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Primary cutaneous amyloidosis associated with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome and Sjögren syndrome: A case report.

Abstract Primary cutaneous amyloidosis (PCA) is a localized skin disorder characterized by the abnormal deposition of amyloid in the extracellular matrix of the dermis. The association between PCA and other diseases, although rare, has been documented for various autoimmune diseases. PCA associated with autoimmune hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjögren syndrome (SS) has not been previously reported in the literature.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 29465536
OWN - NLM
STAT- MEDLINE
DCOM- 20180302
LR  - 20180302
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 97
IP  - 8
DP  - 2018 Feb
TI  - Primary cutaneous amyloidosis associated with autoimmune hepatitis-primary
      biliary cirrhosis overlap syndrome and Sjogren syndrome: A case report.
PG  - e0004
LID - 10.1097/MD.0000000000010004 [doi]
AB  - RATIONALE: Primary cutaneous amyloidosis (PCA) is a localized skin disorder
      characterized by the abnormal deposition of amyloid in the extracellular matrix
      of the dermis. The association between PCA and other diseases, although rare, has
      been documented for various autoimmune diseases. PCA associated with autoimmune
      hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjogren
      syndrome (SS) has not been previously reported in the literature. PATIENT
      CONCERNS: A 50-year-old woman presented with progressive abnormal liver enzyme
      levels and was referred to our department. DIAGNOSES: Due to the patient's
      symptoms, laboratory test results, radiographic findings, and pathologic results,
      she was diagnosed with PCA associated with AIH-PBC overlap syndrome and SS.
      INTERVENTIONS: She was subsequently treated with a combination of ursodeoxycholic
      acid (UDCA), prednisone, and azathioprine. OUTCOMES: While this treatment can
      achieve therapeutic success, it cannot prevent complications from cirrhosis. This
      patient remains alive but experienced an emergent gastrointestinal hemorrhage.
      LESSONS: While we acknowledge that this is a single case, these findings extend
      our knowledge of immunological diseases associated with PCA and suggest a common,
      immune-mediated pathogenic pathway between PCA, AIH-PBC overlap syndrome, and SS.
      After 12 years of follow up, clinical manifestations have developed, and these
      autoimmune diseases have progressed. The combination of UDCA, prednisone, and
      azathioprine can achieve therapeutic success but cannot prevent disease
      progression. Routine follow up for this patient is necessary to document disease 
      progression.
FAU - Yan, Xin
AU  - Yan X
AD  - Department of Hepatology, The First Hospital of Jilin University, Changchun,
      Jilin, China.
FAU - Jin, Jinglan
AU  - Jin J
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Anti-Inflammatory Agents)
RN  - 0 (Cholagogues and Choleretics)
RN  - 0 (Immunosuppressive Agents)
RN  - 724L30Y2QR (Ursodeoxycholic Acid)
RN  - MRK240IY2L (Azathioprine)
RN  - VB0R961HZT (Prednisone)
RN  - Amyloidosis, Primary Cutaneous
SB  - AIM
SB  - IM
MH  - Amyloidosis, Familial/drug therapy/*immunology
MH  - Anti-Inflammatory Agents/administration & dosage
MH  - Azathioprine/administration & dosage
MH  - Cholagogues and Choleretics/administration & dosage
MH  - Drug Therapy, Combination
MH  - Female
MH  - Hepatitis, Autoimmune/*complications/drug therapy/immunology
MH  - Humans
MH  - Immunosuppressive Agents/administration & dosage
MH  - Liver Cirrhosis, Biliary/*complications/drug therapy/immunology
MH  - Middle Aged
MH  - Prednisone/administration & dosage
MH  - Sjogren's Syndrome/*complications/drug therapy/immunology
MH  - Skin Diseases, Genetic/drug therapy/*immunology
MH  - Treatment Outcome
MH  - Undifferentiated Connective Tissue Diseases/*complications/drug
      therapy/immunology
MH  - Ursodeoxycholic Acid/administration & dosage
EDAT- 2018/02/22 06:00
MHDA- 2018/02/22 06:00
CRDT- 2018/02/22 06:00
PHST- 2018/02/22 06:00 [entrez]
PHST- 2018/02/22 06:00 [pubmed]
PHST- 2018/02/22 06:00 [medline]
AID - 10.1097/MD.0000000000010004 [doi]
AID - 00005792-201802230-00001 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2018 Feb;97(8):e0004. doi: 10.1097/MD.0000000000010004.