Literature DB >> 25657923

Collodion baby with polydactyly.

Iffat Hassan1, Atiya Yaseen1, Kaisar Ahmed2.   

Abstract

Entities:  

Year:  2015        PMID: 25657923      PMCID: PMC4314894          DOI: 10.4103/2229-5178.148953

Source DB:  PubMed          Journal:  Indian Dermatol Online J        ISSN: 2229-5178


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Sir, The term collodion baby is used to describe the transient appearance of a collodion membrane at birth and in the neonatal period of a baby. At birth, the typical collodion baby has characteristic clinical picture with a parchment like taut membrane covering the whole body, that cracks within 48 h and desquamates in large lamellae after a few days leaving an almost normal appearing skin that shows some scaling on the trunk. The eyelids and sometimes the lips, are tethered and everted (ectropion and eclabion), the pinnae may be flattened and the nostrils obstructed.[1] Collodion baby associated with polydactyly is an exceedingly rare entity. A 5-day-old baby boy, born of a consanguineous marriage after an uneventful full term pregnancy presented with a glistening membrane that totally wrapped his body. The membrane had begun to crack on the second day. The baby boy was second in birth order. His older sister aged 5 years was also born as a collodion baby. Though not available for clinical examination, she was healthy, with only mild scaling on the trunk and limbs. On physical examination, the child's weight was 2500 g, height was 47 cm and head circumference 34 cm. He had a shiny membrane resembling oiled skin [Figure 1]. The membrane showed numerous fissures in the groins, axillae, neck, wrists, ankles, cubital, and popliteal fossae. In addition, severe ectropion, eclabium and flattening of ears and nose, claw like hands, and limitation of joint movements were noted. He also had postaxial polydactyly (hexadactyly) of both hands and feet [Figures 2 and 3]. The polydactyly was type A in both hands and feet. Routine laboratory investigations revealed no abnormality.
Figure 1

Thick taught parchment-like membrane wrapping the baby showing desiccation at a few places

Figure 2

Hexadatyly of both hands

Figure 3

Poydactyly of both feet

Thick taught parchment-like membrane wrapping the baby showing desiccation at a few places Hexadatyly of both hands Poydactyly of both feet Collodion baby is a rare congenital disorder with incidence of 1 in 300,000 live births.[2] The condition is usually a manifestation of congenital ichthyosiform erythroderma or lamellar icthyosis.[3] Up to 20% of collodion babies subsequently have normal or near-normal skin, known as self-healing collodion baby or “lamellar ichthyosis of the newborn.”[4] Polydactyly can be classified into preaxial, involving the thumb or great toe and postaxial, affecting the fifth digit; and central, involving the three central digits. Postaxial polydactyly has further been classified into types A and B. In type A postaxial polydactyly the extra digit is well formed and articulates with the fifth or an extra digit. In postaxial polydactyly type B, the extra digit is frequently in the form of a skin tag.[45] We report the present case considering the rarity of reports describing the association of a collodion baby with polydactyly.
  2 in total

1.  A and B postaxial polydactyly in two members of the same family.

Authors:  V Ventruto; G Theo; A Celona; G Fioretti; L Pagano; M Stabile; M L Cavaliere
Journal:  Clin Genet       Date:  1980-11       Impact factor: 4.438

2.  Self-healing collodion baby: a dynamic phenotype explained by a particular transglutaminase-1 mutation.

Authors:  Michael Raghunath; Hans-Christian Hennies; Bijan Ahvazi; Melanie Vogel; Andre Reis; Peter M Steinert; Heiko Traupe
Journal:  J Invest Dermatol       Date:  2003-02       Impact factor: 8.551

  2 in total
  1 in total

1.  Pedunculated Hemorrhagic Cystic Swelling: An Unusual Presentation of Polydactyly.

Authors:  Kavita Bisherwal; Archana Singal; Deepika Pandhi; Sonal Sharma
Journal:  Indian Dermatol Online J       Date:  2017 May-Jun
  1 in total

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