case_images: PMC3017945_01
case_images: Patient with multiple dermatologic lesions distributed in limbs (a) and back (b).
case_images: JOP2010-280396.001.jpg
case_images: PMC3017945_01_JOP2010-280396.001.jpg
case_images: fig1
case_images: He presented bullous and papular lesions with erythematous base in periocular tissues, limbs, thorax (Figure 1).
case_images: Right eye (a) and left eye (c) showing subconjunctival fibrosis, corneal opacities and vascularization as well epithelial defects. Right eye upper tarsal conjunctiva with fibrosis (b) and left eye (d) lower tarsal conjunctiva with subepithelial fibrosis.
case_images: JOP2010-280396.002.jpg
case_images: PMC3017945_01_JOP2010-280396.002.jpg
case_images: fig2
case_images: Slit lamp examination showed dysfunctional meibomian glands, conjunctival hyperemia, subconjcuntival fibrosis, corneal superficial vascularization, and opacification with epithelial defects, and the rest of the anterior segment was difficult to evaluate (Figure 2).
case_images: Immunofluorescence showing skin (A). Affected cornea (B) showing IgA deposits along the basal membrane and Bowman's layer (arrow) with inflammatory infiltrates (asterisk) and blood vessels (arrowheads). Comparison with healthy cornea tissue (C) showing intact Bowman's layer (arrow) and stroma (S). (Micrograph A, 400X; B, 100X, counterstain Propidium iodide).
case_images: JOP2010-280396.003.jpg
case_images: PMC3017945_01_JOP2010-280396.003.jpg
case_images: fig3
case_images: A histopathological examination of the skin biopsy revealed neutrophil alignment along basal membrane and subepidermal cleavage with inflammatory cells in the superficial dermis; immunofluorescence confirmed IgA deposits along the basal membrane (Figure 3(a)). We observed a complete destruction of Bowman's layer, replaced by fibrotic tissue with perforant vessels, and immunofluorescence studies confirmed IgA deposits over this area (Figure 3(b)).
case_images: Right eye ultrasound B-scan with both retinal (asterisks) and choroidal (arrow) detachments.
case_images: JOP2010-280396.004.jpg
case_images: PMC3017945_01_JOP2010-280396.004.jpg
case_images: fig4
case_images: Unfortunately he persisted with torpid evolution and the right eye visual acuity displayed LP without color discrimination and the ultrasound showed retinal and choroidal detachments (Figure 4).
case_images: Dermatological lesions improved after treatment (a). Right eye (b) and left eye (c) ocular surface photographs after initial IVIG treatment.
case_images: JOP2010-280396.005.jpg
case_images: PMC3017945_01_JOP2010-280396.005.jpg
case_images: fig5
case_images: Although dermatologic lesions improved significantly after IVIG therapy (Figure 5(a)), unyielding, severe ocular surface disease persisted (Figures 5(b) and 5(c)).