Ascia‐p78: A Case of Generalized Morphea With Linear Scleroderma on the Right Limb

Abstract

Diagnosis of Kawasaki disease (KD) requires presence of persistent pyrexia for ≥5days in absence of an infectious cause [1, 2]. Younger infants with KD often present with atypical or incomplete disease and are at increased risk of complications [3]. Certain overlapping features of KD with sepsis may cause diagnostic delay. One such feature is chest X-ray (CXR) abnormality in KD that is not frequently reported.Case report: 13 week old boy was admitted to a regional hospital in Victoria with <24 hour history of high temperature, poor feeding, irritability, diarrhoea, conjunctivitis with mucous discharge and generalised fine macular rash. Initial assessment revealed an alert yet irritable infant with dry mucous membranes, delayed central capillary refill time, high grade pyrexia, cool peripheries with normal breathing and heart sounds.Respiratory swabs, cerebro-spinal fluid, urine, blood and stool cultures and virology returned normal. Initial fluid bolus (20ml/kg) and IV antibiotics were administered on admission.Initial Hb and Albumin were low; CRP, ESR, WBC, Neutrophil and Platelet counts were raised and remained so in first 5 days of illness. CXR was suggestive of abnormal air space density in both perihilar regions in the right upper zone.Polymorphic rash was observed on day 4. Mild non-pitting oedema of hands and feet was noticed on day 5 and treatment with intravenous immunoglobulin infusion and aspirin were commenced. Irritability and pyrexia settled within hours of the infusion.2D ECHO on day 8 was reported normal. Desquamation of fingers and toes was evident on 2 week review.Discussion: Peri-bronchovascular haze has been described as a frequent CXR finding due to pulmonary vessel intimal thickening during acute phase of KD [4]. We suggest that CXR finding in a febrile infant without chest signs may support an early diagnosis of KD in absence of microbiological evidence of infection. Background: Generalized morphea with linear scleroderma is a rare disorder that usually occurs in childhood and can affect any area of the body surface.