Toxic epidermal necrolysis (TEN) is a rare and potentially fatal mucocutaneous condition that may affect both children and adults. TEN mortality rates vary greatly between both patient populations, but multisystem involvement is common. Management presents many challenges as there is no specific therapy for TEN, and patients often require the expertise of burn units. We report a child who survived TEN with multisystem involvement including the rare but life threatening occurrence of a pulmonary air-leak syndrome while also reviewing controversies surrounding TEN's pathophysiology, sequelae, and multidisciplinary approach to management.
Among primary immunodeficiencies, antibody deficiencies are the most common. Patients often are examined first by multiple medical providers by whom the diagnosis may be missed or delayed, because signs and symptoms are usually sinopulmonary in origin, simulate common respiratory infections or respiratory allergies, and are usually responsive to symptomatic antimicrobial treatment. Because these diseases can be treated effectively, it is important to recognize them before complications occur. We have provided updated information on the more common antibody deficiency syndromes, their diagnostic assessment, and advances in treatment.
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