Stevens-Johnson Syndrome without Skin Lesions: A Rare and Clinically Challenging Disease in the Urgent Setting

Abstract

Stevens-Johnson syndrome, toxic epidermal necrolysis, and erythema multiforme are life threatening diseases causing mucocutaneous eruptions and can be difficult to manage medically. When oral tissues are involved, airway management can be of critical importance. Fluid and electrolyte imbalance are common and protocols to prevent secondary infection are initiated. All three conditions are rapidly evolving. Stevens-Johnson syndrome is more commonly associated with Mycoplasma pneumoniae in the pediatric population and drug hypersensitivity in adults, whereas erythema multiforme is mostly associated with herpes simplex virus in the adult population. These diseases are T-cell-mediated immune reactions, thought to represent a spectrum of the same disease. Clinical and immunohistochemical techniques are capable of differentiating Stevens-Johnson syndrome from erythema multiforme and provide insight into the possible underlying pathology creating the disease. Rare cases of Stevens-Johnson syndrome without skin manifestations have been associated with Mycoplasma pneumoniae and predominantly occur in males. In-hospital management is recommended to provide airway support, maintain fluid intake, electrolyte balance, obtain multi-speciality consultation, and to perform diagnostic testing. We describe a case of a 14 year old male with atypical Stevens-Johnson syndrome and a review of the literature.