Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dermatofibroma (DF) is a benign skin tumor that is well-known among dermatologists. We herein present a rare case of atrophic dermatofibroma presenting linear skin dimpling. The patient was a 25-year-old woman with a history of wild-type recessive dystrophic epidermolysis bullosa who had noticed linear concavity on her right lateral back 1 year before her initial presentation. Anetoderma, atrophic scar, localized morphea, or lupus erythematosus profundus were clinically suspected; however, a biopsy specimen from the dimpling lesion showed the fibrous and histiocytic tumor in the deep dermis. The spindle-to-rhomboid-shaped tumor cells were arranged with irregularly storiform pattern, and immunohistochemistry showed that the tumor cells were positive for factor XIIIa, and negative for CD34 and CD68. Elastica van Gieson staining showed an almost complete loss of elastic fibers, especially at the center of the lesion. The reduction of elastic fibers might have influenced the skin depression in this case. This rare case suggests the need to consider a subtype of DF in the differential diagnosis of dimpling skin lesions.
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PMID:A Rare Case of Atrophic Dermatofibroma Featuring Linear Skin Dimple. 3161 78

Discoid or chronic lupus erythematosus is an autoimmune disease that produces skin lesions on the face and scalp. Rarely do lesions present with linear configuration, but when they do, the lesions often follow the lines of embryologic migration. A 24-year-old man presented with a slowly progressing asymptomatic violaceous linear patch running from the root of his frontal scalp to the nasal tip. A Doppler ultrasound and skin biopsy were performed and the histological findings demonstrated characteristic findings of discoid lupus erythematosus. A full physical examination, review of systems and laboratory investigations showed no indication of systemic lupus. High potency topical steroids and calcineurin inhibitors were prescribed along with photoprotection. At 4-month follow-up, all his lesions had mostly cleared. We report here the first case, to our knowledge, of discoid lupus erythematosus with en coup de saber presentation mimicking morphea.
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PMID:Linear discoid lupus erythematosus mimicking en coup de sabre morphea: A case report. 3166 54

A 60-year-old male patient presented with a solitary pink plaque over the inner aspect of the left forearm of 4 years duration. The lesion was persistent and unresponsive to treatment. A skin biopsy was done with differential diagnosis of lupus vulgaris, tuberculoid leprosy, and granuloma annulare. Histopathology revealed features suggestive of morphea and the patient was treated accordingly. Despite treatment, the lesion continued to grow in size and a repeat biopsy was done as clinical doubt regarding the diagnosis persisted. Dermoscopy, KOH wet mount, and histopathology revealed characteristic features that clinched the diagnosis and aided in correct management of the patient with complete clearance of lesion after 4 months of therapy.
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PMID:The Curious Case of an Elusive Solitary Plaque. 3247 7

There are a number of conditions that follow the lines of Blaschko. Linear discoid lupus erythematosus is a rare variant of chronic lupus erythematosus with less than 20 cases reported in children. It can be misdiagnosed as lichen striatus or linear morphea. We describe a 15-year-old boy with a confirmed histologic diagnosis of linear chronic discoid lupus erythematosus following the lines of Blaschko, with no signs of systemic involvement.
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PMID:Linear lesions on the arm of a child: a diagnostic challenge. 3262 98

The autoimmune basis of segmental vitiligo (SV) has only recently been recognized. Systemic autoimmune diseases are less frequently associated compared to non-segmental vitiligo (NSV), but localized skin disorders - in particular linear morphea - have been repeatedly observed in patients with SV. The inflammatory response is documented on a clinical level with cases displaying erythematous borders or a hypochromic stage, on a histopathological level with predominantly CD8 lymphocytes migrating toward the basal layer and by flow cytometry demonstrating the antimelanocyte specificity of these cytotoxic T cells. The increased risk for halo naevi and NSV in these patients further underline the immune-mediated mechanisms of SV. Nonetheless, the localized and unique distribution pattern points to somatic mosaicism. This places SV in a category of similar diseases such as lichen striatus, blaschkitis, linear lupus erythematosus, and linear scleroderma where an immune reaction against genetically mutated skin cells is believed to be the underlying cause. All these disorders are characterized by a young age of onset, a temporary disease activity with spontaneous resolution, limited response to treatment, and often long-term sequelae. Although challenging, genetic research proving this genetic mosaicism could offer crucial insights into the pathogenesis of both segmental and non-segmental vitiligo.
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PMID:Autoimmunity in Segmental Vitiligo. 3319 42


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