Weber christian disease?

CLINICAL FEATURES OF SYSTEMIC LUPUS ERYTHEMATOSUS: SLE is a chronic inflammatory disease which occurs when the body's immune system attacks its own tissues and organs. The body's immune system becomes hyperactive and attacks normal ,healthy tissue .Lupus can attack every organ in the body. Clinical features are described as follows. 1.Mucocutaneous features. 2.Nervous system features. 3.Ophthalmic features. 4.Cardiovascular features. 5.Pulmonary features. 6.Gastrointestinal tract&liver features. 7.Renal features. 8.Lymphadenopathy and spleenomegaly. 9.Hematological features. 10.Musculoskeletal features. MUCOCUTANEOUS FEATURES: Mucocutaneous features of SLE are classified as Acute,Subacute and Chronic lesions. ACUTE : MALAR RASH/BUTTERFLY RASH. The classic butterfly rash presents acutely as an erythematous,elevated lesion in a malar distribution over the bridge of the nose and cheeks ,commonly precipitated by exposure to sunlight.This rash is transient and heals without scarring. SUBACUTE RASH /SUBACUTE CUTANEOUS LUPUS ERYTHEMATOSUS(SCLE) SCLE lesions begin as small , erythematous, slightly scaly papules that evolve into either a psoriasiform /papulosquamous form or annular form which coalesce to form polycyclic or figurative patterns. These subacute lesions typically are symmetric widespread,superficial and non-scarring lesions.Neck,shoulders,upper chest,upper back. and extensor surface of hand are commonly involved.SCLE has been associated with the presence of anti-Ro/SS-A antibodies, genetic deficiencies of complement C2&C4. certain medications like hydrochlorothiazide. CHRONIC RASHES /DISCOID LUPUS ERYTHEMATOSUS(DLE). DLE lesions are characterised by discrete, erythematous,slightly infiltrated plaques covered by a well formed adherant scale that extends into dilated hair follicles (follicular plugging).They are mostly seen on the face,neck,scalp and ears.The characteristic pattern of slow expansion with active inflammation at the periphery which heal , leaving behind depressed central scars, atrophy,telangiectasias and dyspigmentation (hypo or hyper pigmentation) LUPUS PROFOUNDUS These are firm,nodular lesions which are painful and consists of peri-vascular infiltrates of mononuclear cells plus panniculitis .They usually appear on the scalp,face,arms ,chest back,thighs and buttocks. LUPUS TUMIDUS Characterised by photo distributed lesions with chronic pink,indurated plaques that are slow to heal. ALOPECIA. Alopecia occurs in SLE patients.It involves scalp,eyebrows,eyelashes,beard and body hair.Lupus hair is characterised by thin hair that easily fractures. PHOTOSENSITIVITY. Photosensitivity is defined as the development of rash after exposure to ultraviolet light coming from sunlight or florescent lights. MUCOUS MEMBRANES. The most common manifestations include irregularly shaped,raised white plaques,areas of erythema,silvery white scarred lesions and ulcers with surrounding erythema on the soft or hard palate or buccal mucosa.Oral ulcers in SLE are usually painless.

A 24-year-old woman with a 10-year history of intermittent episodes of redness and photophobia in both eyes presented to the ophthalmology clinic. On examination, the visual acuity was 20/30 in the right eye and 20/25 in the left eye. Slit-lamp examination revealed conjunctival hyperemia and peripheral corneal opacification, with inflammation and crystalline deposits on the corneal stroma consistent with interstitial keratitis. Six months later, the patient reported having vertigo, tinnitus, and hearing loss. What is the diagnosis? A) Syphilis B) Systemic lupus erythematosus C) High lead exposure D) Cogan’s syndrome E) Lymphoma

Good Morning! A 9 year old male presented with pain abdomen on and off since last 2 years. No other symptoms present. USG report has been attached. ESR 7, SGOT 34, SGPT 45, S. Bilirubin 0.69, HBSAg Negative, Urine C/E NAD. Please suggest diagnosis and management. Thank you!

A male child presented with the history of hyperpigmented patches on the buttocks and leg for the past 2 days . The parents gave the history of fever with chills and rigor for the past 7 days for which he was admitted in a nursing home and has now recovered. He has not brought the previous case records. There is a history of joint pain in the child which has not fully recovered. On examination multiple irregular hyperpigmented patches with surrounding erythema present. They are tender on palpation.Cutaneous lymph nodes ( inguinal) are palpable and tender. The patient refuses investigation. Please give your opinion

GASTROINTESTINAL MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS: Gastrointestinal complications are common in SLE. ORAL CAVITY : Mucous membrane involvement occurs in 12-45 %of patients with SLE.Oral or nasopharyngeal ulceration are usually painless and is one of the eleven diagnostic criteria of SLE.The ulcers are discoid or ulcerative DISCOID lesions are painful,characterized by erythema,atrophy and depigmentation and usually occurs on lips. ULCERS are punched out erosions with surrounding erythema,irregularly shaped raised white plaques tend to occur on hard or soft palate and buccal mucosa.These are usually painless. TREATMENT of oral lesions: *Topical glucocorticoids. *Tacrolimus ointment. *Intralesional corticosteroids. *Systemic antimalarial drugs. OESOPHAGUS : DYSPHAGIA,DYSMOTILITY,VASCULITIS, ISCHEMIA,ULCERATION,PERFORATION. DYSPHAGIA:Dysphagia occurs due to SLE or to complications associated with therapy such as pill oesophagitis and oral candidiasis DYSMOTILITY :Hypotensive LES(lower esophageal sphincter) and aperistalysis are documented by manometry. VASCULITIS-ISCHEMIA-ULCERATION- PERFORATION :Ischemia from vasculitis can lead to esophageal ulceration and perforation. TREATMENT : *Hypomotility is treated with metaclopramide. *GERD symptoms are treated with routine GERD precautions and proton pump inhibitors. *Immunomodulatory agents are used for treating SLE,but they can cause opportunistic oesophagitis from candidiasis,cytomegalovirus and herpes simplex virus. STOMACH &DUODENUM : DYSPEPSIA,PEPTIC ULCER DISEASE AND GASTRIC ANTRAL VASCULAR ECTASIA (GAVE) DYSPEPSIA is due to treatment with NSAID's or corticosteroids. PEPTIC ULCER DISEASE is also due to NSAID's and corticosteroids.Therefore prophylactic therapy for peptic ulcer is beneficial in SLE patients with dyspepsia,history of peptic ulcer disease,and on therapy with NSAID's and corticosteroids. GAVE :GAVE is an erosive type of atrophic gastritis characterized by marked veno-capillary ectasia.This causes chronic gastro- intestinal bleeding or irondeficiencyanemia. Dilated small blood vessels cause streaky long red areas that resemble the markings on watermelon when viewed endoscopically SO CALLED AS WATERMELON STOMACH. SMALL &LARGE BOWEL : ABDOMINAL PAIN,GASTROINTESTINAL BLEEDING,DYSMOTILITY,DIARRHEA,PROTEIN LOOSING ENTEROPATHY ,PNEUMATOSIS CYSTOIDES INTESTINALIS ,RECTAL ULCERS, STEATORRHOEA,PANCREATITIS, ACALCULUS CHOLECYSTITIS : ABDOMINAL PAIN : can be caused by mesenteric vasculitis and infarction,acalculous cholecystitis,peptic ulcer disease and pancreatitis. IINTESTINAL VASCULITIS. causes transmural inflammation with fibrinoid necrosis.It is most commonly found in terminal ileum and cecum where the blood supply is from superior mesenteric artery.Endoscopically,the bowel appears thickened and friable with ulceration and submucosal hemorrhages. COLON focal /diffuse ulcers,ischemic colitis or colonic diverticula. PROTEIN LOOSING ENTEROPATHY (PLE) This is characterized by significant hypo- albuminenia and diarrhea without proteinuria These are a result of villous atrophy with inflammatory. infiltrates and submucosal edema.Disease activity is measured by monitoring fecal excretion of radiolabelled albumin. PNEUMATOSIS CYSTOIDES INTESTINALIS PCI is gas filled cysts in the submucosa or subserosa of bowel wall.This radiological sign is suggestive of necrotising enterocolitis STEATORRHEA :occurs secondary to villus blunting and immune complex deposition.