OBJECTIVE: The purpose of the study was to report the ocular manifestations and immunohistopathologic description of two rare forms of genodermatosis: @entity7063 ( @entity7063 ) and @entity57969 ( @entity57969 ). DESIGN: Case reports. @entity1 : Two @entity1 with @entity7063 and @entity57969 presenting with @entity102 were studied. INTERVENTION: Clinical photography to show ocular manifestations and dermatologic findings was performed. Conjunctival biopsy was performed to determine the histopathology and immunohistopathology. RESULTS: In the @entity1 with @entity57969 , the eyelid skin was dry, erythematous, and thickened. The lid margins showed plugging of some of the Meibomian glands, madarosis, and occasional @entity5055 . The epibulbar conjunctiva was injected and had a moderate papillary reaction. Both corneas had pronounced vascularization and stromal scarring. Conjunctival biopsy results disclosed a striking number of degranulating mast cells and moderate numbers of plasma cells. Immunohistopathology showed pronounced immunoglobulin G (IgG)-positive cells in the stroma and immunoglobulin A (IgA) positivity in the epithelium. There was scattered immunoglobulin M (IgM) and moderate C3 and C4 positivity in the stroma. In @entity7063 , the @entity1 had keratinized lid margins, @entity4548 formation, @entity5055 , cicatrizing conjunctivitis, and symblephra formation. The corneal surface was keratinized, with deep and superficial vascularization. Conjunctival biopsy specimens showed pronounced epithelial keratinization and @entity1544 . Degranulating mast cells and eosinophils were prominent in the stroma. Immunohistopathology showed C4 and immunoglobulin D positivity on the keratinized epithelial surface with rare foci of immunoglobulin E-positive cells. Basal epithelial cells were positive for IgA and IgG, and a large number of IgA and IgG plasma cells were present in the substantia propria. CONCLUSION: To the authors' knowledge, these case reports represent the first clinical description of the ocular manifestation of @entity57969 and the first immunohistopathologic description of the affected conjunctiva in @entity57969 and @entity7063 , both of which should be considered in the differential diagnosis of genodermatosis associated with @entity102 .

XXXX associated with @entity7063 and erythrokeratodermia variablis. Two rare genodermatoses.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient', 'PARTICIPANTS'] @entity7063 :: ('MESH:D019871', 'Disease') :: ['CD', 'congenital dyskeratosis'] @entity102 :: ('MESH:D002908', 'Disease') :: ['chronic keratoconjunctivitis'] @entity1544 :: ('MESH:D018307', 'Disease') :: ['squamous metaplasia'] @entity4548 :: ('MESH:D004774', 'Disease') :: ['entropion'] @entity5055 :: ('MESH:D058457', 'Disease') :: ['trichiasis'] @entity57969 :: ('MESH:D056266', 'Disease') :: ['erythrokeratodermia variabilis', 'EKV']
@entity102 :: (MESH:D002908,Disease) :: ['Chronic keratoconjunctivitis']
