Generalized Amyloid Lichen Associated with Primary Biliary Cholangitis: A Case Report
DOI:
https://doi.org/10.52787/agl.v54i2.369Keywords:
Primary biliary cholangitis, liver cirrhosis, primary cutaneous amyloidosis, lichen amyloidosisAbstract
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.
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, and 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.
References
-1. Hamie L, Haddad I, Nasser N, Kurban M, Abbas O. Primary Localized Cutaneous Amyloidosis of Keratinocyte Origin: An Update with Emphasis on Atypical Clinical Variants. Am J Clin Dermatol. 2021;22(5):667-80.
-2. Kaltoft B, Schmidt G, Lauritzen AF, Gimsing P. Primary localized cutaneous amyloidosis-a systematic review. Dan Med J. 2013;60(11):A4727.
-3. Aung T, Noakes R, Murrell DF, Daniel BS, Kelati A. Primary cutaneous amyloidosis: A review of the available studies and gaps in data. Australas J Dermatol. 2023;64(2):e121-e124.
-4. Tursen U, Kaya TI, Dusmez D, Ikizoglu G. Case of generalized lichen amyloidosis. Int J Dermatol. 2003;42(8):649-51.
-5. Gulamhusein AF, Hirschfield GM. Primary biliary cholangitis: pathogenesis and therapeutic opportunities. Nat Rev Gastroenterol Hepatol. 2020;17(2):93-110.
-6. Joshita S, Umemura T, Tanaka E, Ota M. Genetic Contribution to the Pathogenesis of Primary Biliary Cholangitis. J Immunol Res. 2017;2017:3073504.
-7. Guillet C, Steinmann S, Maul JT, Kolm I. Primary Localized Cutaneous Amyloidosis: A Retrospective Study of an Uncommon Skin Disease in the Largest Tertiary Care Center in Switzerland. Dermatology. 2022;238(3):579-86.
-8. Breathnach SM. “Amyloid and amyloidosis”. J Am Acad Dermatol. 1988;18(1):1-16.
-9. Tanaka A, Arita K, Lai-Cheong JE, Palisson F, Hide M, McGrath JA. New insight into mechanisms of pruritus from molecular studies on familial primary localized cutaneous amyloidosis. Br J Dermatol. 2009;161(6):1217-24.
-10. Lin MW, Lee DD, Liu TT, Lin FY, Chen YS, Huang CC, et al. Novel IL31RA gene mutation and ancestral OSMR mutant allele in familial primary cutaneous amyloidosis. Eur J Hum Genet. 2010;18(1):26-32.
-11. Mehrotra K, Dewan R, Kumar JV, Dewan A. Primary Cutaneous Amyloidosis: A Clinical, Histopathological and Immunofluorescence Study. J Clin Diagn Res. 2017;11(8):WC01-WC05.
-12. Chuang YY, Lee DD, Lin CS, Chang JY, Tanaka M, Chang TY, et al. Characteristic dermoscopic features of primary cutaneous amyloidosis: a study of 35 cases. Br J Dermatol. 2012; 167(3):548-54.
-13. Carey EJ, Levy C. Primary Biliary Cholangitis: A New Era. Clin Liver Dis. 2018;22(3):xiii-xiv.
-14. Carey EJ, Ali AH, Lindor KD. Primary biliary cirrhosis. Lancet. 2015;386(10003):1565-75.
-15. Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019;69(1):394-419.
-16. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017;67(1):145-172.
-17. Tan D, Goodman ZD. Liver Biopsy in Primary Biliary Cholangitis: Indications and Interpretation. Clin Liver Dis. 2018;22 (3):579-88.
-18. Goel A, Kim WR. Natural History of Primary Biliary Cholangitis in the Ursodeoxycholic Acid Era: Role of Scoring Systems. Clin Liver Dis. 2018;22(3):563-78.
-19. Trivedi HD, Lizaola B, Tapper EB, Bonder A. Management of Pruritus in Primary Biliary Cholangitis: A Narrative Review. Am J Med. 2017;130(6):744.e1-744.e7.
-20. Prince MI, Ducker SJ, James OF. Case-control studies of risk factors for primary biliary cirrhosis in two United Kingdom populations. Gut. 2010;59(4):508-12.
-21. Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bersaga VN, Heathcote JE, et al. Primary biliary cirrhosis. Hepatology. 2009;50(1):291-308.
-22. Tafarel JR, Lemos LB, Oliveira PM, Lanzoni VP, Ferraz ML. Cutaneous amyloidosis associated with primary biliary cirrhosis. Eur J Gastroenterol Hepatol. 2007;19(7):603-5.
-23. González-Moreno EI, Cámara-Lemarroy CR, Borjas-Almaguer DO, Martínez-Cabriales AS, Paz-Delgadillo J, Gutiérrez-Udave R, et al. Cutaneous amyloidosis associated with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome. Ann Hepatol. 2015;14(3):416-9.
-24. Yan X, Jin J. Primary cutaneous amyloidosis associated with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome and Sjögren syndrome: A case report. Medicine (Baltimore). 2018;97(8):e0004.
Published
How to Cite
Issue
Section
License
Copyright (c) 2024 Edwin Jesús Pardo Escalera, Astrid Dzoara Fuentes Cruz, José Cruz Mendoza Torres
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
