Childhood pemphigus vulgaris is a challenging diagnosis

Abstract

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.

Juvenile pemphigus vulgaris: Literature review and a rare case report

“…Juvenile/adolescent PV affects individuals between the ages of 12 and 18 years 9,22,29,33 . Our findings are in coherence with the published literature.…”

“…The recommended dose of prednisolone is 0.5–1 mg/kg, which is gradually tapered to the lowest effective dose to minimize any associated adverse effects 9,19,26,34 . The steroid dosage may be modified based on the patient's clinical outcome, with gradual tapering in patients showing substantial resolution 19,33 . Oral PV cases may be successfully treated with a short course of low‐dose corticosteroids without the need for additional therapy, as was the case with our patient 26 .…”

“…Biopsies from intact vesicles/blisters yield more accurate results, but are infrequently seen as they tend to rupture easily. 26,33 Lesional skin samples were used for histopathological examination, and perilesional skin samples were used for direct immunofluorescence to detect tissue-bound autoantibodies. 37 Acantholysis and blister/vesicle formation within the epithelial layer, just above the basal layer (suprabasilar split), are the characteristic histological features of PV.…”

“…PV exhibits site predilection that commonly affects the skin and mucous membranes of the oral cavity, genitals, and conjunctiva. Pediatric PV is associated with a higher occurrence of genital and ocular lesions than adult PV 33 . Moreover, the clinical progression of pediatric PV is often more unpredictable than that of adult PV, 2 although it is generally considered to be better than that in adults 19 …”

“…Pediatric PV is associated with a higher occurrence of genital and ocular lesions than adult PV. 33 Moreover, the clinical progression of pediatric PV is often more unpredictable than that of adult PV, 2 although it is generally considered to be better than that in adults. 19 Our patient presented with exclusive erosive oral lesions at multiple sites.…”