PMC9637682_01
Skin manifestations of a 42-year-old male. (A) Diffused erosions covered by crusts on the lips. (B) The erythematous vesiculobullous rash had spread to the entire body, along with epidermal detachment.
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F1
Mucosal involvement with the presence of conjunctival and oral ulcers were also noted (Figure 1).
Evolution of serum liver tests and medications of a 42-year-old male after exposure to NSAIDs. Corticosteroids were given since the diagnosis of Stevens-Johnson Syndrome with titration according to the clinical presentation. The progressive elevation of liver enzymes and jaundice had led to a liver biopsy performed on day 6, along with the confirmed diagnosis of vanishing bile duct syndrome. Mycophenolate mofetil was prescribed after the diagnosis while the patient prepared for liver transplantation, which was not performed due to deterioration of clinical conditions. Intravenous immunoglobulin was administered for 3 days after the bone marrow biopsy revealed hemophagocytic lymphohistiocytosis. The liver enzymes and bilirubin levels remained relatively stable until day 125, when significant elevation was observed. Repeated liver biopsy was performed on day 127, which showed vanishing bile duct syndrome in resolution. T-Bil, total bilirubin level; D-Bil, direct bilirubin level; GGT, gamma glutamyl transferase; ALT, alanine aminotransferase; AST, aspartate aminotransferase; ALP, alkaline phosphatase; IVIG, intravenous immunoglobulin.
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F2
Further laboratory examination revealed progressively elevating liver function enzymes and jaundice on day 6 of hospitalization (AST/ALT: 581/1488 U/L; ALP/GGT: 667/1990 U/L; T-Bil/D-Bil: 19.81/15.35 mg/dl) (Figure 2).
Percutaneous liver biopsies. (A) Cholestatic hepatitis with marked perivenular cholestasis and ballooning of hepatocytes seen under high-power field hematoxylin and eosin (H&E) staining. (B) Initial liver biopsy showing 10 portal tracts, with only three surviving bile ducts that show degeneration under H&E staining. (C) Second liver biopsy showing an increase in the bile duct ratio under H&E staining.
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F3
In a total of 10 portal tracts, only three bile ducts survived, which showed degeneration and cell senescence changes (i.e., increased nuclear to cytoplasm ratio, uneven nuclear spacing, drop-out, and syncytia formation; Figures 3A,B). An increase in the bile duct ratio was seen compared to that of the previous study (Figure 3C).