Case report: severe pyoderma associated with familial Mediterranean fever--favorable response to colchicine in three

G Lugassy1, M Ronnen

  • 1Institute of Hematology, Barzilai Medical Center, Ashkelon, Israel.

Insights

Familial Mediterranean fever (FMF), an autosomal recessive disorder, can manifest with severe skin lesions like pyoderma. Colchicine therapy effectively treated these cutaneous manifestations in three FMF patients.

Area of Science:

  • Genetics and Immunology
  • Dermatology

Background:

  • Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder.
  • FMF is characterized by recurrent episodes of fever, serositis (peritonitis, pleuritis), and arthritis.
  • Diagnosis is primarily clinical, relying on characteristic symptoms and ethnic background, as no specific diagnostic tests exist.

Observation:

  • Three patients presenting with severe, recurrent pyoderma were identified.
  • These patients also exhibited clinical manifestations consistent with Familial Mediterranean fever.
  • The pyoderma lesions in these patients were associated with FMF symptoms.

Findings:

  • All three patients with severe pyoderma showed a favorable response to colchicine therapy.
  • Colchicine, a standard treatment for FMF, effectively managed the associated cutaneous lesions.
  • This suggests a potential link between FMF and severe pyoderma.

Implications:

  • The findings highlight the importance of considering FMF in patients with severe pyoderma, especially those with relevant ethnic backgrounds.
  • Successful colchicine treatment for pyoderma in these cases underscores its therapeutic potential.
  • This association may lead to improved diagnostic approaches and treatment strategies for FMF patients with dermatological manifestations.

Related Concept Videos

Drugs for Treatment of Ulcerative Colitis in IBD01:29

Drugs for Treatment of Ulcerative Colitis in IBD

Ulcerative colitis is a chronic inflammatory condition primarily affecting the colon and rectum. The primary drugs used in the treatment of ulcerative colitis are aminosalicylates. They exhibit anti-inflammatory and immunosuppressive properties. They modulate inflammatory mediators and inhibit the activity of nuclear factor κB (NF-κB). Aminosalicylates also reduce inflammation by inhibiting prostaglandin and leukotriene production and decreasing neutrophil chemotaxis and superoxide generation. 
Drugs for Treatment of Crohn's Disease in IBD Using Immunomodulatory Agents01:29

Drugs for Treatment of Crohn's Disease in IBD Using Immunomodulatory Agents

Crohn's disease is an inflammatory bowel disorder marked by chronic inflammation of the GI tract. Various treatment strategies for Crohn's disease are employed, such as immunomodulatory agents, glucocorticoids, and biologics or anti-TNF therapy. Azathioprine (Imuran), a commonly used immunomodulatory drug for Crohn's disease, is converted in the body to mercaptopurine, which inhibits purine biosynthesis and cell proliferation. Both are utilized in severe cases of Inflammatory Bowel Disease...
Pericarditis III: Medical Management01:17

Pericarditis III: Medical Management

The primary objectives of managing pericarditis are to determine the underlying cause, provide effective therapy for treatment and symptom relief, and promptly detect signs and symptoms of cardiac tamponade. The following outlines the essential aspects of medical management for pericarditis:ObjectivesDetermine the Cause: Identifying the underlying cause of pericarditis is crucial for targeted treatment. Causes include viral infections, autoimmune diseases, post-cardiac injury syndrome, and...
Acute Pyelonephritis II: Diagnostic Studies and Management01:28

Acute Pyelonephritis II: Diagnostic Studies and Management

Introduction:For diagnosing acute pyelonephritis, a comprehensive patient history is collected to identify symptoms such as dysuria, frequent or urgent urination, flank pain, or costovertebral angle (CVA) tenderness that may suggest a kidney infection.Physical ExaminationDuring the physical examination, CVA tenderness is assessed. This involves gentle percussion over the costovertebral angle, where tenderness often indicates a kidney infection.Diagnostic TestsUrinalysis: Used to identify white...
Rocky Mountain Spotted Fever01:26

Rocky Mountain Spotted Fever

Rocky Mountain Spotted Fever (RMSF) is a severe tick-borne illness caused by Rickettsia rickettsii, a Gram-negative, coccobacillary bacterium. This pathogen is an obligate intracellular parasite, requiring a host cell for replication. Transmission occurs through the bite of an infected tick. In the United States, the most important vectors are Dermacentor variabilis (American dog tick) and Dermacentor andersoni (Rocky Mountain wood tick), though other tick species may also serve as vectors.
Cholecystitis01:20

Cholecystitis

Cholecystitis is inflammation of the gallbladder, most commonly caused by obstruction of the cystic duct. This blockage prevents bile from draining, leading to gallbladder distension, inflammation, and potentially serious complications. This condition may present acutely or chronically and can happen with or without gallstones.EtiologyAbout 95% of cholecystitis cases are calculous, caused by gallstones blocking the cystic duct, leading to bile accumulation and inflammation of the gallbladder...