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Punctate vasculitis skin lesions

MedGen UID:
479323
Concept ID:
C3277693
Finding
HPO: HP:0200030

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPunctate vasculitis skin lesions

Conditions with this feature

Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations
MedGen UID:
348124
Concept ID:
C1860518
Disease or Syndrome
Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a small-vessel disease that affects highly vascularized tissues including the retina, brain, liver, and kidneys. Age of onset is often between 35 and 50 years. The most common presenting finding is decreased visual acuity and/or visual field defects. Neurologic manifestations may include hemiparesis, facial weakness, aphasia, and hemianopsia. Migraines and seizures are less frequently described. Renal manifestations may include mild-to-moderate increase in serum creatinine and mild proteinuria; progression to end-stage renal disease (ESRD) is uncommon. Hepatic manifestations frequently include mildly elevated levels of alkaline phosphatase and gamma-glutamyltransferase (GGT). Less common findings include psychiatric disorders, hypertension, mild-to-moderate anemia, and Raynaud phenomenon.

Recent clinical studies

Therapy

Chen JH, Wang KH, Hu CH, Chiu JS
Yonsei Med J 2008 Jun 30;49(3):509-13. doi: 10.3349/ymj.2008.49.3.509. PMID: 18581605Free PMC Article
Geller M
Ann Allergy 1989 Jul;63(1):29-30. PMID: 2662834
Farnam J, Grant JA, Lett-Brown MA, Lord RA, Russell WL, Henry DP
J Allergy Clin Immunol 1986 Aug;78(2):353-7. doi: 10.1016/s0091-6749(86)80089-6. PMID: 3734288
Davis RS, Remigio LK, Schocket AL, Bock SA
J Allergy Clin Immunol 1981 Dec;68(6):479-83. doi: 10.1016/0091-6749(81)90202-5. PMID: 7310013
Sigler RW, Levinson AI, Evans R 3rd, Horakova Z, Kaplan AP
J Allergy Clin Immunol 1979 Jan;63(1):35-8. doi: 10.1016/0091-6749(79)90159-3. PMID: 82566

Prognosis

Farnam J, Grant JA, Lett-Brown MA, Lord RA, Russell WL, Henry DP
J Allergy Clin Immunol 1986 Aug;78(2):353-7. doi: 10.1016/s0091-6749(86)80089-6. PMID: 3734288
Davis RS, Remigio LK, Schocket AL, Bock SA
J Allergy Clin Immunol 1981 Dec;68(6):479-83. doi: 10.1016/0091-6749(81)90202-5. PMID: 7310013
Sigler RW, Levinson AI, Evans R 3rd, Horakova Z, Kaplan AP
J Allergy Clin Immunol 1979 Jan;63(1):35-8. doi: 10.1016/0091-6749(79)90159-3. PMID: 82566

Clinical prediction guides

Monteagudo-Sánchez B, Luiña-Méndez L, Mosquera-Fernández A
Acta Dermatovenerol Croat 2019 Jun;27(2):129-130. PMID: 31351511
Cappell MS, Gupta AM
Am J Gastroenterol 1990 Sep;85(9):1186-8. PMID: 2389731
Sigler RW, Levinson AI, Evans R 3rd, Horakova Z, Kaplan AP
J Allergy Clin Immunol 1979 Jan;63(1):35-8. doi: 10.1016/0091-6749(79)90159-3. PMID: 82566

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