Rajaguru Paramaguru

Dermatitis Herpetiformis as the Initial Presentation of Primary Biliary Cholangitis in a Male with Gluten Sensitivity

Celiac disease is commonly associated with elevated liver enzymes that normalize on a gluten-free diet. Celiac disease is rarely described in patients with primary biliary cholangitis. Dermatitis herpetiformis is the skin manifestation of the celiac disease that is very rarely associated with primary biliary cirrhosis. We present the case of a 62-year-old man who presented with severe chronic pruritus, in whom a diagnosis of primary biliary cholangitis was made initially. However, in the presence of atypical skin lesions, not confirming to chronic cholestasis, an in-depth evaluation including histopathological examination led to the diagnosis of dermatitis herpetiformis associated with gluten sensitivity. Gluten-free diet and medical treatment with dapsone led to beneficial clinical outcomes.

Severe Alcoholic Hepatitis in a Teetotaler

To the Editor: Severe alcoholic hepatitis (AH) is a serious form of alcoholic liver disease associated with rapid onset of jaundice and tender hepatomegaly within 60 days of heavy alcohol consumption (>50 g/day) for minimum of 6 months and serum bilirubin >3 mg/ dL, an elevated aspartate transaminase (AST), an AST: alanine transaminase (ALT) ratio > 1.5, in the absence of other obvious causes of hepatitis and classical histologic features [1]. We describe similar findings in a patient, in the absence of alcohol use. A 44-year-old teetotaler male, pineapple farmer by occupation presented with classical clinical and biochemical features of severe alcoholic hepatitis associated with pruritus for 3 days (Table 1a). He denied over the counter medication use, was not overweight, lacked stigmata of chronic liver disease and portal hypertension, and did not suffer from metabolic syndrome. Blood investigations for acute and chronic viral hepatitis including herpes simplex and zoster, cytomegalovirus, parvovirus, human immunodeficiency virus, human T-cell lymphotropic virus and Table 1 a: Blood investigations of the patient at admission, at discharge and on follow up; b: chemical and toxicology analysis of pineapple samples farmed by the patient and consumed on a daily basis prior to development of symptoms

Reply to: Safety in Ayurveda—Need to bring the house in order by Dr. Sanjeev Rastogi

Sir, We thank Dr. Rastogi for his interest in our recently published study in the Journal [1]. We agree to support his initial opinions in the letter that truly reflects and echoes our study findings. However, we disagree with his statements on causality and subsequent conclusions. For a clear understanding of drug-induced liver injury (DILI), one must be able to fathom the complexities associated with this condition that could clinically mimic almost all other liver disorders [2]. With this in mind, the Roussel Uclaf Causality Assessment Method (RUCAM) and Maria and Victorino systems help to facilitate the causality attribution in suspected DILI. Even though both perform reasonably well in comparison with the Bgold standard^ of expert consensus opinion, the RUCAM is most widely used. Recently, the updated RUCAM was recommended for use in clinical, regulatory, publication, and expert purposes to validly establish causality in cases of suspected herbal-induced liver injury, facilitating an internationally accepted approach to causality assessment [3]. As per RUCAM, DILI scores are often grouped into likelihood levels of excluded (score ≤ 0), unlikely (1–2), possible (3–5), probable (6–8), and definite or highly probable (> 8). Confirmation of DILI requires the gold standard of drug Brechallenge^ which a patient/family member may not consent to and which is currently frowned upon [4]. In our study, we do not consider this a lacuna, but an interest in patient safety, which is why Bdefinite^ DILI was not ascertained. However, significant scores, along with strong clinical suspicion and meticulous investigations including liver biopsy findings (established and novel patterns) that excluded classical causes of liver injury strongly supported Ayurvedic herbal medicines (AHMs) as the potential cause of DILI in our patients. Hence, our results clearly established causality and justify our conclusions on AHM-related DILI in contrast to what Dr. Rastogi opines. Dr. Rastogi’s confidence in concluding the role of Ayurvedic formulations in themanagement of hepatic encephalopathy and metastatic liver disease is surprising. Both the self-quoted references are case reports—a form of publication with the lowest quality in evidence-based medicine and hence such strong conclusions on the effectiveness of Ayurvedic formulations cannot be made [5]. Both case reports are meddled with confounding factors such as continuation of conventional (or modern) medical management along with introduction of Ayurvedic formulations that defeats the purpose of understanding the true effect of AHM, while the follow up period of supposed Ayurveda management of metastatic liver disease was only ten days which is far from the ideal clinical endpoint in such condition. This aspect in contrivingmediocre conclusions can be considered an enlightenment with regard to the paucity of ideal research methodology in Ayurveda practice: a serious concern which officials of the AYUSH concur with [6]. Dr. Rastogi further describes the effectiveness of Ayurvedic medications in a multitude of liver disorders but does not quote any reference to support his statements. Ayurveda and herbal medicines formed the majority of drugs retrieved from our cohort of patients suspected to have severe liver injury and hence it was only logical to include this aspect in the title while our results and discussions have shed light on every aspect of the retrieved drugs. Ayurveda treatment focuses on symptoms rather than disease or syndromes, because of which multiple AHM use was noted in each patient. Hence, identifying a Bclinical condition^ for AHM use was impossible and a simpler representation of predominant symptoms for * Cyriac Abby Philips abbyphilips@gmail.com

Ayurveda metallic-mineral ‘Bhasma’-associated severe liver injury

Ayurveda Bhasma is a metallic-mineral preparation homogenised with herbal juices or decoctions and modified with heat treatment to apparently detoxify the heavy metals. It is widely recommended for the treatment of many disease conditions by practitioners of complementary and alternative medicine in the absence of good quality clinical trial evidence on its safety and efficacy. Heavy metal-induced liver injury is widely reported in the literature, and heavy metal adulteration of non-Bhasma-related Ayurveda and herbal products has been well described. We report a patient who developed severe liver injury requiring listing for liver transplantation for improved survival, after consumption of Bhasma for dyspepsia. This case describes the first documented case and toxicology analysis of Ayurveda Bhasma associated with severe drug-induced liver injury. Physicians must be alert regarding patient’s use of supposedly safe Ayurveda Bhasma that may promote acute severe liver injury in the absence of other known aetiologies.

Strange case of dimorphic skin rash in a patient with cirrhosis: atypical herpes simplex and sweet’s syndrome

A middle-aged man with decompensated cirrhosis and a dimorphic multisite skin rash is diagnosed with rare atypical herpes simplex infection, manifesting Sweet’s syndrome (SS) in the absence of other described associations. SS, an acute febrile neutrophilic dermatosis, has three common forms—classical or idiopathic, malignancy associated and drug induced. Systemic autoimmune, connective tissue diseases and infections are also strong associations. The latter is commonly described in Gram-positive bacteria, salmonellosis and Yersinia. Herpes infections are a rare cause of SS, reported only thrice in literature, one with concomitant lupus, the second with associated mycobacterial infection and third, in metastatic breast disease in contrast to our patient. Atypical rash, especially if dimorphic, warrants histopathological evaluation to confirm underlying disease.

Pseudo Symmer’s Pipe-Stem Fibrosis in Idiopathic Noncirrhotic Portal Hypertension Associated with POEMS Syndrome

Department of Hepatology and Transplant Medicine, PVS Institute of Digestive Diseases, PVS Memorial Hospital, Cochin, Kerala, India Department of Rheumatology, Care Clinics, Sree Sudheendra Medical Mission Hospital, Cochin, Kerala, India Department of Pathology, PVS Memorial Hospital, Cochin, Kerala, India Department of Pathology, Lakeshore Hospital and Research Centre Ltd, Cochin, Kerala, India Department of Gastroenterology, PVS Institute of Digestive Diseases, PVS Memorial Hospital, Cochin, Kerala, India