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...
The diagnosis of tibialis anterior herniation should be considered in patients with positionally variable subcutaneous nodules, especially in the lower extremities. It is caused by a defect in the deep fascial layer and often involves the lower extremities. In addition, knowledge of the diagnosis and treatment is essential for both proper management and/or surgical referral.
Erythema elevatum diutinum (EED) is a rare and chronic cutaneous leukocytoclastic vasculitis. It is predominantly seen on the extensor surfaces of the extremities. Although a specific cause is largely unknown, EED has been noted to occur in association with a wide variety of diseases. A 28-year-old man with systemic lupus erythematosus (SLE) and a 53-year-old woman with an overlap syndrome of rheumatoid arthritis and polymyositis are presented. Both patients developed EED in the setting of chronic recurrent bacterial infections. Patients with a connective tissue disease are at increased risk for such infections secondary to immunosuppression, either from the disease itself or secondary to immunosuppressive therapy. EED has been independently reported to occur in the setting of connective tissue disease as well as in the setting of chronic infection. Our patients had both of these underlying conditions, which are known to predispose patients to immune complex-mediated vasculitides, in this case EED. One patient's EED responded to treatment of the SLE and the other improved, as has been previously reported with dapsone.
background. Tibialis anterior herniation occurs through a defect in the deep fascial layer of the lower extremity. While this entity is not uncommon, it has been rarely reported in the dermatologic literature. objective. To highlight the occurrence of tibialis anterior herniation and its presentation and treatment. methods. Case report. results. We present a case of tibialis anterior herniation in a patient who presented with asymptomatic nodules in her lower Figure 1. Flesh-colored, soft, subcutaneous nodule that is accentuated with dorsiflexion of the foot.
Figure 1. Pseudoainhum of the fourth fingers of the hands.
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