In the second part of this Continuing Medical Education article on paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS), its diagnostic criteria, investigative work-up, and management are reviewed. PNP/PAMS is a rare autoimmune blistering disorder associated with high morbidity and mortality. Recognizing PNP/PAMS's key features and its diagnostic criteria is critical in initiating appropriate work-up. Evaluating PNP/PAMS requires knowledge of its findings on histopathology, direct immunofluorescence, indirect immunofluorescence, and enzyme-linked immunosorbent assay. Lastly, treatments for PNP/PAMS are reviewed with suggestions based on case reports and expert opinions in the literature.

Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS) is a highly fatal autoimmune blistering disease. The condition occurs in patients with underlying benign or malignant neoplasms, most commonly lymphoproliferative disorders. Both humoral and cell-mediated immunities contribute to the pathogenesis, and autoantibodies against plakin family proteins are characteristic. Patients typically present with severe stomatitis and polymorphous skin lesions, which are often resistant to treatment. Bronchiolitis obliterans (BO) is a frequent complication which contributes to the high mortality rate of PNP/PAMS. Given the rarity of this disorder and heterogeneity of clinical presentation, clinicians should maintain a high index of suspicion for PNP/PAMS to avoid delayed diagnosis. In this first part of a two-part continuing medical education (CME) series, risk factors, pathogenesis, and clinical features of PNP/PAMS are discussed.

Fixed drug eruption (FDE) is a cutaneous adverse drug reaction characterized by the onset of rash at a fixed location on the body each time a specific medication is ingested. With each recurrence, the eruption can involve additional sites. Lesions can have overlying vesicles and/or bullae, and when they cover a significant percentage of body surface area, the eruption is referred to as generalized bullous fixed drug eruption (GBFDE). Due to the widespread skin denudation that can be seen in this condition, GBFDE may be confused clinically with Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). While treatments described for GBFDE include supportive care, topical and/or systemic steroids, and, recently, cyclosporine, the mainstay of management involves identifying and discontinuing the causative drug. This review article will provide an overview of FDE with an emphasis on its generalized bullous variant.

Although most dermatologic procedures are done in an office setting, some providers are performing them instead in ambulatory surgery centers (ASCs). This relocation of care comes with significantly higher expenses for patients and insurers. Compounding the issue of increased costs is the paucity of evidence demonstrating better outcomes associated with the use of ASCs. The most common cutaneous procedures have low complication rates when performed in an office setting and regular use of ASCs for these procedures is not justified.

A 21-year-old woman with severe craniofacial hyperhidrosis presents to the dermatology clinic for follow-up. She has failed treatment with aluminum salts and topical glycopyrrolate, and she requests treatment with botulinum toxin injections. You just started at the practice 6 months ago and are unaware of the practice's billing procedures for treating hyperhidrosis with botulinum toxin. You consult a colleague about how to proceed. She tells you that, although treatment for severe hyperhidrosis may be covered by insurance, the reimbursement is poor in comparison with that for cosmetic botulinum toxin. She states that the practice's policy is to charge according to the fee schedule for botulinum toxin injections as a cosmetic procedure.

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