Hellinga, Bibia Charlotte (2020) Long-term evolution of autoimmune blistering diseases in childhood: a retrospective study on 29 children. thesis, Medicine.
Full text available on request.Abstract
Background Autoimmune blistering diseases (AIBDs) rarely occur in childhood and are considered to have a milder clinical course and better prognosis compared to adults, although literature is scarce. Especially little is known about the long-term evolution. Objective The give an overview of molecular characteristics, treatment response and clinical course including long-term evolution of AIBDs started in childhood. Methods A retrospective observational study was conducted at the Dutch National Centre for Blistering Diseases. All patients diagnosed with an AIBD from January 2002 until December 2019 and below 18 years were included. The medical files were searched to obtain clinical information including diagnostic results. Long-term outcomes concerning relapses, late complications and development of other diseases were obtained through a prospective questionnaire by phone. Results Forty-four patients were identified, fifteen linear IgA dermatosis, 15 bullous pemphigoid, 5 epidermolysis bullosa acquisita, 3 dermatitis herpetiformis, 3 pemphigus subtypes and 2 mucous membrane pemphigoid. Thirty-four patients (13 males and 21 females) were analyzed, with a mean age of onset of 4.96 years. Seven patients were below 1 year old, of which 4 were probably triggered (amoxicillin/clavulanic acid and vaccination). Systemic treatment was initiated in 24 patients. Complete remission off therapy was achieved in 29 patients at time of analysis with a mean duration of 19.5 months, with variability between subtypes. Twenty-nine patients were analyzed for long-term evolution, with a mean duration of follow-up of 8.8 years from onset of disease. Relapse was observed in 6 patients, of which 4 were minor with fast remission. One LAD patient developed lichen sclerosis and one EBA patient developed ulcerative colitis after the AIBD. Conclusion AIBDs in childhood show an overall good prognosis, in contrast to adult cases of AIBD. Most patients achieve remission. Systemic treatments were used in the majority of patients with good response, but treatment may be challenging. Relapse may occur after discontinuation of treatment, mostly of mild nature and quickly in remission. An AIBD should be considered in infantile patients if blisters appear after administration of amoxicillin/clavulanic acid or vaccination.
| Item Type: | Thesis (UNSPECIFIED) |
|---|---|
| Supervisor name: | Dr. Bolling, M.C. and Dr. Meijer, J.M. |
| Faculty: | Medical Sciences |
| Date Deposited: | 10 Oct 2023 11:02 |
| Last Modified: | 10 Oct 2023 11:02 |
| URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/3702 |
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