We describe an atypical pediatric case of immunoglobulin A vasculitis (IgAV), also referred to as Henoch-Schönlein purpura, in which formation of spontaneous hematoma of the paraspinal muscles developed. Spontaneous or unprovoked hematomas rarely occur in IgAV. These manifestations have not been described specifically in the pediatric literature as coinciding with IgAV. These findings are alarming for nonaccidental trauma, particularly in a patient without underlying blood dyscrasia. Our objective for this report is to highlight the possible association of muscular hematoma formation with IgAV and to help providers consider this association when trauma and hemophilia has been ruled out.

Immunoglobulin A vasculitis (IgAV) is well documented to present most commonly in childhood with palpable purpura, gastrointestinal (GI) complaints, arthritis, and renal involvement. We report the first case of a paraspinal muscular hematoma as a manifestation of IgAV.

A 4-year-old, previously healthy boy presented to the emergency department with a rash of his lower extremities and buttocks for 2 days. His examination result was normal, with the exception of scattered palpable purpura on the bilateral legs and buttocks. He was discharged with the diagnosis of IgAV and given instructions for supportive care.

He returned to the emergency department 2 days later with acute onset of back pain and spreading of the rash to include areas of his bilateral upper extremities and the helices of his ears. The family observed the patient as uncomfortable with lying flat, standing, and walking, despite taking ibuprofen for pain. An examination was notable for subcutaneous swelling and tenderness to palpation over the paraspinal area to the left of the lumbar spine, without evidence of overlying bruising or petechiae. There were no neurologic deficits or findings indicative of spinal cord compression.

A computed tomography (CT) scan of the chest, abdomen, and pelvis revealed a large intermediate density collection, measuring ∼1 × 9 × 12 cm, consistent with a hematoma overlying the paraspinal musculature, with increased vascular enhancement of the left paraspinal vessels. Given this finding, additional evaluation for trauma was pursued with a CT scan of his head, which revealed a small subdural hemorrhage without mass effect, and a skeletal radiograph survey result was normal. A complete blood cell count was remarkable for normocytic anemia, with a hemoglobin level of 10.6 g/dL, which was a 3-g/dL decrease from his previous hemoglobin level obtained at the initial emergency department presentation. The child was admitted to the pediatric hospital medicine service for further evaluation and treatment.

Because of the atypical nature of his symptomatology, multiple consultants were engaged in his evaluation, including hematology, rheumatology, trauma surgery, ophthalmology, dermatology, and child abuse medicine. A detailed hematologic evaluation was pursued, with tests performed for the following: prothrombin time; partial thromboplastin time; fibrinogen; assays for factor II, factor VIII, factor IX, and factor XIII; von Willebrand activity and antigen; platelet function assay; dilute Russell’s viper venom time; and lupus anticoagulant. Results were ultimately found to be normal. A rheumatologic evaluation was pursued, with tests performed for the following: antineutrophil cytoplasmic antibody, β-2 glycoprotein immunoglobulin G, immunoglobulin A (IgA) and immunoglobulin M, cardiolipin IgA, immunoglobulin G and immunoglobulin M, anti–double-stranded DNA antibody, antinuclear antibody, protease-3 antibody, myeloperoxidase antibody, and adenosine deaminase deficiency. Results were found to be normal. An echocardiogram result was within normal limits, and the result of an ophthalmologic examination was normal. He was evaluated by a child abuse specialist, and it was determined that his presentation was not consistent with inflicted trauma. Because of the initial presentation of a rash consistent with IgAV and the risk of further organ involvement while diagnostic testing was pending, intravenous (IV) methylprednisolone was initiated soon after admission.

Dermatology obtained a skin punch biopsy from the rash on his left lower extremity that ultimately revealed leukocytoclastic vasculitis consistent with IgAV. An MRI of the brain was normal. His rash revealed improvement after the second dose of IV methylprednisolone, and he had no further hematoma development. An ultrasound of the paraspinal soft tissue was performed to reevaluate the hematoma and revealed resolution of the collection previously identified on the CT scan. He received 3 days of IV methylprednisolone and was discharged from the hospital with an oral prednisone taper.

At his rheumatology follow-up appointment 2 weeks after this hospitalization, he had a worsening purpuric rash and new onset of hematuria and proteinuria. He was then admitted to the hospital to receive treatment with cyclosporine and pulse IV methylprednisolone. Nephrology performed a renal biopsy, which revealed ∼2% global glomerulosclerosis, focal mild interstitial fibrosis, and mesangial proliferative IgA-dominant glomerulonephritis consistent with IgAV.

IgAV, also referred to as Henoch-Schönlein purpura, is the most common childhood vasculitis. It is a systemic vasculitis involving IgA deposition in the small blood vessels of affected organs, most commonly in the skin, GI tract, joints, and glomeruli.1  Patients will classically have a nonthrombocytopenic palpable purpura, arthritis, and GI complaints. There is a predilection for purpura to affect dependent areas of the lower extremities and buttocks1 

The pathophysiology of this vasculitis is not completely understood. Its pathogenesis involves deposition of immune complexes containing IgA and vascular infiltration of neutrophils.1  The neutrophils infiltrate and degenerate, leading to leukocytoclasia with thickening and necrosis of the vessel walls. This endothelial damage in the setting of a proinflammatory state contributes to the thrombogenicity of the acute vasculitis, leading to altered hemodynamics.2  This dysregulation is speculated to contribute to hemorrhage and hematoma formation. Extravasated erythrocytes in the dermis surrounding these affected vessels manifest as purpura.1  A complete blood cell count will reveal a normal or increased platelet count and possible moderate leukocytosis with left shift.3  Studies have supported evidence of a genetic predisposition in people who develop IgAV. There is a strong association with the HLA antigen class II region and HLA-DRB1, and, in particular, the HLA-DRB1*01 allele has been reported in people of European descent.4 

The diagnosis of IgAV is based on criteria established by collaboration of the European League Against Rheumatism, the Pediatric Rheumatology International Trials Organization, and the Pediatric Rheumatology European Society.3,5  These diagnostic criteria are palpable purpura plus at least one of the following: diffuse abdominal pain, histopathology revealing leukocytoclastic vasculitis with predominant IgA deposits or proliferative glomerulonephritis with predominant IgA deposits, arthritis or arthralgias, and/or renal involvement.3  Consensus recommendations for treatment with corticosteroids include cases of orchitis; cerebral vasculitis; pulmonary hemorrhage; moderate to severe nephritis; severe GI involvement, such as bowel infarction, perforation, or hemorrhage; or other severe organ- or life-threatening vasculitis manifestations. In severe cases, it is recommended that prompt initiation of high-dose IV methylprednisolone be considered for 3 consecutive days.5  The adjuvant use of cytotoxic immunosuppressant medications or plasma exchange may be used in severe organ- or life-threatening vasculitis manifestations.5 

Atypical clinical manifestations of IgAV described in the literature include reports of cerebral venous thrombosis, a subdural hematoma, subarachnoid hemorrhage, and neuro-ophthalmologic complications. Hematomas are rarely described manifestations of IgAV. The only cases reported of a hematoma in IgAV involve the scrotum, adrenal gland, and orbits, with one case revealing intramuscular involvement in the leg of an adult.68  In one of the largest reported retrospective reviews of 417 patients with IgAV, three-quarters of whom were patients <20 years of age, a hematoma was not identified as one of the manifestations of IgAV.9  To our knowledge this is the first case to describe a pediatric case of IgAV with spontaneous hematoma development.

A paraspinal hematoma would typically occur after an inciting traumatic event, particularly in the presence of underlying hemophilia. In the case of our patient, the child abuse team conducted a thorough investigation, and there was no evidence of trauma. The hematologic evaluation result was also negative for underlying blood dyscrasia. With hematoma formation, there is a risk for spontaneous hemorrhage, which should be suspected with acute worsening of pain or acute decrease in hemoglobin levels, as occurred in our patient. A muscular hematoma should be considered as a rare, yet possible, complication of IgAV.

Dr Azer reviewed literature on this subject, contributed to the conceptualization of this report, and drafted the initial manuscript; Drs File and Leazer conceptualized this report; and all authors reviewed and revised the manuscript, approved the final manuscript as submitted, and agree to be accountable for all aspects of the work.

FUNDING: No external funding.

     
  • CT

    computed tomography

  •  
  • GI

    gastrointestinal

  •  
  • IgA

    immunoglobulin A

  •  
  • IgAV

    immunoglobulin A vasculitis

  •  
  • IV

    intravenous

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Competing Interests

POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.