Cases reported "Skin Diseases"

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1/163. Multiple rheumatoid papules characteristic of the early stage of rheumatoid vasculitis.

    The rheumatoid papule has recently been described as the skin manifestation of rheumatoid arthritis (RA). The papules represent leukocytoclastic vasculitis and necrobiosis clinicopathologically. We report a 59-year-old Japanese woman with multiple recurrent rheumatoid papules located on the bilateral extensor forearms in correlation with the level of RA activity and as a cutaneous manifestation of rheumatoid vasculitis. These papules appeared prior to other cutaneous manifestations. It was suggested that rheumatoid papules represent the early stage of rheumatoid vasculitis and that they are a reliable early clinical marker of rheumatoid vasculitis.
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ranking = 1
keywords = arthritis
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2/163. Multiple cutaneous metaplastic synovial cysts.

    The cutaneous metaplastic synovial cyst is a recently described lesion that characteristically presents as a solitary, tender subcutaneous nodule. On histopathologic examination, the lesion is characterized by a cystic structure with villous-like projections and a lining resembling hyperlastic synovium. The cause of cutaneous metaplastic synovial cysts is unclear, but trauma is presumed to be a precipitating factor, as most reported cases have a history of antecedent cutaneous injury. Here we present a case of multiple, bilateral cutaneous metaplastic synovial cysts in a 72-year-old white man with rheumatoid arthritis. This is the first reported case of a patient with multiple lesions. The characteristic clinical and pathologic features of this lesion, along with proposed cause, are reviewed herein.
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ranking = 1
keywords = arthritis
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3/163. lichen planus associated with milia.

    The formation of milia is well recognized in both bullous and inflammatory dermatoses. There are several reports of milia developing in a rare variant of lichen planus pilaris known as lichen planus follicularis tumidus (LPFT), but the association of milia with other types of lichen planus (LP) has not been documented in the literature. We report five patients who developed milia during the course of either drug-induced or idiopathic LP and one in whom milia developed in a lichenoid tattoo reaction. Milia were noted to occur transiently during the resolving phase of LP. Most cases were severe enough to warrant treatment with systemic steroids. The association of milia with LP is not restricted to the rare clinical variant LPFT. We speculate that a severe lichenoid reaction with basal layer degeneration may precipitate the formation of milia in some cases of LP.
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ranking = 0.025517410662625
keywords = idiopathic
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4/163. Optic disc edema in neonatal onset multisystem inflammatory disease (NOMID).

    PURPOSE: To inform ophthalmologists about neonatal onset multisystem inflammatory disease (NOMID), a rare condition with ophthalmologic manifestations. methods: We report a single case of NOMID with optic disc edema. RESULTS: A 28-month-old child with neonatal rash, arthropathy, central nervous system (CNS) involvement, and optic disc edema was diagnosed with NOMID. CONCLUSIONS: The finding of posterior uveitis or optic disc edema in a child with juvenile onset arthritis may allow the differentiation of NOMID from juvenile rheumatoid arthritis.
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ranking = 2
keywords = arthritis
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5/163. sarcoidosis presenting in infancy: a rare occurrence.

    sarcoidosis is infrequent in children. Clinical features of sarcoidosis occurring in children 8-15 years old include pulmonary, lymphnodes and ocular involvement. The picture is similar to that of adult-sarcoidosis. sarcoidosis in infants, however, is rare and it differs from the adult disease. sarcoidosis occurring in infancy has features clinically similar to that of juvenile rheumatoid arthritis. The case presented here is that of an infant who developed sarcoidosis at the age of 3 months. Her illness had all the features of a multisystem illness except for the hilar or parenchymal lung involvement. Because of the complicated and unusual illness the diagnosis of sarcoidosis was not established till the child was five years old. She received corticosteroids and immunosuppressive treatment in varying doses for a long time. Now at the age of 18, the patient's disease seems to have undergone a complete remission.
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ranking = 1
keywords = arthritis
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6/163. Isolated cutaneous response to granulocyte-monocyte colony stimulating factor in fatal idiopathic disseminated bacillus-Calmette-Guerin infection.

    Severe disseminated bacillus-Calmette-Guerin (BCG) infection is very rare and has been regarded as idiopathic when no immunodeficiency is present. This entity seems to be due to several new types of inherited abnormalities in the pathways important in defence against Mycobacteria. Although improvement with interferon-gamma (IFN-gamma) has been reported in some patients, to our knowledge there are no reports on the effect of other cytokines in the treatment of these patients. We report here the clinical response to IFN-gamma and granulocyte-monocyte colony stimulating factor (GM-CSF) treatment in a patient with idiopathic disseminated BCG infection who failed to respond to multiple antimycobacterial agents. The patient showed partial and transitory response to IFN-gamma, however, GM-CSF treatment led to rapid improvement of skin lesions within 2 weeks without any effect on the progression of the disease in the other organ systems. CONCLUSION: The response of idiopathic disseminated bacillus-Calmette-Guerin infection to granulocyte-monocyte colony stimulating factor treatment was limited to cutaneous lesions. Granulocyte-monocyte colony stimulating factor may have acted to promote wound healing or the levels of this factor achieved in other affected organs may have been inadequate.
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ranking = 0.17862187463837
keywords = idiopathic
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7/163. Exclusion of linkage between dyschromatosis symmetrica hereditaria and chromosome 9.

    Dyschromatosis symmetrica hereditaria (DSH) is an autosomal dominant pigmentary disorder, first reported by Toyama in 1910. It is characterized by a mixture of hypopigmented and hyperpigmented macules of various sizes on the backs of the hands and feet. The disease gene of DSH and its chromosomal localization have not yet been identified. A family with DSH and idiopathic torsion dystonia (ITD), a rare neurological disease, was recently reported. Therefore, we speculated that there was a linkage between the DSH gene and the ITD gene, named DYT1 and localized on chromosome 9, and performed linkage analysis between DSH and microsatellite markers on chromosome 9 in three Japanese DSH families (36 patients in total). We obtained a lod score of < -2 over the whole region of chromosome 9 encompassing DYT1. Thus, we conclude that there is no linkage between DSH and DYT1 as well as any region of chromosome 9.
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ranking = 0.025517410662625
keywords = idiopathic
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8/163. Idiopathic calcinosis of the areola of the nipple.

    Idiopathic calcinosis cutis involving the breast is a rare condition. Previously reported cases were detected by mammography without specific cutaneous findings. We report a case of idiopathic calcinosis of the areola of the nipple in a 32-year-old Korean woman that has unique clinical features resembling scrotal calcinosis.
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ranking = 0.025517410662625
keywords = idiopathic
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9/163. Cutaneous histiocytic lymphangitis: an unusual manifestation of rheumatoid arthritis.

    Two cases are presented of unusual cutaneous lesions associated with rheumatoid arthritis in underlying joints. The lesions were evanescent, erythematous and violaceous partly macular and partly indurated plaques, with a livedo-like pattern of erythema at the edge in one case. Histological changes were identical in the two cases. The major features were dilated, dermal lymphatics containing aggregates of inflammatory cells, mainly histiocytes, with adjacent perivascular lymphoid aggregates. An appropriate name for this reaction would appear to be cutaneous histiocytic lymphangitis.
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ranking = 5
keywords = arthritis
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10/163. Chronic infantile neurological cutaneous and articular/neonatal onset multisystem inflammatory disease syndrome: ocular manifestations in a recently recognized chronic inflammatory disease of childhood.

    OBJECTIVE: To report on the ocular manifestations of the Chronic Infantile Neurological Cutaneous and Articular/Neonatal Onset Multisystem Inflammatory disease (CINCA/NOMID) syndrome, a rare, recently identified, pediatric multisystem inflammatory disease with chronic cutaneous, neurological, and articular manifestations. DESIGN: Descriptive case-report study. SETTING: International collaborative study based on a questionnaire. RESULTS: We included 31 patients. The mean age at onset of eye manifestations was 4.5 years. Optic disc changes were the most common feature, occurring in 26 patients (83%), including optic disc edema, pseudopapilledema, and optic atrophy. Anterior segment manifestations varying from mild to severe were seen in 13 patients (42%); chronic anterior uveitis, in 17 patients (55%). Moderate to severe visual acuity loss in at least 1 eye was seen in 8 patients (26%) as a consequence of the disease. Posterior synechia, glaucoma, and white iritis were not observed in any patient. CONCLUSION: Ocular manifestations with potentially sight-threatening complications occur commonly in the CINCA/NOMID syndrome. The distinctive nature of these complications may assist the ophthalmologist in recognizing this rare disorder and distinguishing it from juvenile rheumatoid arthritis.
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ranking = 1
keywords = arthritis
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