Linear IgA disease histopathologically and clinically masquerading as lichen planus.

In each of 2 cases reported, the patient presented with features of erosive lichen planus or lichenoid drug eruptions and an incisional biopsy taken from the patient was diagnosed histologically as lichen planus. Subsequent recurrences or exacerbations were associated with vesiculobullous lesions. Simultaneous or subsequent direct immunofluorescence studies--from the same tissue sample in one case and from a similar site in the other case--demonstrated classic features of linear IgA disease. Both patients were originally treated for lichen planus with systemic and/or topical corticosteroids with limited success. One patient was treated with sulfapyridine with minimal improvement. Both patients were subsequently treated with dapsone and demonstrated significant clinical improvement. We propose that linear IgA disease may be more common than reported in the oral cavity, inasmuch as many cases of recalcitrant lichen planus, erosive lichen planus, and lichenoid drug eruptions, especially those with a vesiculobullous component, may in reality represent linear IgA disease. We recommend that direct immunofluorescence be done in any case in which bullous lichen planus is suspected.     

Immune-Thrombotic Thrombocytopenic Purpura is a Rare Cause of Ischemic Stroke in Young Adults: Case Reports and Literature Review

Introduction

Immune thrombotic thrombocytopenic purpura (i-TTP), related to acquired ADAMTS-13 dysfunction, can lead to various neurological symptoms including ischemic stroke. To date the clinical, radiological, and biological characteristics of patients having a stroke as the inaugural manifestation of i-TTP are largely unknown.

A meta-analytic review of benefit finding and growth

The authors conducted a meta-analysis to examine the relations of benefit finding to psychological and physical health as well as to a specific set of demographic, stressor, personality, and coping correlates. Results from 87 cross-sectional studies reported in 77 articles showed that benefit finding was related to less depression and more positive well-being but also more intrusive and avoidant thoughts about the stressor. Benefit finding was unrelated to anxiety, global distress, quality of life, and subjective reports of physical health. Moderator analyses showed that relations of benefit finding to outcomes were affected by the amount of time that had passed since stressor onset, the benefit finding measured used, and the racial composition of the sample.     

Cytostatic inhibition of cancer cell growth by lignan secoisolariciresinol diglucoside

Our previous study demonstrated that lignan metabolites enterolactone and enterodiol inhibited colonic cancer cell growth by inducing cell cycle arrest and apoptosis. However, the dietary lignans are naturally present as glycoside precursors, such as secoisolariciresinol diglucoside (SDG), which have not been evaluated yet. This study tested the hypothesis that dietary SDG might have a different effect than its metabolites in human colonic SW480 cancer cells. Treatment with SDG at 0 to 40 μmol/L for up to 48 hours resulted in a dose- and time-dependent decrease in cell numbers, which was comparable to enterolactone. The inhibition of cell growth by SDG did not appear to be mediated by cytotoxicity, but by a cytostatic mechanism associated with an increase of cyclin A expression. Furthermore, high-performance liquid chromatography analysis indicated that SDG in the media was much more stable than enterolactone (95% of SDG survival vs 57% of enterolactone after 48-hour treatment). When the cells were treated with either enterolactone or SDG at 40 μmol/L for 48 hours, the intracellular levels of enterolactone, as measured by high-performance liquid chromatography–mass spectrometry/electron spray ionization, were about 8.3 × 10⁻⁸ nmol per cell; but intracellular SDG or potential metabolites were undetectable. Taken together, SDG demonstrated similar effects on cell growth, cytotoxicity, and cell cycle arrest when compared with its metabolite enterolactone. However, the reliable stability and undetectable intracellular SDG in treated cells may suggest that metabolism of SDG, if exposed directly to the colonic cells, could be different from the known degradation by microorganisms in human gut.     

Intraosseous fibrous lesions of the jaws: a manifestation of tuberous sclerosis

Four patients previously diagnosed with tuberous sclerosis are reported with intraosseous fibrous lesions of the jaws. Review of the literature revealed comparable pathosis occurring in extragnathic bones and several previous reports of similar lesions within the jaws. Therefore, these intraosseous fibrous proliferations are thought to represent an intraoral manifestation of tuberous sclerosis and not coincidental findings. In all 4 cases, the tumors demonstrated significant collagenization with numerous interspersed plump fibroblasts. Although histopathologically similar, the features of the lesions are not specific and also can be found in desmoplastic fibromas and simple odontogenic fibromas. The definitive diagnosis requires appropriate clinicopathologic correlation.     

Immunoprofile of benign and malignant fibrohistiocytic tumors

Formalin-fixed, paraffin-embedded tissue sections from 26 malignant fibrous histiocytomas (MFH) and 61 benign fibrohistiocytic proliferations (BFHP) were evaluated immunohistochemically. An avidinbiotin-peroxidase technique was used to determine immunoreactivity for alpha-1 antichymotrypsin, muramidase, HLA-DR, leucocyte common antigen, S-100 protein, vimentin, desmin, and keratin. MFHs were consistently positive for ACT and vimentin and inconsistently reactive for the other antigens. MFHs were negative for LCA suggesting a mesenchymal origin for these lesions. In the MFH histologic subtypes, antigen expression was not significantly different to be useful in their classification. Also no distinctive pattern emerged relative to immunoreactivity and tumor location. The benign lesions, giant cell tumor of tendon sheath, dermatofibroma, and oral benign fibrous histiocytoma differed from the MFHs in that they were often LCA positive, suggesting origin from hematopoetic mononuclear-macrophages. The immunoprofiles of peripheral fibromas and "giant cell" fibromas were felt to be consistent with origin from mesenchymal cells. Several of the antigens studied could be used to differentiate the benign lesions studied from other benign neoplasms. The antigens were, however, of little value in separation of benign and malignant lesions.     

A molecular blueprint for the pore-forming structure of voltage-gated calcium channels.

A protein that imitates the sequence of a highly conserved segment predicted to line the pore of dihydropyridine-sensitive L-type calcium channels was designed and synthesized. Single-channel conductance properties were studied in planar lipid bilayers. The synthetic protein emulates the ionic conductance, ionic selectivity, and pharmacological properties of the authentic calcium channel, including the stereospecific action of agonist and antagonist enantiomers of the dihydropyridine BayK 8644. The identified sequence is identical in L-type calcium channels from skeletal muscle and isoforms of cardiac muscle, brain, and aorta. It is plausible that this structural motif represents the molecular blueprint for the pore-forming structure of voltage-gated calcium channels.     

Characterization of chronic arthritis in a multicenter study of 852 childhood-onset systemic lupus erythematosus patients

Chronic arthritis (CA) is an unusual condition in childhood-onset systemic lupus erythematosus (cSLE) and data in children is very limited. The aim of the study is to assess CA in a large population of cSLE patients, in a multicenter cross-sectional study including 852 cSLE patients followed in ten Pediatric Rheumatology referral services in state of São Paulo, Brazil. CA was observed in 32/852 (3.7 %) cSLE patients mostly in hands and ankles. Chronic monoarthritis was diagnosed in four cSLE patients, oligoarthritis in nine and polyarthritis in 19. In the latter group, six had rhupus syndrome. Two oligoarticular patients had Jaccoud’s arthropathy. CA was an isolated manifestation observed at disease onset in 13/32 (41 %) cSLE patients, and juvenile idiopathic arthritis (JIA) was the first diagnosis in 18/32 (56 %). The comparison of last visit of patients with CA and without this manifestation revealed higher frequency of splenomegaly (28 vs. 11 %, p = 0.002). The median of SLICC/ACR-DI score [1(0–9) vs. 0(0–7), p = 0.003] was significantly higher in CA patients compared to patients without this manifestation, likewise the frequency of musculoskeletal damage (31 vs. 9 % p = 0.001). Frequencies of treatment with nonsteroidal anti-inflammatory drugs (75 vs. 26 %, p < 0.0001), hydroxychloroquine sulfate (87 vs. 59 %, p = 0.001) and methotrexate (47 vs. 22 %, p = 0.001) were significantly higher in CA patients. This large multicenter study allowed us to characterize CA as a rare and early manifestation of cSLE, frequently mimicking JIA at disease onset. It is predominantly polyarticular, involving more often hands and ankles and it is associated with significant musculoskeletal accrual damage.     

Assessing the psychometric properties of the World Assumptions Scale

The notion that traumatic events shatter survivors' fundamental assumptions about the world has been accepted as a truism in the trauma literature, although tests of this hypothesis provide a somewhat mixed picture. Because this research has relied heavily on the World Assumptions Scale (WAS), it is unclear whether these inconsistent results are due to problems with assumptive world theory, improper tests of the theory, or problems with the WAS. Data were gathered from 742 undergraduate students at four universities to assess the psychometric properties of the WAS. Findings suggest several shortcomings of the WAS, including temporal instability, and mixed evidence regarding construct validity. Implications for trauma research are discussed.