Neuro-Ophthalmic Presentation of Neuro-Sweet Disease.

Acute febrile neutrophilic dermatosis (Sweet syndrome) is a systemic inflammatory condition usually associated with autoimmune or neoplastic processes and characterised by inflammatory dermatologic lesions such as erythematous plaques and papules associated with fever and leukocytosis. Neurological and ophthalmological involvement is rare. The authors describe an unusual case of Sweet syndrome associated with microscopic polyangiitis presenting with papilloedema, anterior uveitis, and skin rash. Years later, he developed acute posterior multifocal placoid pigment epitheliopathy. Treatment with immunosuppressive medications led to a relapsing remitting course with maximum benefit from use of steroids. The authors describe the difficulties in diagnosis and treatment of this rare case.

Bullous Pemphigoid in a Centenarian Male Simulating Toxic Epidermal Necrolysis.

Bullous pemphigoid (BP) is one of the most common autoimmune blistering diseases and classically presents as large, tense bullae. We report a case of BP with toxic epidermal necrolysis (TEN)-like manifestations in a 103-year-old male, the oldest known patient to present with an acute onset of BP. Our patient presented with extensive erosive lesions comprising 12% of the total body surface area, raising suspicion of TEN and Staphylococcal scalded skin syndrome. Detailed clinical, histological, and immunofluorescence analyses were performed, confirming a diagnosis of BP. Atypical presentations of blistering disorders can be a diagnostic challenge and require the use of histologic and direct immunofluorescence testing to distinguish between clinically similar cutaneous diseases. Proper diagnosis is essential to ensure appropriate management and patient care.

Expedient treatment of a collodion baby.

Only ~270 cases of collodion babies have been reported in the literature since 1892. As the name suggests, the term "collodion baby" refers to a phenotype that can be characterized by a yellow, shiny, tight parchment-like membrane stretched over the skin. Although the collodion membrane is only an evanescent condition of the newborn, neonatal complications can occur in 45% of all collodion babies, leading to a mortality rate of ~11% in the first few weeks of life. Most children born as collodion babies will spontaneously desquamate within 2 weeks, but may be as long as 3 months. Eventually, these children develop signs of one of several types of ichthyosis, which gives the skin the appearance of "fish scales." We report a unique case of a Caucasian male that was born as a Collodion baby at the University of Kentucky Children's Hospital in Lexington, Kentucky. Although the impairment of the skin barrier function put the patient at risk for a number of complications, he improved significantly after being treated with emollients and antibiotics. In contrast to previous findings, we found that skin emollients were beneficial and did not increase the risk of infection.