Lamellar Ichthyosis
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Abstract
Objective: We aim to report a rare and severe case of congenital ichthyosis known as lamellar ichthyosis, emphasizing the clinical course, treatment and outcome. Case description: The patient was born as collodion baby phenotype, including bilateral central leukoma. She developed an extensive body surface flaking and required intensive care. She evolved episodes of sepsis, respiratory distress, anemia, nutritional disorder and necrosis of chirodactyl. Due to therapeutic support, with emphasis on clinical, nutricional, eye and skin care, the evolution was satisfactory after gastrostomy that was indicated due
to eclabium sucking difficulty. She was discharged in spite of keeping the cutaneous peeling. Her follow up evolved with no further complications. At present, she is able to take oral nutrition and expects to have the gastrostomy removed at any time. Discussion: Congenital ichthyosis covers a heterogeneous group of diseases with present skin peeling as the primary clinical manifestation. It can be classified according to histological, genetic and clinical peculiarities. Intensive care, especially regarding fluid and nutrient intake, infectious condition and dermatological sequelae are important. A relationship of trust between the medical team and the family is also essential in order to clarify important aspects about patient care in addition to genetic counseling for future pregnancies.
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