Childhood pemphigus vulgaris: Report of two cases with emphasis on diagnostic approach

Surya, Varun; Kumar, Priya; Gupta, Sunita; Urs, Aadithya B

doi:10.4103/ccd.ccd_461_18

Cited by 9 publications

(29 citation statements)

References 15 publications

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“…Pemphigus Vulgaris (PV) is an uncommon autoimmune blistering disease involving the skin and mucous membranes. 1,2 Most patients with PV have circulating autoantibodies against desmogleins 1 and 3, which are transmembrane proteins of the desmosomes. The clinical disorder is a consequence of the loss of cell adhesion, which occurs due to the attack on transmembrane proteins.…”

Section: Introductionmentioning

confidence: 99%

“…There is no difference between PV and CPV concerning the diagnostic tests because CPV has similar clinical, histological, and immunological features seen in PV in adults. 2,3 Among bullous diseases in childhood, CPV is the least frequent entity and thus can be misdiagnosed. 2 The differential diagnosis of CPV includes (i) hand-footmouth disease, (ii) oral candidiasis, (iii) acute herpetic gingivostomatitis, (iv) erythema multiforme, (v) Behcet's disease, (vi) lichen planus, and (vii) recurrent aphthous stomatitis.…”

Section: Introductionmentioning

confidence: 99%

“…2,3 Among bullous diseases in childhood, CPV is the least frequent entity and thus can be misdiagnosed. 2 The differential diagnosis of CPV includes (i) hand-footmouth disease, (ii) oral candidiasis, (iii) acute herpetic gingivostomatitis, (iv) erythema multiforme, (v) Behcet's disease, (vi) lichen planus, and (vii) recurrent aphthous stomatitis. 5 Clinical presentation includes flaccid blisters that produce erosions after rupture.…”

Section: Introductionmentioning

confidence: 99%

“…Anamnesis should focus on duration, number of lesions, and recurrence of symptoms. 2 Ancillary diagnostic methods include biopsy with immunofluorescence (IF) investigation. 2 Direct immunofluorescence (DIF) identifies the presence of autoantibodies and complement fractions.…”

Section: Introductionmentioning

confidence: 99%

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Childhood pemphigus vulgaris is a challenging diagnosis

Filho¹,

Barbosa²,

Abreu

et al. 2021

Autops Case Rep

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Pemphigus Vulgaris (PV) is an uncommon autoimmune and blistering mucocutaneous disease. Childhood Pemphigus Vulgaris (CPV) is a pediatric variant of PV, which affects children below 12 years, being very rare among children under 10 years of age. CPV has similar clinical, histological, and immunological features as seen in PV in adults. The mucocutaneous clinical presentation is the most common in both age groups. Vesicles and erosions arising from the disease usually cause pain. A few CPV cases have been reported in the literature. This study reports a case of an 8-year-old male patient with oral lesions since the age of 3 years, and the diagnosis of pemphigus was achieved only 2 years after the appearance of the initial lesions. CPV remains a rare disease, making the diagnosis of this clinical case a challenge due to its age of onset and clinical features presented by the patient. Therefore, dentists and physicians should know how to differentiate CPV from other bullous autoimmune diseases more common in childhood.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Childhood pemphigus vulgaris is a challenging diagnosis

Filho¹,

Barbosa²,

Abreu

et al. 2021

Autops Case Rep

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“…7 PV is rarely found in children. 8 PV usually occurs in patients between 40 to 60 years of age, with a higher incidence in men. 4 In this case, it was found that the patient was 31 years old, with a chief complaint of new blisters from almost the entire body for two days before the examination.…”

Section: Discussionmentioning

confidence: 99%

Steroid-induced diabetes mellitus in pemphigus vulgaris patient at Bali Mandara Hospital: a case report

Aviana

Birawan²

2021

Bali Dermatology and Venereology Journal

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Background: Pemphigus vulgaris (PV) is a blistering autoimmune disease of the skin and mucous membranes defined histologically by intraepidermal blister due to acantholysis. Systemic corticosteroids and immunosuppressive agents had greatly improved the prognosis of pemphigus. However, steroid use often leads to metabolic complications, such as diabetes mellitus. This case report describes steroid-induced diabetes mellitus in PV, where the side effect of long-term high dosages steroid used and the method to manage it can be used as a study case.Case report: A 31-year-old man complained of new blisters from almost the entire body. He had a history of PV 1 year ago and no history of diabetes mellitus in the patient or family. He had a medication history of methylprednisolone 8 mg every 8 hours. Dermatological status showed erythema macules, extensive erosion almost on the entire body, the Nikolsky sign (+), and the Asboe-Hansen sign (+). Blood laboratory result: random blood glucose 451 mg/dl, HbA1c 12.3%. Histopathological examination: suprabasal blister, in which the basal cells still attached to the basement membrane show a “tombstone” appearance. The diagnosis was steroid-induced diabetes in pemphigus vulgaris. He was treated with steroids and insulin.Conclusion: Steroid use in PV treatment can lead to metabolic complications, such as diabetes mellitus. Regularly monitoring is needed to prevent complications due to steroid use.

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Juvenile pemphigus vulgaris: Literature review and a rare case report

Sriram,

Hasan,

Mansoori

et al. 2024

Clinical Case Reports

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Key Clinical MessagePemphigus vulgaris (PV) is a chronic autoimmune blistering disorder characterized by the loss of intraepithelial adhesion affecting the skin and mucous membranes, predominantly affects females in their fifth and sixth decades of life. Due to its rare occurrence in children and adolescents, there is often a delay in diagnosis and treatment in this age group. PV should always be considered in the differential diagnosis of oral ulcerative and vesiculobullous lesions in both children and adolescents.

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Childhood pemphigus vulgaris: Report of two cases with emphasis on diagnostic approach

Cited by 9 publications

References 15 publications

Childhood pemphigus vulgaris is a challenging diagnosis

Childhood pemphigus vulgaris is a challenging diagnosis

Steroid-induced diabetes mellitus in pemphigus vulgaris patient at Bali Mandara Hospital: a case report

Juvenile pemphigus vulgaris: Literature review and a rare case report

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