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1/6. Linear IgA disease.

    PURPOSE: A case of linear IgA disease is reported to alert ophthalmologists and physicians to this unusual cause of chronic cicatrizing conjunctivitis. methods: Clinical records of a patient suffering from linear IgA disease were reviewed. RESULTS: A 65-year-old woman with a complicated medical history experienced rapidly progressive chronic cicatrizing conjunctivitis leading to corneal perforation. Undiagnosed gingivitis and palatal ulceration had been present for 5 years prior to the onset of ocular symptoms and vitamin C deficiency had followed the consequent dietary restrictions. A diagnosis of linear IgA disease was made on conjunctival biopsy, which demonstrated linear deposits of IgA along the epithelial basement membrane. The perforation was managed successfully with a conjunctival pediculate flap. Control of the inflammation was achieved with systemic prednisolone and cyclophosphamide but at the expense of serious systemic side-effects. CONCLUSIONS: Linear IgA disease causes progressive conjunctival cicatrization in many affected individuals.Although dapsone generally controls the inflammation, heavier systemic immunosuppression was required in this case. Involvement of skin or other mucosal surfaces may become symptomatic before the conjunctivitis, and physicians must be educated to refer patients for ophthalmological review on diagnosis. Conversely, ophthalmologists encountering ocular linear IgA disease should be aware of the possibility of other mucosal involvement requiring physician intervention.
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2/6. erythema exsudativum multiforme induced by granulocyte colony-stimulating factor in an allogeneic peripheral blood stem cell donor.

    We describe a healthy peripheral blood stem cell (PBSC) donor who developed a cutaneous reaction, erythema exsudativum multiforme, during the administration of granulocyte colony-stimulating factor (G-CSF) for mobilization. The cutaneous lesions were located on his hips, apart from the site of G-CSF injection. Treatment with topical corticosteroid was commenced, and the lesions resolved completely within a week. Adverse cutaneous reactions induced by G-CSF have been reported infrequently in healthy donors. Further documentation of cases and their full evaluation will be of great importance for both physicians and PBSC donor.
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3/6. Vesiculobullous eruption from intense pulsed light treatment.

    BACKGROUND: Intense pulsed light (IPL) systems emit non-coherent, polychromatic light and are increasingly used for various dermatologic indications. Although generally regarded as safe therapy, IPL is not without risk. OBJECTIVE: We report a 21-year-old woman who experienced a severe blistering eruption after IPL treatment by a nonphysician. MATERIALS AND methods: Case report. RESULTS: The patient reported a 10-year history of persistent redness on her medial and lateral cheeks. She had tried no previous oral or topical therapies. She was diagnosed with rosacea and was treated with IPL. The following day, intense vesiculation and bullae formation occurred, progressing to dramatic facial edema by day 3 and eschar formation by day 5. Ten weeks later, prominent erythema with papularity remained on both cheeks and the lateral neck, with textural change and reticulated dyschromia. She continued to recover 20 weeks after treatment. CONCLUSION: This case most likely represents high-fluence photothermal tissue injury induced by the laser-like qualities of the IPL source, with resulting acantholysis leading to formation of vesicobullae in the context of an uncertain primary diagnosis. This case highlights the potential hazards of IPL therapy and raises questions regarding appropriate use of this medical technology.
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4/6. Upper aerodigestive tract manifestations of cicatricial pemphigoid.

    Cicatricial pemphigoid is a chronic mucosal blistering disorder with a predilection for subsequent scar formation. Many physicians may be unaware of the various presentations and sequelae of this uncommon disease. This report of the largest series to date focuses on the upper aerodigestive tract manifestations of this disease. During the years 1975 to 1985, 142 patients with cicatricial pemphigoid were seen at the Mayo Clinic. There were 93 women and 49 men; the age range was 21 to 92 years. Mucosal lesions occurred most often in the mucous membranes of the oral cavity and conjunctiva. Involvement of the pharynx, larynx, and esophagus was less common. Stenosis of the nasopharynx or larynx necessitated surgical repair in several persons and caused obstructive sleep apnea in two. The otolaryngologist can make an important contribution to the early recognition, diagnosis, and management of the complications of cicatricial pemphigoid.
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5/6. Herpes gestationis.

    Herpes gestationis is a rare, pruritic, blistering disease of the skin that occurs in pregnancy and the puerperium. Its recognition by those caring for the pregnant patient is important in determining appropriate care for the patient and in alerting the physician to possible effects on fetal outcome. Understanding of this disease has been rapidly expanding, and these advances are reviewed here through the use of an illustrative case report and a review of the literature. The evidence for significant risk of fetal morbidity and mortality, the potential indicators in patients who are at high risk, and the treatment approaches are stressed.
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6/6. Acute upper airway obstruction resulting from systemic diseases.

    Acute upper airway obstruction is usually associated with inflammatory processes such as epiglottitis, and with laryngeal trauma and laryngeal tumors. Not uncommonly, systemic diseases such as Wegener's granulomatosis and sarcoidosis may manifest initially as upper airway obstruction requiring intubation or tracheostomy. We describe our experience in the diagnosis and management of cases of airway obstruction due to previously undiagnosed systemic diseases. We believe that physicians should be familiar with the airway manifestations of these systemic diseases when treating patients with airway distress of apparently unknown cause.
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