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.