'Vanishing carpal bones' in a patient with congenital adrenal hyperplasia - A diagnostic dilemma

The vanishing bone disease, also known as Gorhams disease usually involves long bones. Isolated carpal bone involvement is uncommon. A 14 year old male presented with pain in the right wrist joint since 4 months. He was a known case of Congenital Adrenal Hyperplasia on oral steroids for 3 years. X-rays showed deformed arthritic scaphoid, lunate and capitate. A proximal row carpectomy was performed to provide functional wrist mobility to the patient. Histopathological examination was consistent with Gorham's disease. In a patient with systemic disease receiving steroid therapy Gorhams disease poses a diagnostic challenge.