PMC3017945_01
Patient with multiple dermatologic lesions distributed in limbs (a) and back (b).
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fig1
He presented bullous and papular lesions with erythematous base in periocular tissues, limbs, thorax (Figure 1).
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.
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fig2
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).
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).
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fig3
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)).
Right eye ultrasound B-scan with both retinal (asterisks) and choroidal (arrow) detachments.
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fig4
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).
Dermatological lesions improved after treatment (a). Right eye (b) and left eye (c) ocular surface photographs after initial IVIG treatment.
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fig5
Although dermatologic lesions improved significantly after IVIG therapy (Figure 5(a)), unyielding, severe ocular surface disease persisted (Figures 5(b) and 5(c)).