TY - JOUR T1 - Pain, swelling and irritability in the sun: what is the diagnosis? JF - Archives of disease in childhood - Education & practice edition JO - Arch Dis Child Educ Pract Ed SP - 102 LP - 106 DO - 10.1136/archdischild-2019-317384 VL - 106 IS - 2 AU - Sinead Mary McGlacken-Byrne AU - Lisa Dann AU - Annette Murphy AU - Edina Moylett Y1 - 2021/04/01 UR - http://ep.bmj.com/content/106/2/102.abstract N2 - A 7-year-old boy presented with a 24-hour history of severe burning pain affecting both hands that had started within minutes of playing outdoors. His mother reported that he had been running his hands under cold water and shaking his hands and head to try and relieve the pain. On examination, there was swelling of his hands, eyelids and cheeks. His parents mentioned that his hands and face had ‘swollen in the sun’ every summer from the age of 2 years.A 3-year-old girl presented with a 2-day history of left upper limb swelling following a day of prolonged sun exposure. Examination revealed non-pitting oedema extending from her left shoulder to hand with no associated tenderness, erythema or rash. Her mother reported six previous episodes of irritability following sun exposure during which she would cry and flap her hands ‘for hours’.Oral steroids and antihistamines were prescribed in both cases with little effect. Findings of routine baseline investigations were normal in both cases. A radiograph of the upper limb in the second patient disclosed nothing abnormal.QuestionsWhat is the most likely diagnosis for both patients?Venomous insect bites/stingsErythropoietic protoporphyriaIgE-mediated reactionMixed connective tissue diseaseComplement deficiencyWhich investigative approach will be most helpful?Complement function evaluationPhysical urticarial evaluationPorphyria work-upAutoantibody screenImmunodeficiency work-upHow would you manage these patients?Questions Answers can be found on page 02. ER -