Henoch–Schönlein purpura is a small vessel vasculitis that usually presents with palpable purpura, arthritis, abdominal pain, and nephritis. Subcutaneous oedema of dependent areas is common; however, oedema in the scalp is extremely rare especially in children older than two years. Here, we report a child with massive disfiguring scalp and facial oedema due to Henoch–Schönlein purpura. An eight-year-old boy presented with characteristic palpable purpuric rash and extensive disfiguring scalp and facial swelling for five days. He complained of blurred vision, vomiting, and severe headache on the day of admission. Examination revealed an ill child with extensive oedema of the face and scalp that was tender on palpation. His blood pressure was above the 99th percentile, and he had exaggerated deep tendon reflexes and extensor plantar responses. All biochemical investigations including renal function tests were normal. Noncontrast CT head showed normal brain, with marked soft tissue swelling of the scalp. Ultrasonography showed soft tissue oedema within and surrounding facial muscles without evidence of neck vessel compression. Urine analysis revealed microscopic haematuria on day 14 of the illness, and immunohistochemical staining of renal biopsy confirmed Henoch–Schönlein purpura nephritis. In conclusion, this case report presents a child with severe, disfiguring scalp and facial oedema due to Henoch–Schönlein purpura. It highlights that severe subcutaneous oedema of Henoch–Schönlein purpura can involve any part of the body not limiting to dependent areas.

Background Imerslund-Gräsbeck syndrome is a rare genetic disease characterised by vitamin B12 deficiency and proteinuria. Case presentation A 4-year old Sri Lankan boy presented with gradually worsening difficulty in walking for two weeks duration. He was previously diagnosed and managed as having non-transfusion-dependent α-thalassaemia based on the presence of hypochromic microcytic anaemia, haemoglobin H inclusion bodies in the blood film and compound heterozygous α-thalassaemia genotype with a gene deletion. However, his transfusion requirement increased over the past three months and he gradually lost his motor developmental milestones during two weeks before admission. The neurological examination revealed generalised hypotonia, exaggerated knee jerks and extensor plantar response. His complete blood count showed pancytopenia, and bone marrow biopsy revealed megaloblastic changes. Serum vitamin B12 and red blood cell folate levels were low. MRI revealed sub-acute combined degeneration of the spinal cord with characteristic ‘inverted V sign’. Urine analysis showed non-nephrotic range proteinuria. The diagnosis of Imerslund-Gräsbeck syndrome was made due to the presence of non-nutritional vitamin B12 deficiency and asymptomatic proteinuria. He showed a rapid haematological and neurological improvement to intramuscular hydroxocobalamin. Conclusions This case report presents a rare occurrence of severe vitamin B12 deficiency due to Imerslund-Gräsbeck syndrome masked by co-existent α-thalassaemia, resulting in serious consequences. It highlights the need for a high index of suspicion in evaluating children with severe anaemia, especially in the presence of mixed pathologies.