Case 1
A 63-year-old man presented recurrent blisters on his face, neck, hands and forearms for 3 month. He was previously diagnosed as pemphigoid but responded poorly to topical steroids. Associated symptoms included pruritus and increased skin fragility. He denied a personal or family history of liver disease, hepatitis and iron abnormalities. The patient had consumed alcohol for more than 10 years with a daily amount of 150-300 ml (alcohol by volume, ABV >40%) .
Physical examination revealed multiple tense blisters, superficial erosions with crusting and scars distributed on the face, neck, forearms, dorsum of the hands and feet (Figure 1A-C). His laboratory results were as follows: aspartate aminotransferase (AST) 30 U/L (0–50), alanine aminotransferase 55 U/L (0–50) , gamma-glutamyl transferase (GGT) 147 U/L (0–55), transferrin saturation (TS) 65%. Tests of hepatitis C, hepatitis B and HIV tests were negative. Wood’s lamp examination of his urine demonstrated coral-colored fluorescence (Figure 1D). Skin biopsy found a superficial ulcer, dilated capillaries of the dermal papillae, perivascular extravasation of red blood cells, infiltration of mixed inflammatory cells in the dermis and a negative immunofluorescence staining (Figure 1E). Periodic Acid-Schiff (PAS) staining showed deposition of purple-red material around the dermal vessel and the dermal-epidermal junction (Figure 1F).
The patient was diagnosed with PCT and treated with glycyrrhizin during hospitalization, and his skin lesions were greatly regressed. He was counseled on cessation of alcohol consumption and manipulation of photoprotection. No medication was prescribed after discharge from hospital. The patient was followed-up for 4 years and maintained complete remission.