Adams – Oliver Syndrome

Avadhesh Joshi,Bhag Singh Karnawat,Manoj Kumar Jangid

doi:10.3126/jnps.v35i2.12409

Avadhesh Joshi, Bhag Singh Karnawat + Show 1 more

Open Access

https://doi.org/10.3126/jnps.v35i2.12409

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Abstract

Aplasia cutis congenita is characterized by congenital absence of portion of skin over a localized or widespread area. Adams- Oliver syndrome (subtype-II of ACC) is associated with distal limb reduction anomalies. We describe an infant with this uncommon disease associated with multiple midline lesions, which is a rare occurrence. J Nepal Paediatr Soc 2015;35(2):168-171

Highlights

Aplasia cu s congenita (ACC) is characterized by congenital absence of a por on of skin over a localized or widespread area
Frieden classified ACC into 9 subtypes. (Table 1) Adams-Oliver syndrome (AOS) is subtype- II of ACC which is associated with distal limb reduc on anomalies
Aplasia cu s congenita (ACC) is a rare inherited developmental defect characterized by absence of skin in small patches

Summary

Introduction

Aplasia cu s congenita (ACC) is characterized by congenital absence of a por on of skin over a localized or widespread area. (Table 1) Adams-Oliver syndrome (AOS) is subtype- II of ACC which is associated with distal limb reduc on anomalies. Physical growth and psychomotor development were within normal limits On examina on he had a smooth, round bald scar of 9 cm maximum diameter with dilated veins and a rim of hypertrichosis surrounding it over mid scalp. Stellate bald scar of 21 cm maximum diameter with glistening surface, surrounded by rim of hyper pigmented skin was present over lower mid-back (Fig.[1]). He had hypoplasia of toes in both feet, more marked in li le toes (Fig.[2]).

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