Behçet's disease differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2]

Overview

Behçet's disease needs to be differentiated from other diseases that present with similar symptoms such as erythema nodosum and inflammatory bowel disease.

Differentiating Behçet's disease from other Diseases

Differentiating Behçet's disease from other diseases that may cause arthritis and rash

Abbreviations: ANA: Antinuclear antibody, RF: Rheumatoid factor, Anti-CCp: Anti-cyclic citrullinated protein antibody, Anti U1RNP: Anti-U1 ribonucleoprotein antibodies , Anti Sm : Anti-Sm antibodies, Anti Ro: Anti Ro antibody also called anti-Sjögren's-syndrome-related antigen A antibody, Anti-dsDNA: Anti-double stranded DNA.

Disease Arthritis Auto-antibodies Raynaud phenomenon Rash pattern Distinguishing/specific features
Polyarthritis Tenderness Edema Deformity /Erosion Pattern ANA RF Anti-CCp Anti U1RNP Anti Sm Anti Ro Anti-dsDNA
Behçet's disease +/- +/- +/- - medium and large joints - - - - - - - Recurrent and usually painful mucocutaneous ulcers, acneiform lesions, papulo-vesiculo-pustular eruptions, superficial thrombophlebitis Male dominancy
Systemic lupus erythematosus[1] + + + - Small joints - - - - + Malar rash and photosensitivity
Rheumatoid arthritis (RA)[2] + + + + Small and large joints - ↑↑ ↑↑ - - - - + Subcutaneous nodules Erosive arthropathy
Rhupus[3] + + + + Small and large joints - + Malar rash and photosensitivity Erosive arthropathy
Mixed connective tissue disease (MCTD)[4] - - - + Small and large joints - ↑↑ - - - - + Cutaneous eruptions, gottron’s papules, photodistributed erythema, poikiloderma, and calcinosis cutis Overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM) that lead to more than one diagnosis
Undifferentiated connective tissue disease (UCTD)[5] + - - - Lower extremity - - - - + Erythematous macules, patches, or papules with delicate scale Multiple connective tissue diseases with no enough criteria for a single diagnosis
Systemic sclerosis (SSc)[6] +/- + + +/- Lower extremity ↑↑ - - - - + Hyperkeratosis, edema, and erythema Sclerodactyly, Telangiectasias, Calcinosis, Malignant hypertension, acute renal failure
Sjögren’s syndrome[7] +/- +/- - - Lower extremity, axiallary creases - - - - - Xerosis, scaly skin, annular erythema Keratoconjunctivitis sicca
Vasculitis Giant cell[8] - + + - Distal extremity - - - - - - - - Rare Involvement of cranial branches of arteries, visual loss
Takayasu[9] - +/- +/- - Transient extremity - - - - - - - Erythema nodosum, pyoderma gangrenosum Absent or weak peripheral pulse
Poly-arteritis nodosa[10] - +/- - - General and mild - - - - - - - Tender erythematous nodules, purpura, livedo reticularis, bullous or vesicular eruption Testicular pain or tenderness and neuropathies
Kikuchi’s disease[11] - +/- - - medium and large joints ↑/↓ - - - - - - - Transient skin rashes, malar rash, erythematous macules, patches, papules, or plaques May be associated with SLE
Serum sickness[12] + + +/- - General - - - - - - - - Pruritic rash, urticaria and/or serpiginous macular rash Self-limited
Psoriatic arthritis[13] - - - - Small and large joints - - - - - - - - Psoriasis and onychodystrophy Dactylitis (sausage digits)
Human parvovirus B19 infection[14] + + - - Small joints - - - - - - - - Erythematous rashes Rare in adults, fifth's disease in children

Behçet's disease oral lesions must be differentiated from other mouth lesions such as oral candidiasis and aphthous ulcer

Disease Presentation Risk Factors Diagnosis Affected Organ Systems Important features Picture
Diseases predominantly affecting the oral cavity
Oral Candidiasis
  • Denture users
  • As a side effect of medication, most commonly having taken antibiotics. Inhaled corticosteroids for the treatment of lung conditions (e.g, asthma or COPD) may also result in oral candidiasis which may be reduced by regularly rinsing the mouth with water after taking the medication.
  • Clinical diagnosis
  • Confirmatory tests rarely needed
Localized candidiasis

Invasive candidasis

Tongue infected with oral candidiasis - By James Heilman, MD - Own work, CC BY-SA 3.0, httpscommons.wikimedia.orgwindex.phpcurid=11717223.jpg
Herpes simplex oral lesions
  • Stress
  • Recent URTI
  • Female sex
  • The symptoms of primary HSV infection generally resolve within two weeks
Oral herpes simplex infection - By James Heilman, MD - Own work, CC BY-SA 3.0, httpscommons.wikimedia.orgwindex.phpcurid=19051042.jpg
Aphthous ulcers
  • Painful, red spot or bump that develops into an open ulcer
  • Physical examination
  • Diagnosis of exclusion
  • Oral cavity
  • Self-limiting , Pain decreases in 7 to 10 days, with complete healing in 1 to 3 weeks
By Ebarruda - Own work, CC BY-SA 3.0, httpscommons.wikimedia.orgwindex.phpcurid=7903358
Squamous cell carcinoma
Squamous cell carcinoma - By Luca Pastore, Maria Luisa Fiorella, Raffaele Fiorella, Lorenzo Lo Muzio - http://www.plosmedicine.org/article/showImageLarge.action?uri=info%3Adoi%2F10.1371%2Fjournal.pmed.0050212.g001, CC BY 2.5, https://commons.wikimedia.org/w/index.php?curid=15252632
Leukoplakia
  • Vulvar lesions occur independent of oral lesions
Leukoplakia - By Aitor III - Own work, Public Domain, https://commons.wikimedia.org/w/index.php?curid=9873087
Melanoma
Oral melanoma - By Emmanouil K Symvoulakis, Dionysios E Kyrmizakis, Emmanouil I Drivas, Anastassios V Koutsopoulos, Stylianos G Malandrakis, Charalambos E Skoulakis and John G Bizakis - Symvoulakis et al. Head & Face Medicine 2006 2:7 doi:10.1186/1746-160X-2-7 (Open Access), [1], CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=9839811
Fordyce spots
Fordyce spots - Por Perene - Obra do próprio, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=19772899
Burning mouth syndrome
Torus palatinus
Torus palatinus - By Photo taken by dozenist, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=846591
Diseases involving oral cavity and other organ systems
Behcet's disease
Behcet's disease - By Ahmet Altiner MD, Rajni Mandal MD - http://dermatology.cdlib.org/1611/articles/18_2009-10-20/2.jpg, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=17863021
Crohn's disease
Agranulocytosis
Syphilis[17]
oral syphilis - By CDC/Susan Lindsley - http://phil.cdc.gov/phil_images/20021114/34/PHIL_2385_lores.jpg, Public Domain, https://commons.wikimedia.org/w/index.php?curid=2134349
Coxsackie virus
  • Symptomatic treatment
Hand-foot-and-mouth disease
Chicken pox
Chickenpox - By James Heilman, MD - Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=52872565
Measles
  • Unvaccinated individuals[18][19]
  • Crowded and/or unsanitary conditions
  • Traveling to less developed and developing countries
  • Immunocompromized
  • Winter and spring seasons
  • Born after 1956 and never fully vaccinated
  • Health care workers
Koplick spots (Measles) - By CDC - http://phil.cdc.gov/PHIL_Images/20040908/4f54ee8f0e5f49f58aaa30c1bc6413ba/6111_lores.jpg, Public Domain, https://commons.wikimedia.org/w/index.php?curid=824483

References

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