A rare case of cutaneous lupus erythematosus presenting with periorbital erythema and edema.

Cutaneous lupus erythematosus (CLE) is an autoimmune skin disease that can manifest itself with a variety of skin symptoms. Periorbital erythema, a rare variant of CLE, presents challenges in terms of diagnosis and treatment. Here, we report a case of CLE presenting with periorbital erythema and edema. A 42-year-old female patient presented with complaints of erythema, edema, and scaling on the right eyelid that started four months ago. A skin biopsy was performed on the lesioned skin of the eyelid to differentiate dermatomyositis, cutaneous lupus erythematosus, sarcoidosis, lupus vulgaris, and cutaneous lymphoma. Histopathological examination revealed focal hyperkeratosis and parakeratosis on the surface of the epidermis, vacuolar degeneration in the basal layer of the epidermis, lymphocyte exocytosis with necrotic keratinocytes, edema in the dermis, melanophages, and perivascular, periadnexal lymphocytic reaction. Laboratory tests showed negative antinuclear antibody and anti-dsDNA, but positivity for anti-Ro-52. In the absence of any other complaints, the patient was diagnosed with cutaneous lupus erythematosus presenting with periorbital erythema based on clinical, histopathological, and laboratory findings. Hydroxychloroquine 200 mg/day, topical corticosteroid, and topical tacrolimus were administered. Two months later, significant improvement in the lesions was observed. In conclusion, it should be kept in mind that periorbital erythema can develop as a rare variant of CLE and can be misdiagnosed as contact dermatitis, dermatomyositis, sarcoidosis, or cutaneous lymphoma. Additionally, the ANA and anti-dsDNA antibodies are often found to be negative in these cases. In establishing the diagnosis, firstly considering the disease, followed by histopathological examinations and laboratory tests, is crucial.

STK4 deficiency and EBV-associated lymphoproliferative disorders, emphasis on histomorphology, and review of literature.

Aberrations of the STK4 gene in humans result in an autosomal recessively inherited primary immunodeficiency. We identified three patients with STK4 deficiency who had presented to our hospital and reviewed their biopsy samples with the goal of detailing the characteristics of STK4 deficiency from a pathology perspective. Case 1 was a 20-year-old male who presented with cervical and supraclavicular lymphadenopathy which showed plasmacytic hyperplasia and a concurrent bronchial mass, with AA amyloidosis and EBV-associated "polymorphic lymphoproliferative disorder (LPD) resembling polymorphic post-transplant LPD." The second case was an 8-year-old girl with abdominal lymphadenopathy; biopsy revealed a complex lymphoproliferation which consisted of EBV-associated "polymorphic LPD resembling polymorphic post-transplant LPD," plasmacytic hyperplasia, granulomatous reaction, and a CD4- and PD-1-positive clonal T cell proliferation. The third was a 15-year-old girl with a laryngeal mass, representing a high-grade B cell lymphoma with prominent plasmacytic differentiation. Our cases emphasize the complex and challenging histopathology of lymphoid proliferations in patients with STK4 deficiency.

Reduced immunohistochemical PTEN staining is associated with higher progression rate and recurrence episodes in non-invasive low-grade papillary urothelial carcinoma of the bladder.

Non-invasive low-grade papillary urothelial carcinoma (NILGPUC) of the bladder is regarded as a relatively indolent disease. However, its propensity for frequent recurrences constitutes a major clinical problem. Additionally, there is a progression risk of 10-15% to either a higher grade and/or a higher stage disease in these tumors. The molecular factors that will predict recurrence and progression in low-grade pTa bladder carcinoma have not yet been elucidated. Herein, we investigated the association of phosphatase and tensin homolog deleted on chromosome 10 (PTEN) alterations with recurrence and progression in NILGPUC using immunohistochemistry. Eighty-one cases of bladder cancer initially diagnosed as NILGPUC in a single institution with follow-up were encountered after searching medical records. Tissue microarrays (TMA) that contained both tumor and non-neoplastic mucosa from each case were constructed using paraffin blocks of transurethral resections. Sections from TMA blocks were stained immunohistochemically for PTEN protein and were evaluable in 76 cases. Any absence of staining was recorded and correlated with clinical findings. Ten patients (13.2%) showed progression and 41 (53.9%) showed recurrence. Reduced PTEN expression was observed in 29 cases (38.1%). Cases with reduced PTEN had higher progression rate compared to cases with intact PTEN (p = 0.026). Tumor relapse was more frequent in cases with reduced PTEN (65.5 vs 46.8%), but this difference was not statistically significant (p = 0.112). On the other hand, decreased PTEN expression was associated with higher number of recurrence episodes (p = 0.002). PTEN seems to have a link with the disease course in NILGPUC of the bladder.

The Influence of Inverted Growth Pattern on Recurrence for Patients with Non-Invasive Low Grade Papillary Urothelial Carcinoma of Bladder.

AIMS: To evaluate the impact of a histologically inverted pattern on recurrence in patients with newly diagnosed non-invasive, low-grade papillary urothelial carcinoma of the urinary bladder. METHODS: A total of 81 patients with primary bladder non-invasive, low-grade papillary urothelial carcinoma diagnosed in a single tertiary-care centre who had at least 1-year follow-up after an initial resection were included. All slides from each case were reviewed to determine the growth pattern (exophytic versus endophytic, i.e. inverted) and other histological parameters. Clinical data were retrieved from hospital records. RESULTS: Disease recurrence occurred in 41 (50.6%) patients. Cases with an inverted pattern showed a lower recurrence rate than those with pure exophytic tumours (37.5% versus 52.1%), a longer time to first recurrence (mean 34 versus 21.5 months) and fewer recurrence episodes (p=0.482, 0.564 and 0.051, respectively). All recurring inverted cases recurred only once during follow-up. No tumour with >80% inverted architecture recurred. CONCLUSION: Our results suggest that non-invasive, low-grade papillary urothelial carcinoma of the bladder tends to have a better outcome in terms of disease recurrence if it shows an inverted growth pattern. To indicate the presence and percentage of the inverted pattern in low-grade urothelial carcinomas in the pathology report might be considered as an adjunct to help long-term patient management.

Comparison of biochemical recurrence-free survival between periprostatic and pelvic lymph node metastases of prostate cancer.

OBJECTIVE: To assess the pathologic characteristics and prognostic significance of periprostatic lymph node (LN) metastasis of prostate cancer. The latter was performed by comparing biochemical recurrence (BCR)-free survival in cases of periprostatic LN metastasis versus matched patients showing pelvic LN metastasis. METHODS AND MATERIALS: We identified 15 patients who underwent radical prostatectomy in our institution (1984-2011) showing positive periprostatic and negative pelvic LN with available follow-up information (group 1). These patients were matched 1:2 to patients with positive pelvic LN (group 2) for pertinent clinicopathologic parameters. RESULTS: Main locations of positive periprostatic LN were posterior base and mid posterolateral. Overall higher rate of positive margins, smaller LN, and metastasis size were encountered in group 1 compared with group 2. At 5 years postprostatectomy, 69% of patients in group 1 were free of BCR, whereas 26% of those in group 2 remained BCR free, suggesting that patients with periprostatic node metastasis appeared to have a lower risk of BCR. However, the difference was not statistically significant (P = .072). The same was true when adjusted for the effect of prostate-specific antigen, surgical margin status, size of LNs, size of metastasis, age, and year of surgery. CONCLUSION: Patients with periprostatic node metastasis may have a lower risk of BCR compared with those with metastasis to pelvic LN. Future analysis of larger cohorts will help establish the biologic significance of prostate cancer metastasis to periprostatic LN.