Table 4 Extracutaneous manifestations of Sweet's syndrome

Bone

Acute sterile arthritis, arthralgias, focal aseptic osteitis, pigmented villonodular synovitis, sterile osteomyelitis (chronic recurrent multifocal osteomyelitis) [12,32,44,164,212-215].

Central nervous system

Acute benign encephalitis, aseptic meningitis, brain SPECT abnormalities, brain stem lesions, cerebrospinal fluid abnormalities, computerized axial tomography abnormalities, electroencephalogram abnormalities, encephalitis, Guillain-Barre syndrome, idiopathic hypertrophic cranial pachymeningitis, idiopathic progressive bilateral sensorineural hearing loss, magnetic resonance imaging abnormalities, neurologic symptoms, "neuro-Sweet disease", pareses of central origin, polyneuropathy, psychiatric symptoms [33,68,212-229,423].

Ears

Tender red nodules and pustules that coalesced to form plaques in the external auditory canal and the tympanic membrane [230].

Eyes

Blepharitis, conjunctival erythematous lesions with tissue biopsy showing neutrophilic inflammation, conjunctival hemorrhage, conjunctivitis, dacryoadenitis, episcleritis, glaucoma, iridocyclitis, iritis, limbal nodules, ocular congestion, periocular swelling, peripheral ulcerative keratitis, retinal vasculitis, scleritis, uveitis [12,20,26,88,101,185,202,214,229,231-239,421].

Kidneys

Mesangiocapillary glomerulonephritis, urinalysis abnormalities (hematuria and proteinuria) [16,17,25,26,73].

Intestines

Intestine with extensive and diffuse neutrophilic inflammation, neutrophilic ileal infiltrate, pancolitis (culture-negative) [36,203,240,241].

Liver

Hepatic portal triad with neutrophilic inflammation, hepatic serum enzyme abnormalities, hepatomegaly [12,16,17,20,25,26,212,224,242].

Heart

Aortic stenosis (segmental), aortitis (neutrophilic and segmental), cardiomegaly, coronary artery occlusion, heart failure, myocardial infiltration by neutrophils, vascular (aorta, bracheocephalic trunk and coronary arteries) dilatation [243-247].

Lung

Bronchi (main stem) with red-bordered pustules, bronchi with neutrophilic inflammation, pleural effusion showing abundant neutrophils without microorganisms, progressive pharyngeal mucosal infiltration and edema resulting in upper-airway obstruction, and chest roentgenogram abnormalities: corticosteroid-responsive culture-negative infiltratives, pulmonary tissue with neutrophilic inflammation [17,20,73,101,138,139,165,205,212,246-251,434].

Mouth

Aphthous-like superficial lesions (buccal mucosa, tongue), bullae and vesicles (hemorrhagic: labial and gingival mucosa), gingival hyperplasia, necrotizing ulcerative periodontitis, nodules (necrotic: labial mucosa), papules (macerated: palate and tongue), pustules (individual and grouped: palate and pharynx), swelling (tongue), ulcers (buccal mucosa and palate) [26,75,102,117,203,249,252-254].

Muscles

Magnetic resonance imaging (T1-weighted and T2-weighted) abnormalities: high signal intensities due to myositis and fasciitis, myalgias (in up to half of the patients with idiopathic Sweet's syndrome), myositis (neutrophilic), tendinitis, tenosynovitis [73,75,244,255-257].

Spleen

Splenomegaly [212].