Summary
Definition
History and exam
Key diagnostic factors
- presence of risk factors
- nodules on shins
- uveitis, red eyes, retinal nodules, or candle-wax drippings
- nodules on other skin areas
- anaesthetic skin lesions
Other diagnostic factors
- joint pains
- fever
- diarrhoea, constipation, abdominal pain, haematochezia
- enlarged spleen
- miliary nodules on the retina
Risk factors
- streptococcal infection
- sarcoidosis
- tuberculosis
- coccidioidomycosis
- histoplasmosis
- blastomycosis
- brucellosis
- Behcet's disease
- oral contraceptives
- sulfonamides
- iodides and anti-epileptic drugs
- psittacosis
- leprosy
- inflammatory bowel disease
- pregnancy
- malignancy
Diagnostic investigations
1st investigations to order
- FBC
- anti-streptolysin-O titre
- chest x-ray
- tuberculin skin test, interferon gamma release assay
Investigations to consider
- serum ACE level
- skin lesion biopsy
- coccidioidin skin test
- histoplasmin skin test
- lepromin skin test
- blastomycosis serology
- brucellosis serology
- psittacosis serology
- x-ray symptomatic joints
- rheumatoid factor testing
- Yersinia agglutination titre
- Yersinia stool cultures
- colonoscopy
- serum IgD
- deep punch biopsies including subcutaneous fat, or incisional biopsies
Treatment algorithm
mild to moderately severe symptoms
severe refractory symptoms
Contributors
Authors
Daniela Kroshinsky, MD, MPH

Associate Professor of Dermatology
Harvard Medical School
Director of Pediatric Dermatology
Director of Inpatient Dermatology
Massachusetts General Hospital
Boston
MA
Disclosures
DK declares that she has no competing interests.
Acknowledgements
Dr Daniela Kroshinsky would like to gratefully acknowledge Dr Om P. Sharma, a previous contributor to this topic. Dr Kroshinsky would also like to thank Priyanka Vedak for her contribution to this topic. We would like to gratefully acknowledge the contribution of the late Elisabeth Higgins (Consultant Dermatologist, King’s College Hospital) for her peer review of this topic.
Disclosures
OPS declared that he had no competing interests. PV declared that she had no competing interests. EH declared that she had no competing interests.
Peer reviewers
Robert T. Brodell, MD
Professor of Internal Medicine
Clinical Professor of Dermatopathology
Northeastern Ohio Universities College of Medicine
Warren
Associate Clinical Professor of Dermatology
Case Western Reserve University School of Medicine
Cleveland
OH
Disclosures
RTB declares that he has no competing interests.
Differentials
- Weber-Christian disease
- Alpha-1-anti-trypsin deficiency
- Nodular vasculitis (erythema induratum or Bazin's disease)
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