Ibargüengoitia-Ochoa F, Lira-Plascencia J, Canuto A, López-Torres MF

Language: Spanish
References: 13
Page: 64-70
PDF size: 272.20 Kb.

ABSTRACT

Background: Epidermolysis bullosa encompasses a group of diseases characterized by extreme fragility of the skin and mucous membranes, resulting in the formation of blisters after minimal trauma; There are three main types of epidermolysis. The case is presented to highlight the implications of care both at the time of birth, as well as anesthetic considerations.
Clinical case: A 25-year-old patient in her second pregnancy with recessive dystrophic epidermolysis bullosa. The birth was elective by caesarean section under regional anesthesia.
Conclusions: Special care must be taken during therapeutic interventions to avoid the formation of bullae or exacerbate those already present. Friction forces are more damaging than compression forces. Multidisciplinary planning is necessary for a smooth outcome.

REFERENCES

1. Intong L R, Murrell D F. Inherited epidermolysis bullosa: newdiagnostic criteria and classification. Clin Dermatol 2012;30(1): 70-7. doi:org/10.1016/j.clindermatol.2011.03.012

2. Fine J D, Mellerio J E. Extracutaneous manifestations andcomplications of inherited epidermolysis bullosa: part II.Other organs. J Am Acad Dermatol 2009; 61(3): 367-84.doi: org/10.1016/j.jaad.2009.03.053

3. Baloch MS, Fitzwilliams B, Mellerio J, Lakasing L, BewleyS, O’Sullivan G. Anesthetic management of two differentmodes of delivery in patients with dystrophic epidermolysisbullosa. Int J Obstet Anesth 2008; 17 (2): 153-8. http://dx.doi.org/10.1016/j.ijoa.2007.04.008

4. Bolt LA, O’Sullivan G, Rajasingham D, Shennan A. A reviewof the obstetric management of patients with epidermolysisbullosa. Obstet Med 2010; 3 (3): 101-5. http://dx.doi.org/10.1258/om.2010.100009

5. Intong LRA, Choi SD, Shipman A, Kho YC, Hwang SJE,Rhodes LM, et al. Retrospective evidence on outcomesand experiences of pregnancy and childbirth in epidermolysisbullosa in Australia and New Zealand. Int J WomensDermatol 2017; 3 (1): S1-5. http://dx.doi.org/10.1016/j.ijwd.2017.02.002

6. Araújo M, Brás R, Frada R, Guedes-Martins L, Lemos P. Caesareandelivery in a pregnant woman with epidermolysisbullosa: anesthetic challenges. Int J Obstet Anesth 2017;30: 68-72. http://dx.doi.org/10.1016/j.ijoa.2017.01.010

7. Shah N, Kumaraswami S, Mushi J E. Management of epidermolysisbullosa simplex in pregnancy: a case report.Case Report Women`s Health 2019; 24: e00140. doi:org/10.1016/j.crwh.2019.e00140

8. Bruckner AL, Losow M, Wisk J, Patel N, Reha A, Lagast H, etal. The challenges of living with and managing epidermolysisbullosa: insights from patients and caregivers. OrphanetJ Rare Dis 2020; 15 (1). http://dx.doi.org/10.1186/s13023-019-1279-y

9. El Hachem M, Zambruno G, Bourdon-Lanoy E, CiasulliA, Buisson C, Hadj-Rabia S, et al. Multicenter consensusrecommendations for skin care in inherited epidermolysisbullosa. Orphanet J Rare Dis 2014 ;9 (1): 76. http://dx.doi.org/10.1186/1750-1172-9-76

10. Tang JY, Marinkovich MP, Lucas E, Gorell E, Chiou A, Lu Y,et al. A systematic literature review of the disease burdenin patients with recessive dystrophic epidermolysis bullosa.Orphanet J Rare Dis 2021;16 (1): 175. http://dx.doi.org/10.1186/s13023-021-01811-7

11. Berryhill RE, Benumof JL, Saidman LJ, Smith PC, Plumer MH.Anesthetic management of emergency cesarean section ina patient with epidermolysis bullosa dystrophica polydysplastica.Anesth Analg 1978; 57 (2): 281.3. http://dx.doi.org/10.1213/00000539-197803000-00026

12. Simpson B, Tarango C, Lucky A W. Clinical algorithm tomanage anemia in epidermolysis bullosa. Pediatr Dermatol2018; 355 (5): 1-2. doi:10.111/pde.13587

13. Scherhag A, Dick W. Special aspects of anesthesia in patientswith epidermolysis bullosa based on a case example.Anaesthesiol Reanimat 1998; 23 (5): 129-33. https://pubmed.ncbi.nlm.nih.gov/9854331/