Caffeys disease. Report of a clinical case.

Abstract

Introduction: Caffey s disease is a rare disease that is reported almost exclusively in infants.
Objective: Describe the case of a rare pathology, prioritizing the diagnostic approach.
Clinical case: 4 month –old, healthy male. Consultation due to irritability and edema of the left lower limb for 4 days. No trauma or fever.
Physical examination: indurated edema in the left leg, no redness or local heat. Pain on palpation of the anterior and lateral aspect of the tibia and fibula. Functional limitation, no bony protusions.
Leg x-ray: thickening of the periosteum in the tibia and fibula at the diaphyseal level.
Hemogram: White Blood Cells 15,380 KU/L Hemoglobin: 10.8 g/dL. Platelets: 816,400 10/ul, C-reactive protein: 13.90 mg/dl. He was admitted with a suggestion of probable osteoarticular infection. Clindamycin  gentamicin is started.
Given the persistence of edema and irritability despite treatment, on the fifth day an MRI was requested: findings suggestive of a probable inflammatory-infectious process of soft tissues with bone involvement.
Completed 14 days of clindamycin and 7 days of intravenous gentamicin, blood culture negative. It persists with edema, irritability and pain. After 21 days, the clinical-imaging presentation was reassessed and Caffey's disease was considered.
Anti-inflammatory begins with good evolution.
Conclusions: Caffey's disease is a rare collagenopathy, that affects infants. The diagnosis is clinical - radiological (irritability, soft tissue swelling and radiological alterations).
The long-term prognosis is usually favorable. It is important to consider the diagnosis in infants who present with these symptoms to avoid diagnostic delays and initiation of unnecessary treatments.