Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Atopic keratoconjunctivitis (AKC) is a potentially blinding disease characterized by a bilateral chronic keratoconjunctivitis associated with atopic dermatitis. The disease usually manifests as severe itching and burning, excessive tearing, foreign body sensation, and mucoid discharge. The clinical characteristics of AKC show a broad spectrum including lid dermatitis, chronic blepharitis, cicatrizing conjunctivitis with fornix foreshortening and symblepharon formation, punctate epithelial keratitis, persistent epithelial defects, corneal scarring and neovascularization, lipid keratopathy, conjunctivalization of peripheral cornea, and peripheral ulcerative keratitis. The underlying pathophysiologic mechanism in AKC involves a combination of type-I IgE-mediated, and type-IV delayed hypersensitivity reactions. The immunoregulatory defect responsible for the overproduction of allergen-specific IgE antibody, the key component responsible for antigen binding, and subsequent mast cell degranulation, is probably multifactorial. The histopathologic characteristics of the conjunctiva in AKC include a mast cell and eosinophil invasion of the epithelium, epithelial pseudotubule formation, and prominent mast cell and mononuclear cell infiltration of the substantia propria. A number of ocular conditions have been reported to be associated with AKC, including keratoconus, herpes simplex keratitis, and cataracts. Successful long-term control of this potentially blinding disease requires a multidisciplinary approach involving systemic and environmental aspects. Scrupulous long-term environmental control of allergens is the single most important aspect in the management of patients with AKC. Systemic anti-histamine therapy, and long-term topical mast cell stabilizing therapy are also mandatory. Topical steroids should be reserved for exacerbations of the disease.
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PMID:Atopic keratoconjunctivitis. 2282 13

Atopic keratoconjunctivitis (AKC) is the most severe type of allergic conjunctivitis and may eventually lead to blindness. Although AKC is reported to be more prevalent in adults, we report a child with AKC whose clinical characteristics were not inconsistent with those typically seen in adult patients with AKC, and who was refractory to traditional topical anti-inflammatory and immunosuppressant therapies. An 11-year-old boy presented with a 3-month history of ocular redness and itching and decreased vision for a week in both eyes. Slit-lamp examination revealed typical signs of vernal keratoconjunctivitis, including cobblestone papillae in both upper conjunctiva, superficial punctate keratopathy on the right cornea, and a sterile shield-shaped ulcer on the left cornea. Physical examination revealed eczematous lid changes and a generalized body rash, particularly on the face, neck, and flexor surfaces of the limbs. He was diagnosed to have AKC in both eyes and atopic dermatitis. The patient did not respond well to conventional topical antihistamine, mast cell stabilizers, corticosteroids, or tacrolimus, even in combination with amniotic membrane transplant. After using systemic immunosuppressants, the symptoms were relieved; the inflammation on the skin and ocular surface subsided, the cobblestone papillae disappeared, and the corneal ulcer healed gradually within 8 weeks. This case reveals that pediatric AKC should be differentiated from vernal keratoconjunctivitis because both disorders include upper cobblestone papillae, but the former is accompanied by atopic dermatitis. Pediatric AKC requires appropriate and aggressive treatment to prevent sight-threatening corneal complications. Systemic immunosuppressant should be considered when traditional topical anti-inflammatory therapies have failed.
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PMID:Recalcitrant Atopic Keratoconjunctivitis in Children: A Case Report and Literature Review. 2961 Jan 74