Paraneoplastic Autoimmune Multiorgan Syndrome (Paraneoplastic Pemphigus) in a Child: Case Report and Review of the Literature

Abstract

ABSTRACT. Paraneoplastic autoimmune multiorgan syndrome, also known as paraneoplastic pemphigus, has been observed only rarely among children. We describe a 10-year-old boy with typical clinical and histologic findings of paraneoplastic pemphigus associated with Castleman's disease. His disease was refractory to resection of the tumor and aggressive combination immunosuppressive therapies. The patient died 1 year after presentation, as a result of complications of bronchiolitis obliterans. This case is unusual because of the young age of the patient. Pediatrics 2004;114:e513-e516. URL: www. pediatrics.org/cgi/doi/10.1542/peds.2004-0436; paraneoplastic pemphigus, paraneoplastic autoimmune multiorgan syndrome, child, Castleman's tumor.ABBREVIATIONS. PAMS, paraneoplastic autoimmune multiorgan syndrome; CT, computed tomography; C3, complement component 3; Ig, immunoglobulin. P araneoplastic autoimmune multiorgan syndrome (PAMS) (paraneoplastic pemphigus) encompasses a multitude of mucocutaneous and systemic findings. Mucocutaneous involvement in paraneoplastic pemphigus is prominent, with painful erosive lesions involving the oral, nasal, upper gastrointestinal, respiratory, ocular, and genital epithelium. 1 Cutaneous manifestations are variable and often consist of polymorphous inflammatory macules, papules, and plaques. Histopathologic examinations classically reveal acantholysis, intraepidermal blister formation, and immunoreactant deposition along the basement membrane and within epithelial intercellular spaces. Associated neoplasia is a requisite finding of PAMS but may be occult at the onset of cutaneous lesions. Patients with PAMS frequently have significant pulmonary involvement, which accounts for the high mortality rate.The occurrence of PAMS in childhood has been reported infrequently. When PAMS occurs among children, however, Castleman's disease is the most common underlying neoplasm, with progressive bronchiolitis obliterans resulting in pulmonary destruction and death. We report the case of a 10-yearold child who developed a diffuse mucocutaneous eruption that was clinically and immunopathologically consistent with PAMS. Systemic evaluation revealed a retroperitoneal Castleman's tumor. CASE REPORTA 10-year-old, previously healthy, white boy presented with a 2-month history of a papular eruption of the trunk, conjunctivitis, erosive stomatitis, proximal nail fold inflammation, and nail dystrophy. Prior evaluation included a positive mycoplasma titer and a skin biopsy that was interpreted as indicating erythema multiforme/Stevens-Johnson syndrome. The mucocutaneous lesions were resistant to systemic corticosteroid treatment. The patient was referred to our institution because of progressive symptoms.System review results included fever, cough for 6 to 8 weeks, fatigue, constipation, and weight loss, with poor food intake attributable to painful oral ulcers. The physical examination revealed a thin boy with perioral cyanosis and resting tachypnea. A facial examination revealed bilateral exudative bulbar a...

Bullous diseases: Kids are not just little people

“…( 73 The stomatitis may be initially mistaken for HSV or StevensJohnson Syndrome (SJS) and a paraneoplastic workup may not initially be considered especially in pediatric patients. 74 The course can be complicated by bronchiolitis obliterans, respiratory failure, and death.…”

“…Progression and death occurred despite treatment of the underlying malignancy or with immunosuppressive therapy for PNP. Unlike adults, they found that the skin findings in the pediatric patients tended to be more lichenoid and less blistering.Treatment of PNP includes treatment of the malignancy and palliative immunosuppression which canlead to remission of the blistering disease, butoften in this ominousdisease the ultimate prognosis remains poor 73,76,80,81. Although no evidence based treatment recommendations are available, there is a report suggesting that early recognition and removal of a Castleman's tumor as well as peri-operative IVIG may mitigate the risk of subsequent bronchiolitis obliterans.…”

Paraneoplastic pemphigus: A trait d’union between dermatology and oncology

“…Ogawa et al reported that the mean age of onset in his series was 64.7 [7] . However, PNP can affect every age group, including children and adolescents [8][9][10] . PNP appears to affect males and females equally [2] .…”

“…In contrast, if cell-mediated cytotoxicity is the leading mechanism, lichenoid lesions might be easily seen [33,35,36] . Lichenoid lesions are commonly detected in children, especially on the trunk and limbs [9,10] . Lesions resembling those of pemphigoid are usually present on the extremities [45] .…”

“…PNP and underlying malignancy do not have a parallel evolution [4][5][6] . In fact, PNP lesions generally progress even if malignancy is removed or under controlled [8][9][10][11] .…”