Clinical and serologic expression of localized scleroderma. Case report and review of the literature.
A patient is presented in whom both linear scleroderma and eosinophilic fasciitis developed. The latter is rare in childhood. A positive antinuclear antibody, a positive rheumatoid factor, and seizures also developed. In reviewing the literature, we concluded that the clinical and histologic pattern of cutaneous sclerosis may be more valuable than serologic findings in predicting both the likelihood of, and the expected sites for, systemic involvement. The literature also suggests that central nervous system (CNS) involvement is rare in most sclerosing syndromes but that linear scleroderma may be associated with CNS or spinal disease. The ipsilateral association of linear scleroderma and seizure focus in this patient, as well as the focal onset of these seizures, is unusual and suggests an association between the seizure disorder and the linear cutaneous sclerosis.
D000293:Adolescent; D000974:Antibodies, Antinuclear / Q000032:analysis; D001706:Biopsy; D004802:Eosinophilia / Q000150:complications / Q000175:diagnosis*; D005208:Fasciitis / Q000150:complications / Q000175:diagnosis*; D005260:Female; D006801:Humans; D012217:Rheumatoid Factor / Q000032:analysis; D012594:Scleroderma, Localized / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D012640:Seizures / Q000150:complications; D012867:Skin / Q000473:pathology
D000974:Antibodies, Antinuclear; D012217:Rheumatoid Factor