DISCUSSION
RTS presents at three to six months’ age with erythematous macule or plaque over face, sometimes associated with blisters and edema which gradually progress to involve extremities and gluteal regions sparing trunk and abdomen. 1 Gradually, poikiloderma develops over the initial lesion that persist throughout the life. In few of the cases, there might be systemic involvement that includes dental abnormalities (microdontia, conical teeth, frequent caries, loss of teeth), ocular involvement (bilateral subscapular cataract) and bone abnormalities (absent or deformed radii, deformed ulnar bone, short hands and feet, aplasia of thumb, delayed bone age and osteoporosis)1. There is increased risk of osteosarcoma in childhood and epithelial neoplasms in adult.2 Patients have a normal life span unless associated with malignancy. Strict photoprotection and routine screening for malignancy are the mainstay of management.
Patients in our case had characteristic rash of RTS developing at one month’s age, initially acute features of rash which developed into poikiloderma that was persistent with typical distribution pattern and had delayed growth. However other systemic features of RTS were absent.
Detailed history and careful examination may aid in differentiating other causes of childhood poikiloderma. Acrogeria manifests at the time of birth or shortly afterwards with poikiloderma limited to acral parts3. Hereditary sclerosing poikiloderma presents in childhood with generalized poikiloderma with accentuation in flexures and extensor surfaces along with sclerodermatous plaques on palms and soles4. Dyskeratosis congenita is characterized by triad of severe nail involvement, poikiloderma conspicuous over neck and leucoplakia at a later age5. Kindler syndrome is characterized by poikiloderma that develops at the age of 2 to 3 years in photo-exposed sites along with acral blisters and mucosal stenosis2. Patients with Cockayne’s syndrome develops poikiloderma that spreads centripetally starting from distal limbs along with typical facies, limb abnormalities, wasting and neurological manifestations6. Rare disorders like Bloom’s syndrome, Fanconi’s anemia and Ataxia telangiectasia present with prominent telangiectasia and characteristic facies2.