1/9. The hyperkeratotic variant of disseminated superficial actinic porokeratosis (DSAP).A 78-year-old South Korean man was referred to us from the Medical intensive care Unit (MICU) for an opinion. He was comatose and was on ventilatory care due to aspiration pneumonia. Multiple tiny papules had developed 10 years previously and since then the number and size had been increasing gradually. He had been diabetic for the past 4 years, and had Parkinson's disease diagnosed 1 year previously. Laboratory examinations revealed an elevated level of white blood cells (WBCs) (25,000/microL) and decreased hemoglobin (8.8 g/dL). Other laboratory results were negative or within normal limits. skin examination showed multiple, discrete, crust-like, brownish papules over the erythematous base on the face, upper extremities, and lower extremities. With the clinical impressions of irritated verruca vulgaris, seborrheic keratosis, or cutaneous fungal infection, a skin biopsy was taken from a papule on the left shin, and histopathologic examination revealed several pronounced hyperkeratotic and parakeratotic columns, and characteristic cornoid lamellae in the stratum corneum. Beneath the cornoid lamellae, the granular layer was decreased. A number of round or oval, dyskeratotic, homogenized eosinophilic cells with pyknotic nuclei were scattered in the prickle cell layer below the cornoid lamellae. A mild lymphohistiocytic infiltrate was observed in the papillary dermis and around the blood vessels in the upper dermis. Also, actinic degeneration was present in the upper dermis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/9. Uncommon vascular naevi associated with focal acantholytic dyskeratosis.Cutis marmorata telangiectatica congenita and vascular twin naevi are rare vascular anomalies in which focal acantholytic dyskeratosis is usually not observed. We describe a 44-year-old-man who presented for evaluation of skin lesions that had been present since birth. physical examination revealed anaemic macules adjacent to a naevus telangiectaticus on the chest. Naevus anaemicus was also seen on the shoulders, arms, and left leg. There was bluish-reddish reticulate marking of the skin and cutaneous atrophy. Shortening and hypoplasia of the left leg was observed. Histologic examination of two biopsy specimens revealed focal acantholytic dyskeratosis. In vivo confocal laser scanning microscopy showed dilated capillaries and vessels of the upper dermal plexus in the telangiectatic and decreased capillary blood flow in the anaemic skin sites. The findings were consistent with a diagnosis of cutis marmorata telangiectatica congenita, vascular twin naevi, and incidental focal acantholytic dyskeratosis. The particularities of the present case are the following: firstly, the association of two rare vascular anomalies to which the genetic concept of mosaicism can be applied; secondly, the occurrence of incidental focal acantholytic dyskeratosis in sites of vascular naevi.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/9. Morphological and biochemical studies of human beta-mannosidosis: identification of a novel beta-mannosidase gene mutation.BACKGROUND: There are seven well-known lysosomal storage diseases that produce angiokeratoma corporis diffusum clinically. beta-mannosidosis (MANB1; OMIM248510), first reported in humans in 1986, is a rare hereditary lysosomal storage disease caused by a deficiency of the enzyme beta-mannosidase. Since then, 13 cases of beta-mannosidase deficiency in ten families have been described. A human beta-mannosidase mutation has been reported only by Alkhayat et al. in 1998. OBJECTIVES: To clarify its pathogenesis we did electron microscopic, biochemical and molecular biological investigations of a Japanese patient with beta-mannosidosis. methods: Ultrastructural analyses, enzyme assays, cell culture and mRNA and genomic dna were sequenced to find mutations in the beta-mannosidase gene. RESULTS: Electron microscopy of skin biopsy specimens from the patient showed cytoplasmic vacuolation of lysosomes in blood and lymph vessels, endothelial cells, fibroblasts, secretory portions of eccrine sweat glands, neural cells and basal keratinocytes in the epidermis. This vacuolation was also observed in cultured keratinocytes and fibroblasts. Assays of seven enzyme activities in plasma and cultured skin fibroblasts showed a marked decrease of beta-mannosidase activity. Sequencing the beta-mannosidase cDNA revealed a four-base (ATAA) insertion between exons 7 and 8, resulting in a frameshift at codon 321 and termination at codon 325. Analysis of the patient's genomic dna revealed a novel homozygous A( 1)-->G splice site mutation in intron 7. CONCLUSIONS: To our knowledge, this is the first case of beta-mannosidosis reported in japan and the second report in which a gene mutation is identified. The biological importance of beta-mannose moieties in glycoproteins in basal keratinocytes is suggested.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/9. angiolymphoid hyperplasia with eosinophilia associated with pregnancy: a case report and review of the literature.A case of angiolymphoid hyperplasia with eosinophilia (ALH) is reported in a 33-year-old woman who developed an auricular nodule during the second trimester of her pregnancy. angiolymphoid hyperplasia with eosinophilia usually occurs on the head and neck of young adults and is more common in women than in men. Characteristic histologic features of ALH present in this case included proliferation of thick-walled blood vessels lined by prominent endothelial cells, infiltration of the interstitium by chronic inflammatory cells (mainly eosinophils), and presence of lymphoid follicles with germinal centers. The auricular tumor was completely excised. Thirteen months after excision, the patient remains tumor free. Although there are not many case reports on ALH during pregnancy or involving use of oral contraceptive pills, sex hormones may play a role in the pathogenesis of ALH. This hypothesis, in the context of cases previously described in the literature, and the differential diagnosis of ALH are discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/9. porokeratosis plantaris discreta. A case showing transepidermal elimination.A 16-year old girl developed multiple, well-demarcated, extremely painful, hyperkeratotic nodules on her left sole. Histologic examination revealed a cornoid lamella and transepidermal elimination of blood vessels and collagen fibers which may be caused by the acceleration of keratinization. The pain and tenderness may have been partially related to epidermal disruption.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/9. Erythromelanosis follicularis faciei et colli. case reports.Erythromelanosis follicularis of the face and neck has seldom been described in the literature. It is a condition of unknown aetiology. The clinical features are reddish-brown pigmentation, telangiectatic vessels, and pale follicular papules localized in the peri-auricular area. The disease is asymptomatic and has only been described in men. This report records two patients with erythromelanosis follicularis of the face and neck, a boy and a young adult female.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/9. vasculitis as the basis of cutaneous lesions in Reiter's disease.The cutaneous lesions of Reiter's disease (RD) and pustular psoriasis (PP) are said to be histologically similar and often indistinguishable. We encountered three cases of RD in which biopsy specimens of lesions clinically compatible with keratoderma blenorrhagicum showed a pustular psoriasiform tissue reaction in conjunction with a subjacent superficial leukocytoclastic vasculitis (LCV). In an attempt to ascertain if these changes were distinctive and unique to cutaneous RD, the incidence of such changes in lesions of PP was examined using light microscopy and immunohistochemistry. The role of chlamydial infection in the pathogenesis of the observed vascular changes also was explored by assessing for the presence or absence of vascular deposition of chlamydial antigen in cutaneous RD compared with that in a control group that included cases of LCV and PP. In addition to conventional light microscopic analysis, immunoperoxidase studies to identify immunoglobulin deposition were performed on formalin-fixed, paraffin-embedded tissue from two of three patients with RD and on skin biopsy specimens from 11 patients with PP. Direct immunofluorescence (DIF) studies with antibodies to immunoglobulin (Ig)G, IgM, IgA, C3, and chlamydial antigens were performed on frozen tissue from one patient with RD, two patients with PP, three patients with LCV, one patient with nonspecific dermatitis, and one patient with Behcet's disease, who had a high antichlamydia antibody titer. All three specimens of RD showed a pustular psoriasiform diathesis in conjunction with a subjacent superficial LCV that was of maximal intensity in the dermal papillae capillaries. Through an immunoperoxidase technique performed on formalin-fixed tissue, the RD cases for which tissue was available for study demonstrated Ig deposition in injured blood vessels; using the same technique one of 11 PP biopsy specimens showed vascular Ig deposition in concert with LCV. This patient's biopsy was from a lesion of drug-induced LCV. None of the other specimens showed either light microscopic or immunohistochemical evidence of vasculitis. In the one specimen of RD studied by DIF, vascular deposition of IgG, IgM, C3, chlamydia heat shock protein 60 (CHSP60), and chlamydia-specific lipopolysaccharide (LPS) was observed. In the two specimens of PP studied, vascular deposition of C3, fibrin, CHSP 60, and chlamydia-specific LPS was not observed. Two specimens of LCV and the one specimen of dermatitis with concomitant nonspecific vascular injury showed vascular Ig and C3 deposition; in contrast, no vascular deposition of CHSP 60 or chlamydia-specific LPS was observed.(ABSTRACT TRUNCATED AT 400 WORDS)- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/9. Primary cutaneous angioplasmocellular hyperplasia.Two cases of solitary nodules on the skin of the trunk of young adults showing a peculiar pattern of proliferation of blood vessels and a mixed inflammatory infiltrate with predominance of polyclonal plasma cell infiltration are presented. Follow-up of these patients after complete surgical excision revealed no recurrence. We interpreted the lesions within the morphologic spectrum of plasma cell infiltrates of the skin to be reactive and/or hyperplastic, which we term primary cutaneous angioplasmocellular hyperplasia.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/9. Invasive atypical fibroxanthoma and eruptive actinic keratoses in a heart transplant patient.We report a case of atypical fibroxanthoma (AFX) and eruptive actinic keratoses following heart transplantation in a 65-year-old patient. The AFX was diploid with moderate proliferative activity. It recurred and proved to invade the large vessels of these tumors during immunosuppressive therapy highlights the cumulative intervention of ultraviolet light and immune dysregulation in the development of some skin cancers in transplant patients.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |