Acta gastroenterologica Latinoamericana · 2024
[Generalized Amyloid Lichen Associated with Primary Biliary Cholangitis: A Case Report].
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Pardo Escalera Edwin Jesús, Fuentes Cruz Astrid Dzoara, Mendoza Torres José Cruz
Аннотация
INTRODUCTION: Amyloid lichen is the most common subtype of cutaneous amyloidosis and is characterized by the presence of papules and plaques associated with itching, located on the calves, anterior thighs, and rarely on the forearms. Primary biliary cholangitis is an autoimmune liver disease characterized by destructive lymphocytic cholangitis and the presence of specific antimitochondrial antibodies, which cause biliary injury, cholestasis and progressive liver fibrosis.
MATERIAL AND METHODS: Informed consent was requested from the patient for the publication of their case and accompanying photographs.
CASE PRESENTATION: A 39-year-old male patient with phototype IV came for evaluation due to coffee grounds vomitus on one occasion and an episode of melenic evacuation. During the physical examination, he highlighted the presence of disseminated hyperchromic papules. A cholestatic pattern was identified with hyperbilirubinemia at the expense of direct bilirubin, elevation of gamma-glutamyltransferase, elevation of alkaline phosphatase, with liver enzymes in normal values, in addition, no positive serology was identified for hepatitis A, B and C. Antimitochondrial antibodies were positive by immunofluorescence (1:320). With hemodynamic improvement, a skin biopsy was taken with the presence of laminar hyperkeratosis in the stratum corneum and epidermis with moderate irregular acanthosis, thickening of the interpapillary processes,and hyperpigmentation of the basal layer.
DISCUSSION: The association between localized primary cutaneous amyloidosis and primary biliary cholangitis has been documented rarely.
MATERIAL AND METHODS: Informed consent was requested from the patient for the publication of their case and accompanying photographs.
CASE PRESENTATION: A 39-year-old male patient with phototype IV came for evaluation due to coffee grounds vomitus on one occasion and an episode of melenic evacuation. During the physical examination, he highlighted the presence of disseminated hyperchromic papules. A cholestatic pattern was identified with hyperbilirubinemia at the expense of direct bilirubin, elevation of gamma-glutamyltransferase, elevation of alkaline phosphatase, with liver enzymes in normal values, in addition, no positive serology was identified for hepatitis A, B and C. Antimitochondrial antibodies were positive by immunofluorescence (1:320). With hemodynamic improvement, a skin biopsy was taken with the presence of laminar hyperkeratosis in the stratum corneum and epidermis with moderate irregular acanthosis, thickening of the interpapillary processes,and hyperpigmentation of the basal layer.
DISCUSSION: The association between localized primary cutaneous amyloidosis and primary biliary cholangitis has been documented rarely.