Pediatric autoimmune blistering disorders – a five‐year demographic profile and therapy experience

Mahajan, Rahul; Handa, Sanjeev; Kumar, Sheetanshu; Chatterji, Debajyoti; Saikia, Uma Nahar; De, Dipankar

doi:10.1111/ijd.16314

Cited by 4 publications

(1 citation statement)

References 12 publications

(27 reference statements)

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“…The occurrence of PV is even rarer in the pediatric population, ranging from 1 to 4% of reported cases [ 4 ]. We describe an interesting case of a 12-year-old boy with severe PV that was referred to the University Hospital of Parma, Parma, Italy, for a mucocutaneous disease resistant to anti-infective therapy.…”

Section: Introductionmentioning

confidence: 99%

Severe Pemphigus Vulgaris Resistant to Conventional Therapies and with Hypersensitivity to Rituximab in a 12-Year-Old Child

et al. 2023

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Pemphigus vulgaris (PV) is a rare, potentially lethal blistering disease typically occurring in adulthood and characterized by autoantibodies directed against mucocutaneous desmosomal proteins. Clinically, flaccid vesicles, bullae and erosions after breakage are the main clinical features. According to the literature, the incidence of PV is rare in the pediatric population, ranging from 1 to 4% of reported cases. We describe an interesting case of a 12-year-old boy with severe PV that was referred to our university hospital for a mucocutaneous disease resistant to anti-infective therapy. Following the appearance of bullous lesions on the skin, antibody screening for autoimmune diseases showed positivity for PV and corticosteroid therapy was started. In view of the numerous adverse effects, we decided to set up biological therapy with rituximab, which was interrupted due to the onset of an urticarial reaction. Further second-line therapies were therefore attempted, with only a partial response. For this reason, a desensitizing therapy with rituximab was decided, thus allowing a clear improvement in the clinical picture and quality of life of the patient. To the best of our knowledge, this is the first report of a child with severe PV resistant to conventional therapies and with an urticarial reaction to rituximab. This case highlights that despite PV being extremely rare in the pediatric population, this diagnosis should not be entirely discounted. In case of severe clinical manifestations, rituximab represents a valid option in children and desensitization tests should be recommended in the presence of hypersensitivity to this drug.

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Section: Introductionmentioning

confidence: 99%

Severe Pemphigus Vulgaris Resistant to Conventional Therapies and with Hypersensitivity to Rituximab in a 12-Year-Old Child

et al. 2023

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Diagnosis, treatment, and long‐term outcomes of pediatric pemphigus: a retrospective study at tertiary medical centers

Renert‐Yuval,

Baum,

Greenberger

et al. 2024

Int J Dermatology

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BackgroundPediatric pemphigus is a rare bullous disease that represents a diagnostic and therapeutic challenge; evidence on patients' response to various treatments and long‐term surveillance data are lacking. We aimed to investigate pediatric pemphigus patients' characteristics, diagnosis, therapeutics, response, and long‐term follow‐up.MethodsThis is a retrospective study of all pemphigus patients aged <18 years, diagnosed between 2000 and 2023, from three tertiary medical centers in Israel. The diagnosis was confirmed by positive immunofluorescence.ResultsTwelve pediatric pemphigus patients were included (mean age 10.7 ± 4.3 years, male:female ratio 1:1). Mean diagnostic delay was 11.1 ± 12.6 months (range 1.8–36 months). Most patients had pemphigus vulgaris with mucosal involvement (58.3%). First‐line treatment for all patients included systemic corticosteroids (sCS), with a treatment duration (including tapering down) of 28 ± 18.4 months. Hospitalization did not yield better outcomes. Only three patients achieved sustained complete response with sCS treatment (25.0%), and the rest required additional therapeutics, most commonly rituximab. Rituximab showed a good safety profile and therapeutic response. Follow‐up was recorded up to 18.1 years after diagnosis (mean: 5.6 years). Three of five patients with information available more than 5 years after the pemphigus diagnosis still exhibited disease symptoms.ConclusionsPediatric pemphigus is associated with a significant diagnostic delay. While sCS can induce remission in most patients as a first‐line treatment, long‐term disease control requires additional immunomodulators. Long‐term follow‐up reveals a chronic yet mostly benign disease course in this population and advocates for the use of rituximab in pediatric pemphigus patients.

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Rituximab in the treatment of severe childhood pemphigus vulgaris accompanying phenylketonuria: A case report

Avcı,

Akin,

Aydogan

et al. 2024

IJDVL

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Pediatric autoimmune blistering disorders – a five‐year demographic profile and therapy experience

Cited by 4 publications

References 12 publications

Severe Pemphigus Vulgaris Resistant to Conventional Therapies and with Hypersensitivity to Rituximab in a 12-Year-Old Child

Severe Pemphigus Vulgaris Resistant to Conventional Therapies and with Hypersensitivity to Rituximab in a 12-Year-Old Child

Diagnosis, treatment, and long‐term outcomes of pediatric pemphigus: a retrospective study at tertiary medical centers

Rituximab in the treatment of severe childhood pemphigus vulgaris accompanying phenylketonuria: A case report

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