Childhood Pemphigus Vulgaris Research Articles

Childhood Pemphigus Vulgaris during COVID-19 Outbreak Successfully Treated with Prednisone and Azathioprine: A Case Report and Literature Review.

pemphigus vulgaris is a rare autoimmune blistering disease that involves the skin and mucous membranes and rarely occurs in pediatric age. we present a case of childhood pemphigus in a 9-year-old patient from Burkina Faso, which initially manifested with erosive lesions symmetrically distributed in the oral cavity. After a few months, we also observed hyperchromic lesions of the back. Histopathological examination of skin samples showed intraepidermal acantholysis, while direct immunofluorescence showed deposits of complement (C3) and immunoglobulins G (IgG) in the epidermidis; an ELISA test highlighted the presence of circulating autoantibodies against desmoglein 3. the follow-up of this patient was made difficult by the advent of the COVID-19 outbreak. However, after about one year of combined therapy with systemic steroids and azathioprine the patient reached clinical remission.

Childhood pemphigus vulgaris is a challenging diagnosis

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.

CHILDHOOD PEMPHIGUS VULGARIS WITH UNUSUAL AND AGGRESSIVE CLINICAL PRESENTATION

Childhood pemphigus vulgaris (CPV) is an uncommon disease involving the skin and mucous membranes, and few cases of CPV have been reported. This study reports a case of a 5-year-old male patient with oral lesions since he had 3 years old. In the clinical examination, he presented buccal opening limitation, hoarseness, healed lesions in the skin, and painless ulcers in oral, genital, and ocular mucosa. The oral lesions involved lips, labial mucosa, tongue, palate, oral commissures, lower alveolar mucosa, buccal mucosa, and gum. Posteriorly, videolaryngoscopy showed lesions in the pharynx and larynx. An incisional biopsy and direct immunofluorescence from a sample of the lower lip mucosa was performed, which confirmed the diagnosis of CPV. The patient was treated with systemic medication, which improved his clinical conditional. However, the lesions remain in labial mucosa and ventral surface of the tongue. The patient has a clinical follow-up of 2 years. Childhood pemphigus vulgaris (CPV) is an uncommon disease involving the skin and mucous membranes, and few cases of CPV have been reported. This study reports a case of a 5-year-old male patient with oral lesions since he had 3 years old. In the clinical examination, he presented buccal opening limitation, hoarseness, healed lesions in the skin, and painless ulcers in oral, genital, and ocular mucosa. The oral lesions involved lips, labial mucosa, tongue, palate, oral commissures, lower alveolar mucosa, buccal mucosa, and gum. Posteriorly, videolaryngoscopy showed lesions in the pharynx and larynx. An incisional biopsy and direct immunofluorescence from a sample of the lower lip mucosa was performed, which confirmed the diagnosis of CPV. The patient was treated with systemic medication, which improved his clinical conditional. However, the lesions remain in labial mucosa and ventral surface of the tongue. The patient has a clinical follow-up of 2 years.

Vaccine–induced childhood pemphigus vulgaris: A case report in a 5-year-old female child

Pemphigus vulgaris (PV) is an immunobullous disease affecting the skin and mucosa. It is a disease most commonly manifesting in the third–fifth decades of life. Pediatric PV is rare and usually seen at a mean age of 12 years. Early childhood PV is extremely rare with only few cases reported. There are various inducers of PV in children. Diphtheria toxoid (DT) has rarely been reported as an inducer of PV. We report a 5-year-old female child who developed vesiculobullous mucocutaneous lesions after DT vaccination. The diagnosis was confirmed by histopathology and immunofluorescence. She was treated with pulse methylprednisolone therapy and daily dose azathioprine.

Childhood pemphigus vulgaris successfully treated with rituximab

Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Rituximab is a novel therapy for the treatment of refractory pemphigus. However, there is limited clinical data on safety and efficacy of rituximab in pediatric age group. Herein, we report an 11-year-old boy of childhood pemphigus vulgaris who failed to respond to dexamethasone pulse therapy and was subsequently treated with rituximab and achieved complete remission.

Analysis of Current Therapy and Clinical Outcome in Childhood Pemphigus Vulgaris

Pemphigus vulgaris (PV) is a rare, potentially fatal chronic autoimmune disease of the skin and mucous membrane. The objective of this study was to analyze the clinical outcomes and side effects associated with treatment of childhood PV (CPV). A retrospective review of the English language literature was conducted through PUBMED using the words childhood pemphigus vulgaris, and treatment or clinical outcome. Only patients under 12 years of age were included. Thirty-three cases were found in 29 reports. Mean age at onset was 8.3 years (range 1.5-12 yrs). Mucosal involvement (97.0%) was more common than cutaneous involvement (84.8%). Oral mucosa was the most common site of mucosal involvement (93.9%), followed by genital (20.6%), ocular (11.8%), and nasal mucosa (2.9%). Mean duration of therapy was 4.5 years (range 0.6-14.5 yrs), and mean duration of follow-up was 5.2 years (range 0.6-16 yrs). Complete recovery with no further therapy was achieved in 18.2% and partial recovery with minor relapses while on maintenance therapy in 78.8%. One patient died due to infection (3.0%). Serious side effects were present in 60.6%. The most common were cushingoid features (65.0%), growth retardation (50.0%), and infection (50.0%). Two patients who were refractory to systemic corticosteroids and immunosuppressive agents (ISA) had a favorable clinical response to Rituximab. Current therapy for CPV involving the use of long-term systemic corticosteroids in conjunction with ISA results in prolonged immunosuppression, causing systemic infections and growth retardation. Safer and more effective therapies need to be explored.

Childhood pemphigus vulgaris: Report of a case with a long-term follow-up and successful response to rituximab

Childhood pemphigus vulgaris: Report of a case with a long-term follow-up and successful response to rituximab

Childhood pemphigus

Childhood pemphigus is a rare diasease and comprises only a minority of immunobullous diseases seen in children. Almost all variants of pemphigus in childhood have been described in literature. Pemphigus vulagaris is the most common type seen, after excluding endemic foliaceus. The clinical features are essentially similar to that of adults. Special consideration is required while treating children so as to use drugs which are less carcinogenic and do not affect fertility. We present here a review of cases of childhood pemphigus vulgaris, pemphigus foliaceus, pemphigus vegetans, pemphigus erythematosus, paraneoplastic pemphigus, IgA pemphigus and neonatal pemphigus.

Successful Treatment of Refractory Childhood Pemphgus Vulgaris with Anti‐CD20 Monoclonal Antibody (Rituximab)

Pemphigus vulgaris is an uncommon autoimmune blistering skin disorder that is particularly rare in children. Immunosuppressive treatment can be challenging. Rituximab (anti-CD20 monoclonal antibody) has been used to treat autoimmune disorders by depletion of CD20 B cells. Successful rituximab therapy has been reported in adults with refractory pemphigus vulgaris. We present a girl with childhood pemphigus vulgaris unresponsive to treatment with azathioprine, mycophenolate mofetil, plasmapheresis, and intravenous immunoglobulin with systemic prednisone who responded to treatment with rituximab. She had a corresponding decline in circulating antibodies against desmoglein 1 and 3 and a decline in diphtheria and tetanus-specific antibody titers.

Childhood, neonatal, and stillborn pemphigus vulgaris.

Childhood, neonatal, and stillborn cases of pemphigus vulgaris were reviewed. From an examination of the pemphigus vulgaris literature, 46 childhood cases, nine neonatal cases, and three stillborn cases were found and investigated. In the childhood cases, the ratio between the sexes is approximately the same. The mean age of onset is 12 years, with only 11 children being 10 years of age or younger. Some children were treated with adjuvants to corticosteroids, most of them with azathioprine. While only one childhood case has been reported as fatal, the long-term prognosis and the relationship of early treatment and outcome are unknown. Neonatal prognosis, however, is excellent. Both neonatal and stillborn cases are probably the result of transplacental transmission of maternal antibodies. The connection between maternal antibody titer and fetal mortality is unknown. All stillborn cases reviewed died during the eight month of gestation. Immunosuppressive treatment probably affects fetal survival. In women with an active disease, who have had the disease, or who are monozygotic siblings to such patients, the possibility of the fetus developing the disease must be considered. It is essential that physicians be aware of the existence of childhood pemphigus vulgaris in order to make an early diagnosis and to avoid treatment delay. More childhood case reports are needed to obtain better information on optimal treatment, and authors should be encouraged to report the follow-up of their cases.

Juvenile pemphigus vulgaris

Although pemphigus vulgaris is uncommon in adolescence, its occurrence in two patients in this age group is reported. In both cases the lesions were confined to the oral mucosa and the diagnosis was established by the typical histologic picture and positive direct and indirect immunofluorescence. The literature on childhood pemphigus vulgaris is reviewed, and the importance of prompt diagnosis by early biopsy and supportive immunofluorescent tests is emphasized.

Childhood pemphigus vulgaris.

CHILDHOOD pemphigus vulgaris, although rare, can be as virulent as its adult counterpart.1 The case described below revealed several previously unemphasized features, including the importance of early mucous-membrane biopsy and the efficacy of alternate-day steroids.Case ReportA 13-year-old Negro girl was well until 14 weeks before admission when she noted "sores" on her gums that gradually spread to involve the entire oral cavity and lips until progressive dysphagia and hoarseness necessitated hospitalization. Past medical history, developmental history, and review of systems were unremarkable.Examination revealed widespread superficial erosions, with a positive Nikolsky sign, on the gums, tongue, oropharyngeal surfaces, . . .

Pemphigus vulgaris in childhood.

Pemphigus vulgaris rarely occurs in children. We present the youngest reported case. All six cases of childhood pemphigus vulgaris proven by biopsy began with lesions confined to the oral mucosa. In each case diagnosis was delayed until the more typical cutaneous lesions began to appear. Either mucosal or cutaneous biopsy specimens are diagnostic, and early biopsy of erosive mucosal lesions is indicated. Vigorous therapy with systemic steroids is important in the prevention of fatality.