Marshall's syndrome.
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Marshall's syndrome is a form of acquired cutis laxa without systemic involvement, which is preceded by an inflammatory dermatitis with a neutrophilic component.We report a case of a 6-year-old boy with clinical and histopathological features of this syndrome.The etiology remains unknown and there is no definitive treatment.
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Affiliation: School of Medicine of Campos, Campos dos Goytacazes, (RJ), Brazil.
ABSTRACT
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Marshall's syndrome is a form of acquired cutis laxa without systemic involvement, which is preceded by an inflammatory dermatitis with a neutrophilic component. We report a case of a 6-year-old boy with clinical and histopathological features of this syndrome. The etiology remains unknown and there is no definitive treatment. Related in: MedlinePlus |
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f01: Clinic - Acute phase. Indurated well-circumscribed erythematous plaques on theback Mentions: A 6-year-old boy, phototype IV, from Rio de Janeiro, Brazil, arrived at the PediatricDermatology Service with infiltrated, well circumscribed erythematous and edematousplaques with polycyclic configuration, on his face, back and extremities for two months,associated with episodes of fever (Figures 1 and2). He had not taken any medication. Laboratorystudies revealed normal blood count values (except for a high eosinophils rate - 7%).Serological reactions for syphilis (VDRL), HIV and baciloscopy were negative. Serumα1antitrypsin was not available. A biopsy specimen obtained from one of the infiltratedplaques showed a neutrophilic disease with edema of the dermal papillae and denseneutrophilic dermal infiltrate (Figure 3).Treatment with Dapsone (1mg/Kg/day) and Prednisone (0.5mg/Kg/day) was initiated. Allinflammatory plaques resolved slowly and left atrophic, wrinkled areas, causing apremature aging appearance (Figure 4). A biopsyspecimen of the atrophic cutaneous areas showed decreased thickness of the reticulardermis. Acid orcein staining showed marked decrease in the number of elastic fibers inthe reticular dermis. The existing fibers were shortened and fragmented, but collagenappeared normal (Figure 5). The patient evolvedwith other recurrent episodes and, as a result, new wrinkled areas every time he failedto follow treatment recommendations. |
View Article: PubMed Central - PubMed
Affiliation: School of Medicine of Campos, Campos dos Goytacazes, (RJ), Brazil.