Collodion Baby Syndrome
Differential diagnosis in this case on the basis of abnormal skin are diseases leading to collodion baby syndrome. 🔷🔷Cigarette paper skin are usually localised patches of cigarette paper like skin, in this case skin all over the body is affected. 🔷🔷But yes this can be a very rare case of EDS type 1 with cigarette paper skin all over the body with abnormal joint mobility. . ⏩But most probable diagnosis here is Collodion baby. ⏩Collodion baby is a rare congenital disorder characterized by parchment-like taut membrane covering the whole body, often resulting in ectropion and eversion of the lips. ⏩These babies are usually born before term. The parchment-like membrane undergoes desquamation with time. ✅Once this layer peels off, underlying skin disorder is usually revealed. ⏩One-tenth of these patients later develop normal skin and called self-healing collodion baby. ⏩Another end of spectrum in keratinizing disorder presenting at birth is Harlequin fetus. ✅These babies have horny plates all over the body. ⏩Prognosis is poor with most of these babies die within first few days to weeks of life because of respiratory difficulty, inability to feed, and severe skin infections. ✅Patients who survive beyond infancy have severe ichthyosis and variable neurologic impairment. ⏩In all cases, barrier function of the skin is affected once collodion dries up and starts cracking. This leaves the baby susceptible to infection, temperature instability, and excessive trans-cutaneous fluid losses leading to dehydration. ⏩Thick membrane can create problems related to free movement leading to difficulties with breast-feeding. If chest wall movements are restricted, it may cause hypoventilation. ⏩Inability to close eyelids may cause corneal damage if proper precautions are not followed. ⏩Primary pathology in ichthyosis is disordered cornification, which leads to formation of collodion membrane
