<unk>  is a rare disorder characterized by<unk> l absence of skin. Frieden classified<unk>  in 1986 into 9 groups on the basis of location of the lesions and associated<unk> l anomalies. The scalp is the most commonly involved area with lesser involvement of trunk and extremities. Frieden classified<unk>  with fetus papyraceus as type 5. This type presents as truncal<unk>  with symmetrical absence of skin in stellate or butterfly pattern with or without involvement of proximal limbs. It is the most common<unk> l cicatricial alopecia, and is a<unk> l focal absence of epidermis with or without evidence of other layers of the skin. The exact etiology of<unk>  is still unclear but intrauterine infection by varicella or herpes virus, drugs such as methimazole, misoprostol, valproate, cocaine, marijuana etc., fetus papyraceus, feto-fetal transfusion, vascular coagulation defects, amniotic membrane adherence, abnormal elastic fiber biomechanical forces and trauma are implicated. It can be associated with Johanson-Blizzard syndrome, Adams-Oliver syndrome, trisomy 13, and Wolf-Hirschhorn syndrome. It can also seen with exposure to methimazole and carbimazole in utero. This dermatological manifestation has been linked to Peptidase D haploinsufficiency and a deletion in Chromosome 19.
Aplasia cutis congenita
Encephalocraniocutaneous lipomatosis
Acrocephalosyndactylia
Pentalogy of Cantrell
Ghosal hematodiaphyseal dysplasia
Diaschisis
Rhizomelia
Sirenomelia