A case of likely acute febrile neutrophilic dermatosis in a 17-year-old male presenting to general paediatrics

BMJ Case Rep. 2020 Jun 11;13(6):e233309. doi: 10.1136/bcr-2019-233309.

Abstract

We present the case of a 17-year-old male with a sore throat, tender cervical lymphadenopathy, bilateral erythematous and enlarged tonsils, fever, joint pain, widespread tender purpuric nodules, ulcerative lesions and erythematous pustules. The diagnosis was initially unclear. He had raised neutrophils, erythrocyte sedimentation rate and C-reactive protein. His skin biopsy showed a neutrophilic dermatosis with superficial pustulosis and leukocytoclastic vasculitis. Most likely, the patient suffered from a rare condition called acute febrile neutrophilic dermatosis (AFND). AFND is a very rare disorder of poorly understood aetiology, with only a few hundred reported cases in the literature. The complexity and rarity of this condition, and the difficulty in diagnosing, is an example of the challenge facing paediatricians as the paediatric admission age threshold increases to include older adolescents and young adults up to the age of 25 years, as per the National Health Service (NHS) long-term plan.

Keywords: dermatological; dermatology; disease and health outcomes; paediatrics; rheumatology.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Age Factors
  • Anti-Inflammatory Agents, Non-Steroidal / administration & dosage*
  • Arthritis* / diagnosis
  • Arthritis* / etiology
  • Biopsy / methods
  • Dermatology / methods
  • Diagnosis, Differential
  • Glucocorticoids / administration & dosage*
  • Humans
  • Lymphadenopathy* / diagnosis
  • Lymphadenopathy* / etiology
  • Male
  • Organizational Innovation
  • Pediatrics / methods
  • Rheumatology / methods
  • Skin / pathology
  • State Medicine / trends
  • Sweet Syndrome* / blood
  • Sweet Syndrome* / diagnosis
  • Sweet Syndrome* / drug therapy
  • Sweet Syndrome* / physiopathology
  • United Kingdom

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Glucocorticoids