NON BULLOUS CONGENITAL ICHTHYOSIFORM ERYTHRODERMA
ABSTRACT: Non bullous
congenital ichthyosiform erythroderma (NBCIE) is an inherited genodermatosis,
very rare autosomal recessive inflammatory ichthyosis, chronic, characterized
by dryness and scaling. Purpose: To describe the clinical manifestations of
NBCIE since it is a rare case, occurs in 1 in 300.000 people. Case: Baby MR 5
month old, Javanese boy, with main complaint white scale and redness patches on
almost all of his body since 1.5 month old. Born as collodion baby, then the
membrane was slowly thinning and became generalized erythroderma on almost all
of his body accompanied with fine white scale, large, thick, plate-like scale
only on lower leg. No ectropion and eclabium. No relatives in the pedigree
suffer from the same disease. Histopathology examination showed non-bullous
congenital ichthyosiform erythroderma. Case management: Emollient after bath
and pH balance soap. Conclusions: Diagnosis of NBCIE is established from
history taking, clinical features, and histopathology examination. Emollient
therapy and pH balance soap will eventually lead to improvement.
Key words: non bullous
congenital ichthyosiform erythroderma, genodermatosis, collodion baby
Penulis: Lita Setyowatie,
Iskandar Zulkarnain
Kode Jurnal: jpkedokterandd140668