1/20. Hyperreactivity of pseudoxanthoma elasticum-affected dermis to vitamin D3.A 68-year-old woman with pseudoxanthoma elasticum (PXE) was treated with oral vitamin d(3). After 2 weeks, new papules developed on the pre-existing plaques. biopsy specimens of a fresh papule showed more abundant calcium deposition than before therapy. Electron microscopy revealed electron-lucent areas, which suggested unusual mineralization. From these findings, we propose that in patients with PXE, certain cutaneous tissues are highly sensitive to vitamin d(3), resulting in microdeposition of calcium salts.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
2/20. osteoma cutis coexisting with cutis laxa-like pseudoxanthoma elasticum.pseudoxanthoma elasticum (PXE) is a heritable disorder of elastic fibers characterized by yellowish, coalescing papules on the loose and wrinkled flexural area. There have been no reports of osteoma cutis associated with PXE. A 17-year-old Korean girl presented cutis laxa-like marked wrinkling on the flexural area, and a skin biopsy specimen revealed multiple foci of ossification with irregularly clumped, basophilic-stained elastic fibers in the reticular dermis and calcium deposits along the elastic fibers. Ultrasonographic evaluation showed multiple tiny osteomas diffusely scattered along the entire abdominal wall, axillae, and medial aspect of the upper arms. We report the first case of osteoma cutis coexisting with cutis laxa-like PXE.- - - - - - - - - - ranking = 0.25keywords = dermis (Clic here for more details about this article) |
3/20. Coexistence of pseudoxanthoma elasticum-like papillary dermal elastolysis and linear focal dermal elastosis.An 89-year-old Japanese woman had pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck and linear focal elastosis on the flexor aspects of the legs and thighs. The lesions of both diseases had similar histologic patterns-loss of elastic fibers in the papillary dermis and accumulation of normal-appearing elastic fibers in the subpapillary or mid dermis. Immunohistochemical analysis showed elastin, and microfibril-associated proteins (fibrillin 1 and 2 and microfibril associated glycoprotein 1 and 4) were absent or decreased in the papillary dermis and present in the accumulated elastic fibers in both lesions. The association of 2 diseases on different areas of the same patient and histologic and immunohistologic similarities between the diseases suggest that, despite the clinical differences, both diseases are closely related.- - - - - - - - - - ranking = 0.75keywords = dermis (Clic here for more details about this article) |
4/20. pseudoxanthoma elasticum (PXE)-like calcification in adult dermatomyositis.A case of pseudoxanthoma elasticum (PXE)-like calcification in adult dermatomyositis (DM) is described. The patient was a 38-year-old woman with a history of dermatomyositis for 3 months. Yellowish, hard, papulo-plaque lesions, which looked like those of pseudoxanthoma elasticum, were noted on her left axilla. calcium deposition was confirmed by X-ray, histopathological, and electron microscopic examinations. Histopathological and histochemical examinations showed acicular calcium deposition in the middle and deep dermis surrounded by mucin. Electron microscopic examination revealed that the calcium deposition was not on collagen fibers. These morphological features were distinct from those of PXE. We proposed the possibility that degenerated mucin or degenerated elastic fiber might result in subsequent calcium deposition in reticular calcinosis in adult DM. The calcification clinically disappeared without any specific treatment except for prednisolone and cyclophosphamide.- - - - - - - - - - ranking = 0.25keywords = dermis (Clic here for more details about this article) |
5/20. pseudoxanthoma elasticum with abnormal nailfold microcirculatory findings.pseudoxanthoma elasticum (PXE) is an inherited disorder of elastic tissue. Here we report a 34-year-old male patient who developed multiple symptomless yellowish papules over his neck for several months. He visited our dermatologic out-patient-clinic because his sister had similar skin lesions and mild visual impairment. The pathologic features of the skin biopsy showed fragmented calcified elastic fibers in the mid-to-lower dermis under the H&E, Verhoeff-van Gieson and Von Kossa stains. Under electron microscopy, calcified degenerated elastic fibers were noted. No other internal organ involvement was found except angioid streaks on the fundus. In addition, morphological changes of the nailfold capillaries, including increased tortuosity, dilated venous limbs of capillary loops, and decreased red-blood-cell velocity, were observed under the capillaroscopy. Though former reports have indicated that cardiovascular manifestations are caused by degeneration of elastic fibers of blood vessels, this study is the first to emphasize the microcirculatory disturbance of nailfold capillary, including morphology and blood-cell velocity, in PXE.- - - - - - - - - - ranking = 0.25keywords = dermis (Clic here for more details about this article) |
6/20. Linear focal elastosis: histopathologic diagnosis of an uncommon dermal elastosis.BACKGROUND: Linear focal elastosis is an uncommon dermal elastosis that occurs predominantly on the back. Although first described in the lumbar region of elderly white men, more recent reports note similar findings on the trunk and limbs of adolescent Asian men. methods: We present a typical case of an eighty-three year old white man with a one-year history of asymptomatic linear yellow and erythematous plaques on his lumbar region. RESULTS: light microscopic examination revealed skin with an unremarkable epidermis with coarsely clumped elastic fibers in the reticular dermis, which were highlighted by an elastic tissue stain. CONCLUSION: The histopathologic differential diagnosis includes pseudoxanthoma elasticum, connective tissue nevus, elastofibroma, and solar elastosis. Although some of these diagnoses can be excluded by histologic examination, correlation with the clinical findings is necessary to arrive at the correct diagnosis of linear focal elastosis. Linear focal elastosis is an uncommon dermal elastosis that was originally described as asymptomatic linear yellow plaques in the lumbar region in elderly white males 1. Recent reports have extended the spectrum of this entity to include red-yellow linear atrophic to raised plaques on the trunks and limbs of adolescent males, predominantly of Asian descent. Twenty-one cases of linear focal elastosis have been reported in the literature. We report an additional case of linear focal elastosis in an elderly male and review the literature.- - - - - - - - - - ranking = 0.5keywords = dermis (Clic here for more details about this article) |
7/20. Elastosis perforans serpiginosa associated with pseudo-pseudoxanthoma elasticum during treatment of Wilson's disease with penicillamine.BACKGROUND: Elastosis perforans serpiginosa (EPS) is a reactive perforating dermatosis characterized by the elimination of abnormal elastic fibers from the upper dermis through the epidermis. In a few cases, it occurs as a side effect of treatment by D-penicillamine (DPA). The first case of EPS induced by DPA was described in 1972 in a patient treated for Wilson's disease. Subsequently, cutaneous changes resembling pseudoxanthoma elasticum (PXE) were observed in patients treated with DPA and were reported as pseudo-PXE. CASE REPORT: We report herein the clinical, pathological and ultrastructural study of 2 new cases of DPA-induced EPS and pseudo-PXE. These patients had been treated for Wilson's disease since 14 and 16 years, respectively. Characteristic abnormal elastic fibers were found on histopathological examination of both EPS and pseudo-PXE skin and confirmed by an ultrastructural study. There was no ABCC6 mutation. Discussion: penicillamine is able to induce widespread, cutaneous and systemic, elastic fiber damage. Our patients present typical features of DPA-induced elastosis, presenting as EPS and pseudo-PXE. ABCC6 mutation is associated with PXE and, as expected, it was absent in our cases of pseudo-PXE. This elastopathy has been related to morphologic changes in elastic fibers secondary to prolonged therapy in most cases. DPA may interfere with elastin cross-linking through inhibition of the enzyme lysyl oxidase, or by formation of complexes with the cross-linked precursors, impairing a normal maturation of elastic fibers. However, no fatal complication of DPA-induced elastopathy has been reported so far. An improvement of the cutaneous lesions is expected after the drug discontinuation.- - - - - - - - - - ranking = 0.5keywords = dermis (Clic here for more details about this article) |
8/20. White fibrous papulosis of the neck: a chinese case.A 48-year-old man who presented with papules on his neck was reported. The patient had multiple, discrete, non-follicular papules on the neck that were round-to-oval, 2--3 mm in diameter and pale. They had remained entirely asymptomatic. On histologic examination, there were circumscribed areas of thickened collagen bundles in the papillary to mid-dermis. This clinical and histologic picture suggested the diagnosis of "white fibrous papulosis of the neck". As far as we are aware, this is the first report of the condition from china.- - - - - - - - - - ranking = 0.25keywords = dermis (Clic here for more details about this article) |
9/20. Elastosis perforans serpiginosa-like pseudoxanthoma elasticum in a child with severe Moya Moya disease.A 2-year-old girl with Moya Moya disease who had relapsing cerebrovascular strokes presented with loose skin folds, 'chicken' skin appearance and perforating elastosis serpiginosa-like lesions in the genitocrural region. Histologically, calcified material perforating the epidermis and adjacent short curled and mineralized elastic fibres suggested a variant of pseudoxanthoma elasticum (PXE). As PXE is known to be caused by various mutations in the transmembrane transporter ABCC6 gene, we hypothesized that a novel ABCC6 mutation may underlie this unique combination of PXE and elastopathic vascular damage. Therefore, the complete ABCC6 coding region of the patient and her parents was screened for genetic alterations. No bona fide disease-causing mutation of ABCC6 could be found in the child and in her parents. However, two novel allelic amino acid substitutions (Arg1273Lys and Glu1293Lys; exon 27) were found in the girl and her father, localized in close proximity to the region that codes for the functionally critical second nucleotide-binding fold of ABCC6. Although a causal involvement of these amino acid substitutions could not be proven based on this study, both heterozygote substitutions may possibly have interacted with other undetected recessive maternal ABCC6 changes in the child. To the best of our knowledge, this is the first report of an association between early-onset PXE and severe Moya Moya syndrome possibly related to ABCC6 changes.- - - - - - - - - - ranking = 0.25keywords = dermis (Clic here for more details about this article) |
10/20. collagen and elastin changes in D-penicillamine-induced pseudoxanthoma elasticum-like skin.A patient with cystinuria who was treated with large doses of D-penicillamine for 19 years developed skin abnormalities resembling those seen in pseudoxanthoma elasticum. Biochemical and histological examination of the dermis showed that the collagen content, the ratio of the major genetic forms of collagen and the distribution of collagen types was normal. light microscopy demonstrated the presence of vastly increased amounts of elastin in the dermis, and the individual elastin fibres were shown by electron microscopy to be abnormal; chemical analysis showed the elastin to be poorly cross-linked. Some of the collagen also appeared structurally abnormal, and biochemically resembled that seen in the dermis of a young child with respect to cross-linking and hexosyl-lysine content. The therapy led to an increased deposition of collagen and elastin fibres which appeared abnormal, and resulted in an increase in total skin surface area. These data indicate that D-penicillamine was not fully effective in inhibiting collagen and elastin cross-linking, and appeared to prevent or inhibit the natural maturation process of the collagen.- - - - - - - - - - ranking = 0.75keywords = dermis (Clic here for more details about this article) |
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