Case Presentation: 76-year-old Hispanic male, presented with two weeks of progressing rash, worsening AMS and new symptoms of fever and severe extremities pain. Previously treated for allergy, UTI, and Pneumonia without improvement. His past medical history included Hypertension, DM-2, and ischemic stroke. On examination he was disoriented and unable to answer questions. A disseminated non palpable, non-blanching petechial rash was noted on the upper and lower extremities, including the palms and soles. Initial workup revealed WBC 17.38, platelets 517, sodium of 148, creatinine 1.9, EGFR 3, hematuria and proteinuria. CSF ruled out initially suspected meningococcal meningitis. Brain MRI showed increased T2/FLAIR signal in the periventricular and subcortical white matter, acute to subacute infarct throughout the left-brain hemisphere. CT chest showed small pleural effusions, peripheral septal thickening and architectural distortion suggesting background pneumonitis/interstitial lung disease and mild bilateral bronchiectasis. Immunology analysis was negative for ANA Antibody (Ab), Anti-neutrophil ab, anti-DNA ab, Glomerular basement membrane Ab, Cardiolipin Ab likewise for viral hepatitis tuberculosis Varicella Dengue Treponema. Blood and urine cultures were negatives. IgA vasculitis was diagnosed based on biopsy of the skin lesions resulting in small vessel IgA mediated immune complex leukocytoclastic vasculitis with purpura and focal epidermal necrolysis.Patient mental condition improved but developed acute lower GI bleed. EGD and colonoscopy evidenced clots and old maroon blood without source of bleeding founded. Patient continue experiencing GI bleeds and despite multiple blood transfusion he quickly deteriorated, and family opted to continue with hospice care.
Discussion: We have reported a unique case of adult-onset IgA vasculitis manifesting as typical rash, life-threatening GI bleeding and acute kidney injury. Though it primarily affects children (over 90% of cases), the occurrence in adults has been rarely reported (3.4 to 14.3 cases per million). This low incidence could result from underdiagnosis or misdiagnosis. For example, in this case, the rash was mistaken for an allergy, and joint pain was misinterpreted as “muscle pain even at minimal movements.” A retrospective review of 250 adults reported the clinical findings: a purpuric rash in 96% of cases, arthritis in 61%, GI disease in 48%, and renal disease in 32%. Proteinuria was found in 99% of these cases, and hematuria in 93%. When the disease is fatal, death is usually due to either gastrointestinal or renal causes. The treatment regimen remains controversial. Most cases are treated with NSAIDs, corticosteroids, or cyclophosphamide. Plasmapheresis and/or IVIG have been reported to benefit cases with massive GI bleeding that were unresponsive to recombination therapy.
Conclusions: IgA vasculitis is a heterogeneous disorder with manifestations in renal, gastrointestinal, musculoskeletal and skin. Less frequently affect lungs and central nervous system. This truly multi-system disease cause significant morbidity and mortality in adults.The diagnosis can be easily missed due to its rarity. A high index of suspicion and early clinical recognition can prevent treatment delays and reduce the risk of severe outcomes.
