[Fatal outcome of Netherton syndrome due to excessive use of topical corticosteroids in an adult patient]. / Évolution fatale du syndrome de Netherthon due à une utilisation excessive de dermocorticoïdes chez un adulte.
Ann Dermatol Venereol
; 147(1): 36-40, 2020 Jan.
Article
em Fr
| MEDLINE
| ID: mdl-31653452
ABSTRACT
INTRODUCTION:
Netherton syndrome (NS) is a rare disease caused by SPINK5 mutations associated with ichthyosis (erythroderma and desquamation), alopecia and atopic manifestations. There are no effective treatments. Topical corticosteroids may be used for a limited period in the event of eczema. Herein we report on a patient with fatal complications related to misuse of topical corticosteroids. PATIENTS ANDMETHODS:
A 38-year-old woman with NS had been using betamethasone for about ten years for severe pruritus. Consumption was estimated at 7.2kg per year. On examination, she had osteoporosis, Cushing's syndrome, corticotropic insufficiency and inframammary, axillary, and intergluteal superinfected intertrigo. During hospitalization for necrotic leg wounds on severe skin atrophy, she sustained a fracture on falling down. The course was marked by the onset of septic shock of unknown etiology, complicated by acute adrenal insufficiency leading to fatal multi-organ failure.DISCUSSION:
Many iatrogenic cases related to topical corticosteroids in children have been reported in the literature, including one case of fatal outcome (CMV infection) in an infant. Such iatrogenic cases are rarer in adults and we observed no fatal cases. In NS, the adverse effects of topical corticosteroids are amplified due to the major defect in the skin barrier which enhances the systemic passage of these drugs. In the absence of any effective therapeutic alternative, weaning patients off topical corticosteroids is usually difficult.CONCLUSION:
This case illustrates the severity of iatrogenic effects secondary to misuse of topical corticosteroids in NS as well as the need to find effective new treatments for this syndrome.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Betametasona
/
Síndrome de Netherton
/
Glucocorticoides
Tipo de estudo:
Etiology_studies
Limite:
Adult
/
Female
/
Humans
Idioma:
Fr
Revista:
Ann Dermatol Venereol
Ano de publicação:
2020
Tipo de documento:
Article