Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic ulcers of the skin were observed in three Belgian Landrace sows. Lesions were located on ears, limbs, and in the mammary region and were resistant to treatment that included corticosteroid therapy. Major histologic changes were located at the dermo-epidermal junction. Ulcers were deep, and the adjacent epidermis had marked hyperkeratosis, acanthosis, and intracellular edema. Dermatitis was prominent, essentially located in the superficial dermis. By electron microscopy, basal lamina appeared disrupted. Dermo-epidermal separation occurred beneath the basal lamina. Collagen was morphologically normal. Desmosomes, hemidesmosomes, and anchorage fibers were present in areas adjacent to lesions. Ulcerative dermatitis of sows is morphologically similar to pemphigus, pemphigoid, systemic lupus erythematosus, epidermolysis bullosa simplex, erythema multiforme, toxic epidermal necrolysis, and drug eruption. However, significant differences exist between ulcerative dermatitis and these conditions.
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PMID:Pathology of an ulcerative dermatitis in Belgian Landrace sows. 403 39

The MRL-lpr/lpr(MRL/l) mouse is a new animal model for human systemic lupus erythematosus (SLE) and skin lesions with hair loss and scab formation are one of the characteristic manifestations in this mouse. We investigated the histopathology of the skin lesions in MRL/l mice and studied the related autoimmune phenomenon. Light microscopical observations revealed hyperkeratosis, acanthosis, hypergranulosis, liquefaction, vasodilation in the dermis and T-cell infiltration into the dermis at the age of 5 months (mo). Immunohistological studies showed the presence of immunoglobulins and/or complement depositions at the dermal-epidermal junction (DEJ). In some mice there was deposition of immunoglobulin at the DEJ at 2 mo and in 90%-100% of MRL/l mice at over 5 mo. Temporal relationship was present among cutaneous immunoglobulin depositions, the occurrence of anti-DNA antibodies and proteinuria. These findings suggest that MRL/l mice might provide a new aid for studying the biological mechanisms of the development of skin lesions in human SLE.
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PMID:Dermatopathological studies on skin lesions of MRL mice. 638 29

A detailed histopathologic study was conducted blindly on biopsy specimens from 66 patients with oral discoid lesions (oral DLE) mixed in random order with biopsy specimens from control groups of 25 cases of oral lichen planus (LP), 25 cases of oral leukoplakia, 13 cases with an uncertain diagnosis termed DLE? LP? and 7 cases of galvanic lesions related to amalgam fillings. Fifty-two histopathologic variables were examined, and a ranking was calculated for the variables that provided the best discrimination between the subgroups of clinically typical oral DLE and LP and between typical DLE and leukoplakia, respectively. Histopathologic variables not previously described in oral DLE but characteristic of this disease were identified: multinucleated epithelial cells containing three or more nuclei and the presence of epithelial islands in the connective tissue. Patients with systemic lupus erythematosus (SLE) in addition to oral DLE showed generally the same histopathologic features as those without SLE. The inflammatory infiltrates were slighter and more diffuse among the SLE patients as compared to the non-SLE patients. The simplest possible combination of histopathologic variables diagnostic for oral DLE consisted of the following five: (1) hyperkeratosis with keratotic plugs, (2) atrophy of the rete processes, (3) deep inflammatory infiltrate, (4) edema in the lamina propria, and (5) thick patchy or continuous PAS-positive juxtaepithelial deposits. This combination is suggested as new histopathologic criteria for oral DLE. These criteria were tested among clinically atypical cases of DLE and the other groups of mucosal lesions, with a sensitivity of 92% and a specificity of 96% against both LP and leukoplakia for the presence of two or more of the five criteria.
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PMID:Oral discoid lupus erythematosus. III. A histopathologic study of sixty-six patients. 658 19

The patient is a newborn girl, born at term by cesarian section, weighing 3,550 g, and measuring 50 cm in length. According to her mother, she had been presenting erythematous spots involving the scalp, face, and trunk since birth that had increased in size with time. The mother denied any other changes and reported normal growth and development. Physical examination at 2 months of age revealed an infant in good general condition, hydrated, with no fever and no abnormalities detected on careful physical examination. The dermatologic examination revealed numerous circumscribed erythematous-brownish flat maculae with sharp borders and irregular contours, with no follicular hyperkeratosis, but with telangiectasias and areas of atrophy. They were lenticular and nummular, especially on the face (peri-orbital heliotropic lesions), and appeared as plaques on the trunk, with a tendency to confluence. The lesions also involved the scalp, neck, and extremities (Fig. 1). Histopathologic examination revealed an atrophic epidermis with focal areas of hydropic degeneration of the basal layer. A discrete perivascular and periannexal lymphohistiocytic infiltrate was observed in the superficial dermis, with melaninophages, frequent extravasation of red blood cells, angiectasia, and edema (Fig. 2). Serologic tests were reactive for antinuclear factor (ANF) (titer 1:100) of the speckled pattern, the presence of anti-Ro antibodies, and absence of anti-RNA and anti-Sm antibodies (Table 1). Blood counts and electrocardiogram were normal (Table 1). HLA typing showed positivity for DR-3 (Table 2). At 5 months of age the patient already showed a marked improvement of the skin lesions with only some areas of discrete pigmentation, a few atrophic areas, and rare telangiectasis (Fig. 3). The serologic tests (ANF, anti-Ro) had become nonreactive and the anti-RNA and anti-Sm tests continued to be negative. Examination of the mother revealed an asymptomatic 25-year-old woman reporting no manifestations suggestive of lupus. General and special physical examination revealed no abnormalities. Dermatologic examination showed no active or residual lesions of discoid or systemic lupus erythematosus. The pregnancy had been uneventful. Histopathologic examination of the girl's skin revealed the epidermis without obvious changes, minimal edema in the dermis, and a discrete perivascular inflammatory mononuclear cell infiltrate. Direct immunofluorescence of normal skin not exposed to the sun was negative. Blood counts revealed mild anemia and a tendency to leukopenia and thrombocytopenia (Table 1). Serologic tests showed reactive ANF (titers 1:400 and 1:800, speckled pattern), the presence of anti-Ro antibodies, and the absense of anti-RNA and anti-Sm antibodies (Table 1). HLA-typing revealed positivity for DR-3 (Table 2).
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PMID:Neonatal lupus erythematosus. 883 29

Four cases of Hypertrophic Lupus Erythematosus (HLE) were reported. The lesions of HLE were observed on the forearms, face and hands in all four cases. Clinically, the lesions were erythematous, hyperkeratotic plaques. The clinical course was marked by chronicity and progression of the lesion. Histologically, marked hyperkeratosis, parakeratosis, acanthosis, degenerative changes of basal cells in H/E stain, and thickened, multilayered basement membrane in PAS stain, were observed. The observations of Dylon stain revealed that localized amyloid deposition was observed in all four cases of HLE lesions, as fluorescent-orange colored amyloid deposits in the papillary dermis and subepidermal areas at near orjust below the dermo-epidermal junction appeared under fluorescent microscope. On the basis of clinical and histological observations, we suggest that chronic irritation, such as sunlight exposure over a long-duration, might have caused the characteristic abnormalities at the dermo-epidermal junction and also initiated the frequency of amyloid deposits locally secondary to the diseases. We compared our HLE cases to other types of lupus erythematosus (LE) skin lesions, as to whether deposition of amyloid materials were frequently observed or not. Amyloid deposition was observed in one case of DLE and none of the SLE cases. Localized amyloid deposition was more frequently observed in skin lesions, secondary to HLE disease, as compared to other types of LE.
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PMID:Amyloid deposition is frequently observed in skin lesions of hypertrophic lupus erythematosus. 1243 94

Scarring alopecia is a very frequent feature of chronic discoid lupus erythematosus (DLE). So far in the literature, only clinic-pathologic features or histopathologic-immunopathologic traits of DLE scarring alopecia (DLESA) have been reported. We describe the most significant features of clinical morphology, histopathology, serum and tissue immunopathology of 36 DLESA patients (41.9% of all our scarring alopecia patients). Clinically, 33.3% presented a single lesion and 52.7% presented multiple lesions of scarring alopecia, while 13.8% exhibited a picture resembling Pseudopelade of Brocq, with the classic 'footprints in the snow' appearance. The most frequent morphologic features were sclero-atrophy (80.5%) and erythema (63.8%). The main histopathologic aspects appeared to be fibrosis (100%), follicular hyperkeratosis (91.4%), epidermal atrophy (88.5%), lymphocytic infiltrate (88.5%), thickened basement membrane (77.1%) and basal vacuolar degeneration (74.2%). Antinuclear antibodies were present in 42.8% of patients and antigastric mucosa, antithyroid and anticardiolipin antibodies in 17-21% of patients. A positive lupus band test was demonstrated in 81.8% of cases and perivascular deposit in 30.3% of patients. Histopathology alone allowed a correct diagnosis only in 68.5% of cases; in the other cases, the diagnosis was assessed also taking into account immunopathologic findings. Our study defines the clinic, histopathologic and immunopathologic features of DLESA patients and points out that a multiparametric approach is mandatory to assess the diagnosis of DLESA.
Lupus 2004
PMID:Scarring alopecia in discoid lupus erythematosus: a clinical, histopathologic and immunopathologic study. 1530 73

A 54-year-old man of Persian origin presented to our department with a 1-year history of ulcers on the right leg that had been unresponsive to numerous topical treatments, accompanied by lymphedema of the right leg. Medical history included hypergonadotropic hypogonadism, which had not been further investigated. He was treated for 20 years with testosterone IM once monthly, which he stopped a year before the current hospitalization for unclear reasons. The patient reported no congenital lymphedema. Physical examination revealed two deep skin ulcers (Figure 1) on the right leg measuring 10 cm in diameter with raised irregular inflammatory borders and a boggy, necrotic base discharging a purulent hemorrhagic exudate. Bilateral leg pitting edema and right lymphangitis with lymphadenitis were noted. He had low head hair implantment, sparse hair on the body and head, hyperpigmentation on both legs, onychodystrophia of the toenails (mainly the large toe and less prominent on the other toes), which was atrophic lichen-planus-like in appearance and needed no trimming (Figure 2), normal hand nails, oral thrush, and angular cheilitis. Other physical findings were gynecomastia, pectus excavatum, small and firm testicles, long extremities, asymmetrical goiter, systolic murmur 2/6 in left sternal border, and slow and inappropriate behavior. The patient's temperature on admission was 39 degrees C. Blood cultures were negative for bacterial growth. Results of laboratory investigations included hemoglobin (11.2 g/dL), hematocrit (26.8%), normal mean corpuscular volume and mean corpuscular hemoglobin volume, and red blood cell distribution width (16%). Blood smear showed spherocytes, slight hypochromia, anisocytosis, macrocytosis, and microcytosis. Blood chemistry values were taken for iron (4 micro g/dL [normal range 40-150 micro g/dL]), transferrin (193 mg/dL [normal range 220-400 mg/dL]), ferritin (1128 ng/mL [normal range 14-160 ng/mL]), transferrin saturation (1.5% [normal range 20%-55%]), serum folate (within normal limits), and vitamin B12 (within normal limits). Direct Coombs' test equaled positive 2 + IgG. All these values indicated anemia of chronic diseases combined with hemolytic anemia. Further blood work-up tested antinuclear antibody (positive <1:80 homogeneous pattern), rheumatoid factors (143 IU/mL [positive >8.5 IU/mL]), C-reactive protein (286 mg/L [normal range 0-5 mg/L]), anticardiolipin IgM antibody (9.0 monophosphoryl lipid U/mL [normal range 0-7.00 MPL U/mL]) and antithrombin III activity (135% [normal range 74%-114%]). Results of other blood tests were within normal limits or negative, including lupus anticoagulant, beta2 glycoprotein, anticardiolipin IgG Ab, anti-ss DNA Ab, C3, C4, anti-RO, anti-LA, anti-SC-70, anti-SM Ab, P-ANCA, C-ANCA, TSH, FT4, anti-T microsomal, antithyroglobulin, protein C activity, protein S free, cryoglobulins, serum immunoelectrophoresis, VDRL, hepatitis C antibodies, hepatitis B antigen, and human immunodeficiency virus. Endocrinological work-up examined luteinizing hormone (22.9 mIU/mL [normal range for adult men 0.8-6 mIU/mL]), follicle stimulating hormone (49.7 mIU/mL [normal range for adult men 1-11 mIU/mL]), testosterone (0.24 ng/mL [normal range for adult men 2.5-8.0 ng/mL]), bioavailable testosterone (0.02 ng/mL [normal range for adult men >0.6 ng/mL]), and percent bioavailable test (8.1% [normal value >20%]). These results indicate hypergonadotropic hypogonadism. Plasminogen activator inhibitor 1 was 6 U (normal value 5-20 U/mL). Karyotyping performed by G-banding technique revealed a 47 XXY karyotype, which is diagnostic of Klinefelter's syndrome. Doppler ultrasound of the leg ulcers disclosed partial thrombus in the distal right femoral vein. X-rays and bone scan displayed osteomyelitis along the right tibia. Histological examination of a 4-mm punch biopsy from the ulcer border revealed hyperkeratosis, acanthosis, hypergranulosis, and mixed inflammatory infiltrate containing eosinophils compatible with chronic ulcer. Multiple vessels were seen, compatible with a healing process. Direct immunofluorescence of the biopsy revealed granular IgM in the dermo-epidermal junction. Indirect immunofluorescence was negative. Thyroid function tests showed normal thyroid stimulating hormone and free throxine4. Multinodular goiter was seen on thyroid scan and ultrasound. Thyroid fine needle aspiration was compatible with multinodular goiter (normal follicular cells, free colloid, macrophages with pigment). IV treatment with amoxicillin-clavulanic acid 1 g t.i.d. was administered for 2 weeks, with a decrease in temperature and normalization of the leukocyte level. Oral antibiotic treatment with amoxicillin-clavulanic acid was continued for 10 more days, followed by 25 days of ciprofloxacin for the osteomyelitis. Local treatment included saline soakings followed by application of Promogran (Johnson & Johnson, New Brunswick, NJ) and Kaltostat (ConvaTec Ltd., a Bristol-Myers Squibb Company, New York, NY) with slight improvement. At the same time, the patient was treated with warfarin sodium due to deep vein thrombosis under international normalized ratio 2-3. The patient was treated with IM testosterone once monthly for 1 year, which resulted in a reduction in the diameter and depth of the leg ulcers (Figure 3). Blood tests were not performed for follow-up of the immune state.
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PMID:Klinefelter's syndrome presenting with leg ulcers. 1536 65

Nail changes occur in about 25% of systemic lupus erythematosus (SLE) cases. Onycholysis has been reported as the most frequent abnormality in SLE. Nailbed hyperkeratosis may be observed in both SLE and discoid lupus erythematosus (DLE). Involvement of the nail apparatus in DLE is extremely uncommon and never restricted to it. We report on a patient in whom the clinical features on the proximal nailfold were similar to those observed on the skin of a patient with typical DLE. This has, to the best of our knowledge, not yet been reported. The patient also exhibited a very distinctive prominent subungual hyperkeratosis. Interestingly, the patient developed biological alterations suggesting a systematization of the disease. Only a combination of systemic corticoids, retinoids and antimalarials was able to achieve nail improvement and this partial resistance to therapy may be explained by the very unusual subungual hyperkeratosis.
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PMID:Hyperkeratotic nail discoid lupus erythematosus evolving towards systemic lupus erythematosus: therapeutic difficulties. 1548 10

A retrospective study was performed to assess the prevalence of apoptotic epidermal keratinocytes in biopsy specimens from 226 horses with inflammatory dermatoses and from 27 normal specimens. One or more apoptotic keratinocytes were found in specimens from 28 of 226 (12%) horses with various dermatoses, and in one of 27 (4%) specimens from normal horses. The prevalence (proportion of cases with apoptotic epidermal keratinocytes) of apoptotic keratinocytes in the group composed of discoid lupus erythematosus, erythema multiforme, photodermatitis, and systemic lupus erythematosus was significantly greater (52% prevalence in these cases) than that in a grouping of all other dermatoses (prevalence 8%). There was no correlation between the number of apoptotic keratinocytes and the extent of epidermal hyperplasia or hyperkeratosis.
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PMID:The prevalence of apoptotic keratinocytes in equine epidermis: a retrospective light-microscopic study of skin-biopsy specimens from 253 horses with normal skin or inflammatory dermatoses. 1561 Apr 80

A 32-year-old woman presented with fever and swelling in the axillae of 2 months' duration, and erythema of the face, fluid-filled lesions on the trunk, oral ulcers, crusting of the lips, and redness and watering of the eyes for 3 days. The patient was initially diagnosed with tuberculous lymphadenitis and was given antituberculous treatment. One month later, she developed the present complaints (see below). The patient was photosensitive. There was no joint pain or Raynaud's phenomenon. She had experienced three intrauterine deaths in the past with no live births. On examination, the patient was pale. Bilateral axillary lymphadenopathy was present. The lymph nodes were mobile, non-tender, and not matted. Mucocutaneous examination revealed a malar eruption, flaccid bullae on the back (Fig. 1), crusting of the lips (Fig. 2), oral ulcers, and redness and discharge from the eyes. On investigation, immunoglobulin G (IgG), IgM, and IgA for tuberculosis were negative. There was anemia and leukopenia, the erythrocyte sedimentation rate (ESR) was raised, albumin in urine was positive, enzyme-linked immunosorbent assay (ELISA) for human immunodeficiency virus (HIV) was negative, and venereal disease research laboratory (VDRL) test was nonreactive. Antinuclear antibody (ANA), dsDNA, and antiphospholipid antibody were positive. Fine needle aspiration cytology (FNAC) of the axillary lymph node showed loss of nodal architecture. The extensive infiltrate consisted of lymphocytes, histiocytes, immunoblasts, and necrosis of cortical and paracortical areas. There was histiocytic necrotizing lymphadenitis without granulocytic infiltration. These features were suggestive of Kikuchi-Fujimoto's disease (Fig. 3). Skin biopsy showed epidermal atrophy, basal cell vacuolation, focal hyperkeratosis, pilosebaceous atrophy, and follicular plugging. The dermis showed edema and a lymphocytic infiltrate in the upper dermis and around the blood vessels. These features were suggestive of systemic lupus erythematosus (SLE). Direct immunofluorescence of lesional skin showed a strong continuous basement membrane zone (BMZ) band of C3 and fibrinogen and a strong discontinuous granular BMZ band of IgG. IgA was negative. Covered skin showed moderate and strong positivity for IgM and IgG, respectively. C3, IgA, and fibrinogen were negative. These findings were suggestive of SLE (Fig. 4). Based on the clinical findings and investigations, a diagnosis of Kikuchi-Fujimoto's disease with SLE was made.
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PMID:Is Kikuchi-Fujimoto disease a manifestation of systemic lupus erythematosus? 1665 Jan 77


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