Abstract
BACKGROUND:
Congenital ichthyosis is a broad term for a variety of keratinizing conditions characterized by extensive scaling and varying degrees of erythema. Little is known about the disease's effects on families and the quality of life experienced by kids with congenital ichthyosis. Defects in keratinization are the cause of the varied group of illnesses known as ichthyoses. Making prognosis assessments, treatment choices, and providing genetic counseling in patients with ichthyosis require accurate clinical diagnosis. However, clinical variability in some cases makes it difficult to make a definite diagnosis. Ichthyoses are a diverse category of diseases caused by aberrant epidermal differentiation and desquamation, as well as defects in keratinization or cornification. Clinically, it is distinguished by scaling and dry, rough skin that covers much or the entire body surface. Congenital ichthyosis nomenclature and nosology have changed over time, resulting in a bewildering array of terminologies and classification schemes.
AIM: To study the clinical presentation of various types of congenital ichthyosis in the pediatric age group.
MATERIAL AND METHOD:
The Department of Dermatology carried out this cross-sectional observational study. Based on clinical patterns, 50 ichthyosis cases in total were enrolled in the study. For comparative research, the total number of new patients who visited the department throughout the time period was enrolled. A predetermined pattern of questionnaires was used to obtain the history. Itching, reduced sweating, heat intolerance, a history of collodion infant, skin blistering, seasonal variation, cyclical loss of skin, photosensitivity, and photophobia were all topics of inquiry.
RESULTS:
Congenital ichthyosis affected 50 people, with ichthyosis vulgaris accounting for 72% of cases and lamellar ichthyosis accounting for 10%. Bullous ichthyosis form erythroderma (BIE), Sjogren-Larsson syndrome, and non-bullous ichthyosis form erythroderma (NBIE) each made up 2% of the total. Ichthyosis vulgaris was about equally prevalent in both sexes. Lamellar ichthyosis was more common in females. In the NBIE, the distribution of sexes was equal.
CONCLUSION:
There are several distinct types of ichthyoses that have distinguishing characteristics and can be accurately diagnosed. However, due to significant clinical heterogeneity, a definitive diagnosis can be difficult in some patients and families. The diagnosis is generally aided by knowing if ichthyosis is inherited or acquired, present at birth or later in life, and whether it affects only the skin or is a component of a multisystem condition. Other helpful clinical findings include scale quality and distribution, erythroderma presence or absence, blistering, and related abnormalities of the skin adnexa. Recognizing the inheritance pattern requires a good understanding of family history.
KEYWORDS: Congenital ichthyosis, Collodion baby, Ichthyosis Vulgaris and Keratinization