Tumid lupus erythematosus in C1-inhibitor deficiency.
J R Coll Physicians Edinb
; 54(2): 159-160, 2024 Jun.
Article
en En
| MEDLINE
| ID: mdl-38756017
ABSTRACT
A case of a 28-year-old woman with known C1-inhibitor deficiency (functional, Type II) with persistent bilateral non-pruritic, mildly photosensitive facial rash for 10 months following delivery of her second child is presented. Histology of the skin was suggestive of tumid lupus erythematosus (LE), but no other features of systemic LE (ANA, dsDNA negative) were evident. She had stopped danazol which was controlling the underlying disease, and once this was restarted and treatment for tumid lupus was started, she improved. More rigorous control preventing all C1-inhibitor deficiency-related attacks proved successful. The hypothesis that uncontrolled classical pathway complement activation that led to the lupus-like skin lesions is being presented as a clinical case, highlighting the complex interrelationships between immunodeficiency and autoimmunity in inborn errors in immunity.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Lupus Eritematoso Cutáneo
Límite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
J R Coll Physicians Edinb
Asunto de la revista:
EDUCACAO
/
HISTORIA DA MEDICINA
/
MEDICINA
Año:
2024
Tipo del documento:
Article
País de afiliación:
Reino Unido