Marshall's syndrome
A 6-year-old boy, phototype IV, from Rio de Janeiro, Brazil, arrived at the Pediatric\nDermatology Service with infiltrated, well circumscribed erythematous and edematous\nplaques with polycyclic configuration, on his face, back and extremities for two months,\nassociated with episodes of fever ( and\n). He had not taken any medication. Laboratory\nstudies revealed normal blood count values (except for a high eosinophils rate - 7%).\nSerological reactions for syphilis (VDRL), HIV and baciloscopy were negative. Serum\nα1antitrypsin was not available. A biopsy specimen obtained from one of the infiltrated\nplaques showed a neutrophilic disease with edema of the dermal papillae and dense\nneutrophilic dermal infiltrate ().\nTreatment with Dapsone (1mg/Kg/day) and Prednisone (0.5mg/Kg/day) was initiated. All\ninflammatory plaques resolved slowly and left atrophic, wrinkled areas, causing a\npremature aging appearance (). A biopsy\nspecimen of the atrophic cutaneous areas showed decreased thickness of the reticular\ndermis. Acid orcein staining showed marked decrease in the number of elastic fibers in\nthe reticular dermis. The existing fibers were shortened and fragmented, but collagen\nappeared normal (). The patient evolved\nwith other recurrent episodes and, as a result, new wrinkled areas every time he failed\nto follow treatment recommendations.