Pancreatitis, panniculitis and polyarthritis (PPP) syndrome.

A young male presented with intermittent high-grade fever, asymmetric polyarthritis and erythematous, tender nodules over left shin for 2 months duration. He had a history of alcohol dependence with multiple episodes of acute pancreatitis. With polyarthritis progressing relentlessly, unresponsive to non-steroidal anti-inflammatory drugs and steroids, a provisional diagnosis of sarcoidosis was considered. Indeed, he was treated with azathioprine and rituximab with no effect. Biopsy of the skin nodule revealed subcutaneous fat necrosis, foam cells, deposition of eosinophilic amorphous material and calcification. Synovial fluid aspiration from the arthritic knee obtained purulent but surprisingly culture-negative material, rich in triglycerides. Abdominal CT confirmed chronic pancreatitis. Final diagnosis of pancreatitis, panniculitis and polyarthritis (PPP) syndrome was made. He underwent surgical pancreatic ductal drainage leading to complete remission of symptoms. PPP syndrome triad occurs due to leakage of pancreatic enzymes into systemic circulation and subsequent fat necrosis. Surgical drainage of pancreatic duct is often curative.

Correlation of desmoglein 1 and 3 immunohistochemistry with autoantibody levels and clinical severity in pemphigus.

BACKGROUND: Pemphigus is a chronic potentially fatal autoimmune bullous disorder. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the two common subtypes. PV is the most common and aggressive type characterized by oral mucosal erosions and cutaneous lesions. PF presents with blisters on the scalp, face, and upper trunk, and spares the mucosae. Direct immunofluorescence (DIF) is the gold standard for diagnosis. Immunohistochemistry (IHC) is an emerging alternate diagnostic tool. In this study, our objectives were to identify the staining patterns of desmoglein 1 (dsg 1) and desmoglein 3 (dsg 3) IHC and to correlate the same with autoantibody levels and clinical severity in patients with PV and PF. METHODS: Forty-nine clinically, histologically, and DIF-confirmed cases of pemphigus were included in the study. The IHC patterns were scored from 0 to 3+ with 3+ dsg 1 IHC exhibiting intense membranous staining in the upper layers of the epidermis and 3+ dsg 3 IHC showing intense basal layer staining. Enzyme-linked immunosorbent assay (ELISA) for anti-dsg 1 and 3 antibodies was performed in 38 cases where serum samples were available. The pemphigus disease activity index system was utilized for clinical scoring. RESULTS: A 0 to 1+ score was observed for dsg 1 IHC in 100% of PF cases. A score of 0 to 1+ was observed for dsg 3 IHC in 97.3% of PV cases. One hundred percent of cases with PF and 83.9% of patients with PV tested positive for ELISA anti-dsg 1 and 3 antibody titers, respectively. Anti-dsg 1 and 3 ELISA titers significantly correlated with the dsg 1 and dsg 3 IHC scores. The mucosal scores showed a significant association with both dsg 1 and 3 IHC (p < 0.001). The cutaneous scores showed a significant association with the dsg 3 IHC (p < 0.001). CONCLUSION: The IHC patterns for dsg 1 and 3 proved reliable in giving concordant results with the ELISA antibody titers and clinical severity.

Ribociclib-induced extensive vitiligo-like lesions: possible pathomechanisms with clinical, dermoscopic and histological correlation.

Cyclin dependent kinase (CDK) 4/6 inhibitors are targeted agents which act on cyclin-D and these combined with hormonal therapy have been approved for the treatment of locally advanced or metastatic breast cancer. CDK 4/6 inhibitors have been found to have a tolerable adverse event profile; however, they have been associated with various dermatological adverse events. We report a case of ribociclib-induced vitiligo and discuss the clinical, dermoscopic and histological features with a review of the various possible pathomechanisms involved.

Recurrent extramedullary plasmacytomas without multiple myeloma: A case report with review of the literature.

BACKGROUND: Extramedullary plasmacytoma (EMP) is an uncommon presentation and usually occurs in conjunction with multiple myeloma (MM). An EMP without developing MM at any point is an extremely rare presentation, and only seven such cases have been reported in the literature to date. PRESENTATION OF CASE: We present a case of EMP, who presented with multiple recurrent lesions in rare sites like nasal cavity, testis and skin without the involvement of bone marrow at any point of disease course. He was treated with multiagent chemotherapy (DT-PACE) and continues to be in remission at 29 months post-chemotherapy, which is the longest amongst all the cases reported so far. DISCUSSION AND CONCLUSIONS: There are no clearly defined guidelines to treat EMP. Our case had a clinical presentation at very unusual sites and was treated with DT-PACE regimen as against the previous seven reported cases and had the most prolonged period of remission.

Dipeptidyl peptidase-4 inhibitor-associated cutaneous eruptions: a retrospective observational study.

BACKGROUND: Dipeptidyl peptidase (DPP)-4 plays a complex role in immune regulation and its inhibition can have effects on the pathogenesis of various skin diseases. Studies have shown that DPP-4 inhibitors are associated with an increased risk of bullous pemphigoid (BP). AIM: To analyse the clinical and histopathological features of cutaneous adverse events in patients on DPP-4 inhibitors. METHODS: We performed a retrospective review of patients with suspected DPP-4 inhibitor-associated cutaneous adverse events, at a tertiary teaching hospital from 1 January 2017 to 31 December 2020. Exclusion criteria included previous history of chronic skin disease and lack of histopathological reports or follow-up records. The clinical characteristics, latency period, Naranjo Adverse Drug Reaction Probability Scale and clinical outcomes were evaluated. RESULTS: In total, 18 patients (10 men, 8 women; mean age 68.6 years, range 38-89 years) were included. The DPP-4 inhibitors used were teneligliptin (n = 6), vildagliptin (n = 6), sitagliptin (n = 4), linagliptin (n = 1) and saxagliptin (n = 1). The mean interval between therapy initiation and lesion onset was 8.8 months (range 1-24 months). The dermatoses noted were BP (n = 12; 66.6%), lichenoid dermatitis (n = 4; 22.2%), psoriasiform dermatitis (n = 1; 5.6%) and spongiotic dermatitis (n = 1; 5.6%). Eight patients (44.4%) had necrotic keratinocytes as one of the distinct histological features. Causality assessment using the Naranjo scale rated the causative role of DPP-4 inhibitors as 'possible' in all patients. Of the 18 patients, 11 (61.1%) noted improvement in their condition following discontinuation of DPP-4 inhibitors, with 5 having complete remission within 6 months of stopping the drug. CONCLUSION: DPP-4 inhibitor-associated dermatoses are not necessarily limited to BP. It is necessary to recognize the possibility of other dermatoses in patients on DPP-4 inhibitors as drug substitution/cessation may improve disease morbidity.

Generalized granuloma annulare resolution following biopsy: a remote reverse Koebner phenomenon.

This generalized granuloma annulare case of palisading type, with extensive lesions of 10 months duration resolving 1 week post biopsy without any treatment is the most exceptional depiction of this poorly understood remote reverse Koebner phenomenon.

Bilious mask: parasite masquerading as malignant gall bladder polyp.

A 31-year-old woman, presenting with right upper quadrant pain, was suspected to have malignant gall bladder polyp based on MRI and CT scan findings. She underwent radical cholecystectomy with excision of bile duct and hepaticojejunostomy. Surprisingly, histology revealed parasitic remnants within the bile duct wall with no evidence of malignancy. A year later, this young woman suffers significant impairment of quality of life, perhaps an aftermath to the radicality of the surgery. Our case demonstrates a masquerade of 'malignant biliary polyp' by a parasite. This raises the need for extensive investigations such as endoscopic ultrasound guided biopsies, parasitic serology and positron emission tomography (PET) scans when patients present with probable malignant biliary lesions, even in difficult-to-access areas. A preoperative diagnosis might have averted an unnecessary major surgery in this young woman.

Correlation between insulin resistance and liver histology in patients with nonalcoholic steatohepatitis with and without obesity.

INTRODUCTION: Insulin resistance (IR) plays a central role in pathogenesis of nonalcoholic steatohepatitis (NASH). The aim of this study was to correlate histopathological grading and IR in overweight/obese patients with NASH as compared with lean NASH. METHODS: Patients with NASH who underwent liver biopsy between January 2012 and December 2012 were included. Anthropometric, clinical, and biochemical features, necro-inflammatory grades, and fibrosis stage on liver biopsies were scored according to Brunt and non-alcoholic fatty liver disease (NAFLD) activity score (NAS). RESULTS: Of 42 patients, 33 (78.6%) had body mass index (BMI) ≥ 23 kg/m2 (overweight/obese) while 9 had BMI < 23 kg/m2 (lean). Mean fasting blood sugar (FBS) and HbA1c levels in overweight/obese patients with NASH were higher than in lean NASH (p < 0.05). The median homeostatic model assessment-estimated insulin resistance (HOMA-IR) among NASH patients with BMI ≥ 23 kg/m2 was higher than among those with BMI < 23 kg/m2 (3.02 [0.34-17.22] vs. 2 [0.52-5.26]; p = 0.045). However, fasting insulin levels were comparable among lean and overweight/obese patients with NASH. Metabolic syndrome could be predicted with 75% sensitivity and 85.3% specificity at a HOMA-IR cutoff value of 3.9. No significant difference was observed with regard to HOMA-IR levels with Brunt grades, Brunt staging, Brunt grades 1 and 2, Brunt scores < 2 and > 2, and NAS scores, and NAS scores < 4 and > 4. CONCLUSIONS: Although IR was significantly higher in overweight/obese patients with NASH as compared with that in lean patients with NASH, there was no difference in the correlation of HOMA-IR with histology between these groups.

Blastomycosis Misdiagnosed as Tuberculosis, India.

Chronic pulmonary blastomycosis is often misdiagnosed and treated as tuberculosis in disease-endemic and non-disease-endemic areas. We report the case of a 32-year-old man who after visiting Chicago, Illinois, USA, returned to India and received treatment for tuberculosis for 12 months before receiving the correct diagnosis of blastomycosis.

A Case Series and Review of Bacillus Cereus Endocarditis from India.

INTRODUCTION: Bacillus cereus is a gram positive bacilli found commonly in the soil and environment. It is a bacteria rarely associated with endocarditis. CASE HISTORY: Intravenous drug abuse, presence of valvular defects, pacemakers, immunodeficiency are some of the known risk factors for B.cereus endocarditis. We present here a case series of two patients with B.cereus endocarditis along with a review of the literature. CONCLUSION: This is the first report of B.cereus endocarditis from India to the best of our knowledge.

First two bilateral hand transplantations in India (Part 4): Immediate post-operative care, immunosuppression protocol and monitoring.

INTRODUCTION: Being able to counter immune-mediated rejection has for decades been the single largest obstacle for the progress of vascular composite allotransplantation (VCA). The human immune system performs the key role of differentiating the 'self ' from the 'non-self '. This, although is quintessential to eliminate or resist infections, also resists the acceptance of an allograft which it promptly recognises as 'non-self'. MATERIALS AND METHODS: Pre-operative evaluation of the recipient evaluation included immunological assessment in the form of panel reactive antibodies (PRA), human leucocyte antigen (HLA) typing, donor-specific antibody detection assays (DSA) and complement-dependent cytotoxicity assays (CDC). Induction immunosuppression was by thymoglobulin and the maintenance by the standard triple-drug therapy. RESULTS: Both the recipients were managed by the standard triple drug therapy and have had only minor episodes of rejections thus far which have been managed appropriately. DISCUSSION: Induction immunosuppression was by thymoglobulin and the maintenance by the standard triple-drug therapy. Various groups have tried various other formulations and regimes as well. CONCLUSION: A comprehensive plan has to be drawn up for immunological screening, selection and the post-operative immunosuppressant usage. The ultimate goal of these immunosuppression modalities is to achieve a state of donor-specific tolerance.

Ofuji's disease in an immunocompetent patient successfully treated with dapsone.

Eosinophilic pustular folliculitis or Ofuji's disease is a non-infectious eosinophilic infiltration of hair follicles, which usually presents with itchy papules and pustules in a circinate configuration. We report this case of an immunocompetent patient with erythematous papules and plaques without macropustules diagnosed as eosinophilic pustular folliculitis-a rarely reported entity outside Japan. He was successfully treated with oral dapsone.

Chromoblastomycosis due to Fonsecaea pedrosoi: an old wine in a rare bottle.

Chromoblastomycosis is a chronic subcutaneous mycosis commonly caused by Fonsecaea, Phialophora, and Cladophialophora spp. Out of these, Fonsecaea pedrosoi is the most common etiological agent, implicated in 70%-90% of the cases reported worldwide. The histopathological diagnosis of chromoblastomycosis is based on visualization of medlar or sclerotic bodies in the tissue. These sclerotic bodies divide by planar division. Rarely, budding is seen in these sclerotic bodies. As this entity can be confused with phaeohyphomycosis, it is important to be aware of such a presentation also. We report two cases of chromoblastomycosis that showed budding sclerotic bodies.

Osteopetrosis: a rare cause of anemia.

Normocytic anaemia is caused either by hypoproliferation of haemopoietic tissue or increased destruction of red cells. Osteopetrosis is a rare cause of anaemia. Infantile osteopetrosis (also called malignant osteopetrosis) is diagnosed early in life. But it is the adult osteopetrosis (also called benign osteopetrosis) which is diagnosed in late adolescence or adulthood that present as anaemia which is difficult to diagnose and treat. Approximately one half of patients are asymptomatic, and the diagnosis is made incidentally, often in late adolescence because radiologic abnormalities start appearing only in childhood. In other patients, the diagnosis is based on family history. Still other patients might present with osteomyelitis or fractures. We are presenting here an unusual case of osteopetrosis which was referred to us for the evaluation of anaemia.