It continued to progress to form an apparent linear furrow extending from the receding hairline to the vertex and temporal part of the scalp. At this juncture, the patient experienced moderate pain when laughing. Five years later the patient experienced a constant boring pain affecting the right eyeball. Consultation with an ophthalmologist led to sacrificial enucleation of the right eye despite the lack of a precise diagnosis. Nevertheless, the initial condition continued to progress causing disfigurement of the right side of the face. Examination of the face was marked by perceptible asymmetry. A linear atrophic plaque in the form of a furrow was identified on the right side of the forehead extending from the eyebrow to the vertex and temporal part of the scalp. The skin over the furrow was taut and bound down (Fig. 1). A hematoxylin and eosin-stained section prepared from the lesion revealed marked thickening of the dermis. The collagen bundles were hypertrophied and closely packed together. The staining was homogeneously eosinophilic. It was largely devoid of inflammatory infiltrate. The changes in the blood vessels were conspicuous by narrowing of the lumina, thickening of the walls, and a sparse perivascular lymphocytic infiltrate. Pilosebaceous units were completely absent, whereas a few atrophic pulled-up sweat glands were located in the mid-dermis. Similar changes were observed in the subcutaneous tissue. The epidermis was largely atrophied, with flattening of the rete ridges.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
6/9. Atrophoderma (Pasini-Pierini). Findings on direct immunofluorescent, monoclonal antibody, and ultrastructural studies.A patient with atrophoderma (Pasini-Pierini) was studied. Microscopic examination showed small collections of mononuclear cells around dermal blood vessels. Electron microscopic study demonstrated macrophages and lymphocytes around vessels and between fibers in the dermis; the epidermis, dermis, collagen, and elastic fibers appeared normal. Monoclonal antibody studies of the cells in the perivascular infiltrate demonstrated cells reacting with anti-Leu-1 (pan-T-cell antibody), anti-Leu-3a (the helper/inducer T-cell antibody), and OKM 1 antibody-reacting cells (macrophages). Direct immunofluorescent studies showed IgM and C3 staining in the small blood vessels of the papillary dermis, scattered IgM cytoids at the basement membrane, and focal fibrinogen in the mid-dermis. Mononuclear cells in the perivascular infiltrate, similar in type and percentage concentration, have been demonstrated also in patients with anetoderma, another rare atrophic cutaneous disorder. macrophages and T lymphocytes around papillary dermal blood vessels may play a role in the pathogenesis of atrophoderma and anetoderma.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
7/9. Juvenile dermatomyositis: histological findings and pathogenetic hypothesis for the associated skin changes.skin biopsies from patients with scleroderma and juvenile dermatomyositis (DM) share many histologic features. Characteristics common to both diseases are particularly evident in the dermal microvasculature and include endothelial swelling and concentric thickening of the vascular basement membrane. Biopsies performed on 3 patients with the severe vasculitic form of juvenile DM showed these changes as well as dropout of vessels and linear deposition of collagen. The latter findings, seen late in the course of the disease, are indistinguishable from those of advanced scleroderma. A hypothesis is presented which attempts to relate these histological findings to a common underlying pathophysiologic mechanism.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
8/9. Intracerebral involvement in scleroderma en coup de sabre: report of a case with neuropathologic findings.Linear scleroderma en coup de sabre (LScs) is a rare disorder not infrequently associated with neurologic symptoms, notably epilepsy. However, histopathologic documentation of intracerebral lesions in LScs is very limited and the etiology of the central nervous system symptoms has therefore never been convincingly established. We describe a 27-year-old woman with LScs and a longstanding history of epilepsy. Radiographic studies demonstrated a focal, intraparenchymal lesion in the left frontal lobe directly subjacent to the area of scleroderma on the forehead and scalp. The resected cerebral lesion revealed localized band-like sclerosis of the leptomeninges and associated vessels, as well as intraparenchymal calcifications and anomalous, ectatic vessels. These findings suggest that LScs may represent a neurocutaneous syndrome of vascular dysplasia similar to the sturge-weber syndrome, rather than a localized form of collagen vascular disease, as suggested by some.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/9. Scarring alopecia and sclerodermatous changes of the scalp in a patient with hepatitis c infection.hepatitis c virus (HCV) is a common cause of chronic hepatitis and is frequently associated with extrahepatic disease. Recently, cutaneous disorders have been a presenting manifestation of HCV infection. porphyria cutanea tarda (PCT) is one of the cutaneous diseases associated with hepatitis c. PCT manifests in an acute form with tense bullae and erosions and in a chronic form with milia, scarring, and sclerodermatous changes. HCV has also been implicated as a cause of vasculitis through immune complex deposition. We report a patient in whom HCV was associated with sclerodermoid PCT and a medium vessel vasculitis. This case underscores the importance of HCV and its potential cutaneous manifestations, as well as the importance of recognizing cutaneous manifestations of internal disease that may be the first clue to diagnosis of HCV.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |