1/14. purpura as a cutaneous association of sickle cell disease.A common chronic feature of sickle cell disease is the presence of painful, punched-out leg ulcers. Other cutaneous findings in patients with homozygous sickle cell disease have not been described in the literature. We present a case of a 50-year-old black woman with sickle cell disease who was admitted for acute onset of arm and hip pain. After admission she deteriorated clinically, with multiorgan failure and mental status changes. Examination of the skin revealed erythematous papules and plaques with scaly centers and purpura on the upper trunk. The clinical differential diagnosis was vasculitis versus sepsis. skin biopsy of two representative lesions was performed. hematoxylin- and eosin-stained sections showed a superficial perivascular mixed inflammatory infiltrate with numerous eosinophils and extravasated erythrocytes, some of which exhibited bizarre morphology of sickled red blood cells. These findings indicated that the patient's cutaneous lesions, possibly multifactorial in origin, could be a component of her sickle cell crisis. This case is presented as an unusual one in which evaluation of erythrocyte morphology contributed to patient management and to emphasize the importance of examining erythrocyte morphology as a part of the histologic evaluation of stained tissue.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/14. Haemorrhagic papular rash associated to Flavimonas oryzihabitans bacteraemia in a child.Flavimonas oryzihabitans is a gram-negative rod that has rarely been implicated in human infections. The involvement of this organism has been documented in serious infections, the majority of which were cases of bacteraemia or peritonitis. We report the first isolation of the organism in greece, from a case of bacteraemia, associated with haemorrhagic papular rash, in a paediatric patient and describe the phenotypic characteristics of the strain.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
3/14. Benign Degos' disease developing during pregnancy and followed for 10 years.Degos' disease, or malignant atrophic papulosis, is a rare and often fatal multisystem vasculopathy of unknown etiology. The cutaneous manifestations comprise erythematous papules, which heal to leave scars with a pathognomonic central porcelain-white atrophic area and a peripheral telangiectatic rim. Involvement of the gastrointestinal tract is observed in 50% of cases, with intestinal perforation being the most common cause of death. Other organ systems can also be affected; 20% of cases involve the central nervous system. Systemic manifestations usually develop from weeks to years after onset of skin lesions or, in rare instances, may precede skin lesions. In the patient with Degos' disease reported in this article, the characteristic skin lesions developed during pregnancy, a precipitating event not previously reported. She has survived an unusually long time (10 years) without visceral or neurological involvement, despite florid cutaneous lesions. Moreover, we could detect the presence of antiphospholipid antibodies, the significance of which are currently unclear. These observations therefore confirm that there may be a strictly cutaneous form of Degos' disease with a favourable prognosis.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/14. Benign cutaneous Degos' disease.malignant atrophic papulosis is a rare systemic vaso-occlusive disorder characterized by thrombosis of vessels of the dermis, gastrointestinal tract, central nervous system and, occasionally, other organs. Cutaneous lesions consist of erythematous, dome-shaped papules that develop a central area of necrosis to leave a porcelain-like scar. The most accepted theory of pathogenesis is based on endothelial cell damage. There is no effective treatment of the disease. We describe a 26-year-old man with Degos' disease, a diagnosis based on the clinical and histologic pattern of skin lesions. The good response to antiplatelet therapy and the absence of systemic involvement over 8 years' follow-up is noteworthy. We believe that this case represents the benign form of the disease, typically referred to as benign cutaneous Degos' disease.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
5/14. malignant atrophic papulosis. Report of a case with multiple ophthalmic findings.malignant atrophic papulosis is a rare and fatal condition with multiple organ involvement. We describe a patient with progressive ocular and dermatological findings.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
6/14. Nervous system involvement in Degos disease.Degos disease, or malignant atrophic papulosis, is a rare obstructive vasculopathy of unknown origin, characterized by distinctive skin lesions, visceral involvement, and an unfavorable outcome. The gastrointestinal tract and the central nervous system are most frequently affected, but cases limited to benign skin lesions have also been described. Neuroradiologic reports of this condition are exceptionally rare. We report the case of a 29-year-old woman with central and peripheral nervous system involvement who presented with progressive clinical deterioration and a meningovascular pattern at cerebral MR imaging; other organs were spared in this patient.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
7/14. A case of systemic malignant atrophic papulosis (Kohlmeier-Degos' disease).malignant atrophic papulosis (MAP) is a rare and clinically distinctive vasculopathy. It is characterized by narrowing and occlusion of the lumen by intimal proliferation and thrombosis, which leads to ischemia and infarction in the involved organ systems. Its rarity and rapidly fatal course make the disease a difficult diagnostic and therapeutic challenge.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
8/14. Degos disease with an inconspicuous clinical course.BACKGROUND: Degos disease is a rare systemic disorder with involvement of the skin and visceral organs, leading to death in about 50% of cases within 1 or 2 years. In recent years, several cases with cutaneous lesions only have been recognized. methods: We report on a young male patient presenting with single inconspicuous papules with bluish/black centres on the trunk and the upper limbs that, upon healing turn white. These lesions recurred on different locations over the past 6 years, and were never more than two to three at one time. RESULTS: Histopathological examinations revealed archetypal features for Degos disease. The patient had no other complaints, neither visceral organs nor the central nerve system were involved. Laboratory examinations were within normal range. CONCLUSIONS: This case increases the number of reports on a benign course of Degos disease. It raises the question if the 'malignant' and the 'benign' course of the disease represent two distinct diseases or variants of a systemic vasculitis with unknown cause.- - - - - - - - - - ranking = 2.1772853185596keywords = organ, nerve (Clic here for more details about this article) |
9/14. clomipramine-induced hypersensitivity syndrome with unusual clinical features.Drug-induced hypersensitivity syndrome is a severe drug eruption with fatal visceral organ failures associated with human herpesvirus-6 (HHV-6) reactivation. We present a case of clomipramine-induced hypersensitivity syndrome, which displayed in succession two different reactions akin to those described for Gianotti-Crosti syndrome and infectious mononucleosis. HHV-6 reactivation was confirmed serologically after the latter reaction, suggesting that our case may have represented a biphasic reaction to HHV-6 reactivation.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
10/14. optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease).A fatal case of malignant atrophic papulosis (Degos disease) with optic nerve and spinal cord involvement is described. magnetic resonance imaging (MRI) of the optic nerve showed abnormal signal enhancement on fat suppressed T1 weighted images after intravenous meglumine gadopentetate infusion. On T2 weighted sagittal images, a sawtooth pattern was observed over seven vertebral segments of the spinal cord. On necropsy, a severe loss of myelinated nerve fibres in the left optic nerve was seen, with thrombotic obstruction of the central retinal artery. Spongy degeneration was observed in all levels of the spinal cord, with patchy and motheaten patterns caused by thromboses and endothelial proliferation in subarachnoid vessels. Findings on MRI were consistent with findings on pathological examination.- - - - - - - - - - ranking = 1.4182825484765keywords = nerve (Clic here for more details about this article) |
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