Continuing medical education
Neutrophilic dermatoses: Pyoderma gangrenosum and other bowel- and arthritis-associated neutrophilic dermatoses

https://doi.org/10.1016/j.jaad.2017.11.063Get rights and content

Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders that present with unique clinical features but are unified by the presence of a sterile, predominantly neutrophilic infiltrate on histopathology. The morphology of cutaneous lesions associated with these disorders is heterogeneous, which renders diagnosis challenging. Moreover, a thorough evaluation is required to exclude diseases that mimic these disorders and to diagnose potential associated infectious, inflammatory, and neoplastic processes. While some neutrophilic dermatoses may resolve spontaneously, most require treatment to achieve remission. Delays in diagnosis and treatment can lead to significant patient morbidity and even mortality. Therapeutic modalities range from systemic corticosteroids to novel biologic agents, and the treatment literature is rapidly expanding. The second article in this continuing medical education series reviews the epidemiology, clinical characteristics, histopathologic features, diagnosis, and management of pyoderma gangrenosum as well as bowel-associated dermatosis-arthritis syndrome and the arthritis-associated neutrophilic dermatoses rheumatoid neutrophilic dermatitis and adult Still disease.

Section snippets

Epidemiology

Key points

  1. Pyoderma gangrenosum is a rare inflammatory skin disease

  2. Pyoderma gangrenosum is more common in females than in males

  3. Approximately 50% of cases have associated medical comorbidities

Pyoderma gangrenosum (PG) is a rare inflammatory skin disease with an estimated incidence of 3 to 10 cases per million people per year.1 It affects individuals of all ages but is most common in adults between 40 and 60 years of age, with an average age of onset of 44 years.2, 3 It is more common in females versus

Bowel-associated dermatosis-arthritis syndrome

Key points

  1. Bowel-associated dermatosis-arthritis syndrome is most commonly seen in patients who have undergone bowel bypass surgery or other gastrointestinal surgeries with a blind-loop, as well as patients with a history of IBD

  2. Patients classically present with a constellation of symptoms resembling serum sickness, including myalgias, arthritis, and an inflammatory skin eruption

  3. The pathogenesis is thought to involve intestinal bacteria overgrowth and subsequent immune complex formation

  4. Treatment includes

Conclusion

In conclusion, NDs are a heterogeneous group of inflammatory skin disorders that are unified by the presence of sterile, predominantly neutrophilic infiltrates on histopathology. NDs are often associated with underlying infectious, inflammatory, and neoplastic disorders or medications. The available evidence points to altered expression of inflammatory effector molecules, abnormal neutrophil function, and genetic predisposition in the pathogenesis of these disorders. Treatment often involves

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    Date of release: December 2018

    Expiration date: December 2021

    Supported by a private donation from Mr Jerry and Mrs Joan Berstein.

    Conflicts of interest: None disclosed.

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