Miss XYZ, 7yr old female, born out of 3rd degree consanguineous marriage presented with
1. Skin lesions since birth
2. Exacerbation since 1 week
• Mother gives history of noticing redness, scaling, tightening and dryness of skin since birth.
• H/O parchment like membrane covering the body at birth which gradually peeled off to expose red, tight, dry, scaly skin.
• H/O associated scaling of scalp
• H/O inability to completely close the eyes ++
• H/O INTOLERANCE TO HEAT
• H/O frequent exacerbations which were more in winter and during episodes of fever/cough/cold.
• Generalised involvement of entire body including scalp, palms and soles in the form of erythema, scaling, ichthyosis and tightness
• Ichthyosis-predominately over the extremities
• Scaling-fine, whitish scales over the body. Thick, loosely adherent scales over the scalp
• Oral cavity-no erosions
• Eyes-eversion and ectropion of B/L lower eyelids
• Ears-scaling and debris (+) in external auditory canal.