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Lupus tumidus is a rare subtype of chronic cutaneous lupus erythematosus that was first described by Gougerot and Bournier in 1930. Clinically, lupus tumidus presents as smooth, shiny, red-violet plaques of the head and neck that may be pruritic and have a fine scale. These lesions characteristically clear without scarring and recur in their original distribution. Histologic features include superficial and deep lymphohistiocytic infiltrates and abundant dermal deposits of mucin. We describe lupus tumidus as a distinct form of cutaneous lupus erythematosus and report 4 cases.

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A 44-year-old woman presented with a chronically draining lesion on her cheek just lateral to the nasofacial sulcus. The lesion was refractory to treatment with oral antibiotics. Physical examination revealed poor dentition, and a panoramic radiograph demonstrated periapical abscesses in the maxillary right lateral incisor and canine. A diagnosis of cutaneous fistula of odontogenic origin was made, and the patient was treated with tooth extraction. The cutaneous fistula subsequently resolved. Intraoral examinations and radiographs are critical for making the diagnosis of cutaneous draining sinus tract of odontogenic origin. Many patients undergo unnecessary surgical therapies before having the correct diagnosis made, but root canal therapy or surgical extraction is the treatment of choice. A dental origin must be considered for any chronically draining sinus of the face or neck.

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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...

Infantile perianal pyramidal protrusion is an important, relatively newly recognized condition, which is thought to be under-reported in the pediatric literature. Previous reports refer to these lesions as skin tags or skin folds. More recently, the name infantile perianal pyramidal protrusion has been suggested, based on the characteristic presentation of the lesion in prepubertal children, especially girls. Recognition of these lesions has many implications regarding proper diagnosis and management. They may easily be mistaken for condyloma accuminata or as a sign of trauma, leading to an investigation of sexual abuse. This situation may be avoided with awareness of this entity in combination with thorough history taking and a classic presentation. We present a classic presentation of infantile perianal pyramidal protrusion and further discuss this condition.