Poster Session

P148. Complications of Darier's disease exacerbation in pregnancy: a case report

Flavia Marcelle Maia (BR), Maria da Conceicao Simoes (BR), Valeria do Nascimento (BR), Maria Melisande Pires (BR)

[Maia ] Maternidade Municipal Mae Esperanca, [Simoes] Maternidade Municipal Mae Esperanca, [do Nascimento] Maternidade Municipal Mae Esperanca, [Pires] Maternidade Regina Pacis

Introduction: Darier's disease, also known as acantholytic dermatosis, is an autosomal dominant disease and hereditary genodermatosis resulting from a mutation in the ATP2A2 gene found on chromosome 12q23-24.1. The diagnosis is by genetic study and / or cutaneous biopsy. The histopathological study reveals acantholysis that results in supra-basal fibrillation with papillomatosis and dyskeratosis. Case report: Patient: RSR, 21 years old, had an exacerbation of Darier's disease during gestation, a gestational age of 30 weeks, clinically hospitalized at the Ary Pinheiro Base Hospital (PVH-RO-Brazil) with severe pruritus, fever , Chills, hyperkeratotic papules and malodorous pustules and linear distribution, located in the neck, supra-mammary area, groin area and perineum. Discussion: In this report due to the exacerbation of Darier's disease, intense pruritus predisposed to a bacterial infection present on the skin due to loss of integrity. The cultures of pustules secretion in the perineal region were positive for Streptococcus agalactiae, indicating obstetric concern about the possible risks of premature rupture of the membranes, premature birth and high morbidity of neonatal sepsis after vertical transmission during vaginal delivery. Interventions: Prophylaxis was decided to Prevent neonatal infection by Group B Streptococcus with crystalline penicillin G + aminoglycoside for 10 days. Conclusion: Darier's exacerbation is treated with oral and / or topical retinoids. However, its use is contraindicated in pregnancy due to the risk of teratogenicity. The pregnant woman should be well informed about skin care during high-risk prenatal care: use of moisturizers, emollients and sunscreen, avoid exposure to the sun, use of airy clothing and use of antihistamine to soften pruritus In an attempt to maintain skin integrity, avoiding opportunistic infections, which, when present, can lead to severe maternal-fetal complications.