Abstract
ABSTRACTPemphigus 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.
Highlights
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
There is no difference between PV and childhood PV (CPV) concerning the diagnostic tests because CPV has similar clinical, histological, and immunological features seen in PV in adults.[2,3]
CPV diagnosis is still challenging due to the varied clinical features and the clinical similarity to other pediatric autoimmune bullous diseases
Summary
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. An 8-years-old black male patient sought care in the stomatology clinic after looking for medical care without a definitive diagnosis and therapeutic success His mother reported that the initial lesions appeared at the age of 3 years, initially in the tongue, in other oral cavity areas. Only oral lesions remained, but the clinical condition worsened the month, and lesions in the genital mucosa relapsed At this moment, prednisone (1mg/kg/day) and dapsone (1 mg/kg/day) were again prescribed until achieving clinical regression of genital lesions. When the patient completed one year and three months of treatment, the initial treatment, prednisone was gradually tapered to a maintenance dose of 12.5 mg daily, and dapsone was withdrawn due to side effects At this moment, infection was diagnosed in the oral cavity and other sites of the patient’s body. The patient currently completed a clinical follow-up of 2 years and 10 months, and his clinical status substantially improved
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