A Rare Dermatological disorder in Newborn

Dr.Vivek Shyam DNB 2nd year Deptt. of Pediatrics

Case Reporty Ectropion, absense of eyelashes, and eye brows, dysmorphic ears

mouth was fish like (eclabium). Skin was semitransparent, parchment like feel with varying degree of fissures at groin and axilla, and was glistening membrane resembling scales of skin. Nails were hypoplastic and joint stiffness. y AF was normal, Chest-adequate air movements. No wheeze or rales , rhythmic heart sounds in good intensity, neonatal reflexes couldnt be elicitated,Genitalia was consistent with age and sex, gestatioanal age was estimated 38wks by ballaord scoring , extremities were complete and symmetrical although hardened and with poor distal and digital development ,Bwt. 2.65 kg. HC33 cms, Length 49 cms. no other gross cong. Anomaly seen Male baby,LSCS , 1st issue, 2nd degree consanguineously marrige, unventfull antenatal history,

Collodian baby- Discussiony Rare disorder. y 1:300,000 cases of newborns worldwide. M=F equal. y Term was coined in 1892 by Hallopeau and cockayne. y Autosomal recessive inheritance. y Single phenotype for several genotype.. y Disorder of Cornification (Ichthyosis). y All types of ichthyosis have dry, thickened, scaly or flaky

skin.In many types the skin is said to resemble the scales on a fish.

y This is a condition in which babies are born covered by a shiny

transparent membrane,this gradually peels off after 2 wks. y Collodion membrane is thickened stratum corneum saturated with water, as water evaporates leaving large fissures in skin and membrane is shed, revealing red skin underneath. y Ultimately the child may develop normally or may develop one of the severe disorders of keratinization. y Disfigurement and considerable psychological stress.

Disorders of cornification TypesHarlequin Fetus. Collodion baby. Lamellar Ichythyosis and Congenital Ichthyosiform Erythroderma. IchthysosisVulgaris. X-linked Ichthyosis. Epidermolytic Hyperkeratosis. Ichythyosis linearis Circumflexa. Ichthyosiform dermatoses : Sjogren-larsson Synd., Netherton Synd. Refsum Synd.

Morbidityy Cutaneous infections. y Aspiration Pneumonia. y Hypothermia. y Hypernatremic dehydration. y Subsequently Ichthyosis or reports of spontaneous healing

occurs in 10-25%

Treatmenty High-humidity environment. (humidified incubators) y Hydration. y Application of non-occlusive lubricants. y General Supportive care

(IVF ,Vit.K, Broad spectrum antibiotics.Skin dressing with vaseline, fissures with Mupirocin) Gradually,enteral feeds as tolerated. Dermatological opiniony Multidisciplinary approach-psychological support to children and education of parents.

Conclusion- Early recognition and early institution of appropriate therapy can definitely reduce morbidity and mortatlity.

thankyou