Clinical Spectrum of Henoch Schonlein Purpura in Adults: A Hospital Based Study.

BACKGROUND AND OBJECTIVES: Henoch Schonlein purpura (HSP) is a small vessel vasculitic disorder common in children and has been extensively studied. Although it is known to also occur in adults there is relative paucity of data as regards to its clinical spectrum, complications and outcome, particularly in Indian context. Hence the study was undertaken with the objective to evaluate the various skin manifestations, systemic complications of HSP in adults and also compare it with data available in children in various published clinical studies.Study design, materials, methods : In this retrospective, observational, hospital-based cohort study conducted at Goa Medical College the premier teaching institute from Goa, clinical data of adult patients (>18 years age) fulfilling the diagnostic criteria as per European League Against Rheumatism (EULAR) 2010 criteria for HSP was obtained, over period of 6 years. All the clinical manifestations, complications, investigations, outcomes were recorded. Skin biopsy histopathology and immunofluorescent test findings were also obtained. The data was analysed and results were compared to the data available in pediatric studies to ascertain the similarities and differences. RESULTS: In our study cohort of 30 patients, we found a higher incidence of atypical and more extensive skin lesions particularly bullae (40%), necrotic ulcers (53.3%), urticarial wheals (53%) unlike in children as well as differences in distribution especially sparing of buttocks in adults. The incidence of gastrointestinal involvement was 80% which was higher than that reported by other studies in adults (35% to 70%). A significant 40% of patients had upper GI bleeding with endoscopy revealing small hemorrhages in gastric mucosa. Lower GIT bleed was seen in 8 patients. Renal involvement (microscopic hematuria, overt glomerulonephritis, nephrotic syndrome) was seen in 65% patients which was higher than that reported in children (43 %). Skin biopsy immunofluorescence was found to be positive in almost 66 % cases confirming IgA deposition which is the hallmark pathological finding. CONCLUSIONS: HSP, though less common in adults than children, presents with atypical and more severe cutaneous manifestations like bullae, necrotic ulcers, urticarial wheals. Systemic involvement appears to be more frequent and causing more morbidity and mortality as compared to the data in children mentioned in standard literature and most of the patients required steroid therapy for treatment unlike in children where majority of these cases are self-limiting. Skin involvement does not necessarily mirror gastrointestinal involvement in terms of severity and temporal occurrence.

Molecular profiling of oral squamous cell carcinoma associated with oral submucous fibrosis.

Context: Areca nut, a causative factor for oral submucous fibrosis (OSMF), is identified as a Group 1 human carcinogen. Oral squamous cell carcinoma (OSCC) associated with OSMF is now one of the most common malignancies in South and Southeast Asian countries. Aim: The present study was aimed to have clarity whether OSCC associated with OSMF is a pathologically different disease having different prognosis. Settings and Design: The difference between OSCC associated with OSMF and OSCC not associated with OSMF was studied in relation to expression of molecular markers, Ki-67, a proliferative and prognostic marker for OSCC and matrixmetalloproteinase-9 (MMP-9), and alpha smooth muscle actin (α-SMA), markers for invasiveness. Subjects and Methods: Expression was analyzed immunohistochemically using paraffin-embedded tissues from ten normal oral mucosa (Group I), thirty OSCC associated with OSMF (Group II), and thirty OSCC not associated with OSMF (Group III). Results: Group II showed OSCC occurring at younger age with more cases of well-differentiated OSCC. It also showed lower expression of Ki-67, MMP-9, and α-SMA as compared to Group III, and the difference was statistically significant. In addition, statistically significant low expression of markers was found in well and moderate grades of Group II as compared to those of Group III. Conclusion: OSCC associated with OSMF may have better prognosis and survival rate as it is found to occur at younger age with better grade of tumor differentiation and less expression of molecular markers Ki-67, MMP-9 and α-SMA. Thus, OSCC associated with OSMF can be considered a different disease pathologically and biologically. In-depth analysis of this molecular profiling can help to establish diagnostic, prognostic and therapeutic modalities for this unique malignancy.

Spindle Cell Carcinoma of the Mandibular Gingiva - A Case Report.

Spindle cell carcinoma is a malignancy of epithelial origin often mimicking its mesenchymal counterpart thus posing a diagnostic challenge. It is a rare biphasic malignant tumour mostly encountered in the upper aerodigestive tract. The chief differential diagnoses of spindle cell carcinoma are true superficial sarcomas and they especially need to be differentiated from fibrosarcoma. This presentation reports a spindle cell carcinoma of the gingiva and highlights the difficulties encountered in the diagnosis. It also emphasizes the importance of accurate and thorough diagnosis of malignant spindle cell lesions to determine the appropriate therapeutic modality.

Eosinophilic Pustular Folliculitis Post Chemotherapy in a Patient of Non-Hogkins Lymphoma: A Case Report.

Eosinophilic pustular folliculitis (EPF) was originally described by Ofuji in Japanese patients without any systemic disease. Later it was widely associated with HIV. Lately a large number of hematological malignancies have been associated with EPF. We hereby report an association of non-Hogkins lymphoma with EPF, probably the first in Indian context.