Rosen & Barkin's 5-Minute Emergency Medicine Consult (673 page)

• Lymphocutaneous:
  
  • Most common form
    
  • Inoculation of fungus (
    Sporothrix schenckii
    ) into skin/soft tissue
    
  • Disease with or without hematogenous spread after traumatic inoculation with soil or plant material
    
  • Secondary to animal bites/scratches, especially from cats, trauma
    
  • Increased risk: Farmers, gardeners, landscapers, forestry workers
    
• Pulmonary:
  
  • Inhalation of conidia aerosolized from soil/plant decay
    
  • Increased risk: Alcoholics, diabetics, COPD, steroid users
    
• Multifocal extracutaneous:
  
  • Cutaneous inoculation and hematologic spread
    
  • Increased risk: HIV/immunosuppressed patients
    

• Fungal infection caused by
  S. schenckii
  :
  
• Dimorphic fungus
  
• Occurs as mold on decaying vegetation, moss, and soil in temperate and tropical environments
  
• Animal vectors, notably cats and armadillos
  

DIAGNOSIS

• Several clinical manifestations/syndromes
  
• Determined by mode of inoculation and host factors
  
• Lymphocutaneous:
  
  • Initial lesions appear days to weeks after inoculation
    
  • Begin as papules, become nodular, often ulcerate:
    
    • Distal extremities more commonly involved
      
    • Size: Millimeters to 4 cm
      
    • Pain absent or mild
      
    • Drainage is nonpurulent
      
  • Systemic symptoms usually absent
    
  • Secondary nodular lesions develop along lymphatics draining the original site.
    
  • May wax and wane over years if untreated
    
• Fixed cutaneous:
  
  • Plaque-like or verrucous lesion at the site of inoculation (typically face and extremities)
    
  • Ulceration uncommon
    
  • Do not manifest lymphangitic progression
    
  • Common in endemic regions of South America
    
• Extracutaneous:
  
  • Osteoarticular:
    
    • Secondary to local or hematologic inoculation
      
    • Septic arthritis more common than osteomyelitis
      
    • Joint inflammation, effusion, and pain
      
    • Single or multiple joint involvement of extremities: Knee, elbow, wrist, ankle
      
    • Indolent onset, few systemic symptoms
      
    • Tenosynovitis, septic arthritis, bursitis, nerve entrapment syndrome
      
    • Usually poor outcome due to delayed diagnosis
      
• Pulmonary:
  
  • Syndrome resembles mycobacterial infection (TB)
    
  • Fever, weight loss, fatigue, night sweats
    
  • Productive cough, hemoptysis, dyspnea
    
  • Uniformly fatal if untreated
    
• Multifocal extracutaneous (disseminated):
  
  • Low-grade fever, weight loss
    
  • Diffuse cutaneous lesions
    
  • Arthritis/osteolytic lesions/parenchymal involvement
    
  • Chronic lymphocytic meningitis
    
  • Ocular adnexa, endophthalmitis
    
  • Genitourinary, sinuses
    
  • Can be fatal if untreated
    
  • Often occurs in immunocompromised host
    

• Activity with exposure to soil, moss, organic material, or to cats in endemic areas
  
• Fixed cutaneous or lymphocutaneous: Healthy host
  
• Disseminated/extracutaneous: Diabetics, COPD, HIV/AIDS
  

• Fixed cutaneous/lymphocutaneous: Lesions found on exam
  
• Disseminated: Nonspecific findings
  

Diagnosis dependent on isolation
S. schenckii
from site of infection:

• Culture from aspirated material, tissue biopsy, or sputum
  

• Blood tests not indicated with cutaneous disease
  
• Cultures of sputum, synovial fluid, CSF, blood as indicated by extracutaneous manifestations
  
• No reliable serologic assays available
  

• Pulmonary:
  
  • Chest radiograph reveals cavitary lesions
    
• Extracutaneous/disseminated:
  
  • Consider bone scan in immunocompromised host.
    

• Lymphocutaneous/fixed cutaneous:
  
  • Biopsy reveals pyogranulomatous inflammation, 3–5 mm cigar-shaped yeast
    
• Pulmonary:
  
  • Gram stain of sputum may yield yeast; sputum cultures often positive
    
• Extracutaneous/disseminated:
  
  • CSF reveals lymphocytic meningitis, increased protein/decreased glucose
    

• Lymphocutaneous:
  
  • Leishmaniasis
    
  • Nocardiosis
    
  • Mycobacterium marinum
    
  • Tularemia
    
• Fixed cutaneous:
  
  • Bacterial pyoderma
    
  • Foreign-body granuloma
    
  • Inflammatory dermatophyte infections
    
  • Blastomycosis
    
  • Mycobacteria
    
• Osteoarticular:
  
  • Rheumatoid arthritis
    
  • Gout
    
  • Tuberculosis
    
  • Bacterial arthritis
    
  • Pigmented villonodular synovitis
    
• Pulmonary and meningitis:
  
  • Mycobacterial infections
    
  • Histoplasmosis
    
  • Coccidioidomycosis
    
  • Cryptococcal disease
    

TREATMENT

Airway/hemodynamic stabilization for severely ill patients with extracutaneous manifestations

• Lymphocutaneous/fixed cutaneous:
  
  • Itraconazole (drug of choice): Efficacious, but expensive and potential for hepatotoxicity, has numerous drug–drug interactions, black box in heart failure
    
  • Terbinafine: Less expensive alternative if failure of itraconazole, only in cutaneous disease
    
  • Saturated solution of potassium iodide (SSKI): Inexpensive but bitter taste and side effects (anorexia, nausea, diarrhea, fever, salivary gland swelling) lead to limited acceptability
    
  • Local heat therapy for cutaneous disease (>35°C) inhibits fungal growth, use in pregnant patients or others who cannot tolerate medication, therapy may take 3–6 mo
    
• Pulmonary:
  
  • Itraconazole or amphotericin B in early disease, effective in ∼30% of cases
    
  • More advanced disease often requires resection plus amphotericin B
    
• Osteoarticular:
  
  • Itraconazole: 1st-line therapy for more than 1 yr, amphotericin B if refractory
    
• Disseminated:
  
  • Amphotericin initially
    
  • Itraconazole in stable, immunocompetent patients
    
  • HIV and sporotrichosis: Suppressive therapy with itraconazole is recommended after initial infection
    

• Amphotericin B: Lipid form 3–5 mg/kg daily (preferred, especially in pregnancy and peds); if using deoxycholate form (pt with no risk of renal dysfunction) 0.7–1 mg/kg daily and infuse over 2 hr
  
• Itraconazole: Lymphocutaneous: 100–200 mg (peds: 6–10 mg/kg/d, max. 400 mg) PO TID for 3 days, then 100–200 mg per day for 2–4 wk after lesions resolve, pulmonary/osteoarticular: 200 mg PO TID for 3 days, then BID for 12 mo
  
• SSKI: 5 drop (peds: 1 drop) in water or juice TID; increase by 5 drops per dose each week up to a max. 40–50 drops TID (peds: max. of 1 drop/kg) as tolerated, for 6–12 wk or until lesions resolve
  
• Terbinafine: Lymphocutaneous only: 250–500 mg PO per day for 2–4 wk after lesions healed
  

FOLLOW-UP

• Systemic signs/symptoms
  
• Pulmonary, CNS, multifocal disease
  
• Immunosuppressed host with disseminated disease
  

Lymphocutaneous/fixed cutaneous form, nontoxic

Infectious disease consultant as appropriate

Infectious disease specialist, dermatology, appropriate specialist given disease involvement (orthopedics, neurology)

Fixed cutaneous, lymphocutaneous, pulmonary, extracutaneous/disseminated disease secondary to
S. schenckii
:

• Inoculation with soil, moss, or organic material (skin break or inhalation)
  
• Contact with cats
  
• Healthy hosts develop fixed cutaneous/lymphocutaneous disease, immunocompromised hosts develop extracutaneous/disseminated disease
  
• Disseminated disease presents with nonspecific symptoms that often result in delayed diagnosis and poor outcome.
  
• Oral itraconazole is 1st-line therapy except for disseminated disease, where amphotericin is used initially
  

• Barros MB, de Almeida Paes R, Schubach AO. Sporothrix schenckii and Sporotrichosis.
  Clin Microbiol Rev
  . 2011;24:633–654.
  
• Francesconi G, Valle AC, Passos S, et al. Terbinafine (250 mg/day): An effective and safe treatment of cutaneous sporotrichosis.
  J Eur Acad Dermatol Venereol
  . 2009;23:1273–1276.
  
• Freitas DF, do Valle AC, de Almeida Paes R, et al. Zoonotic sporotrichosis in Rio de Janeiro, Brazil: A protracted epidemic yet to be curbed.
  Clin Infect Dis
  . 2010;50:453.
  
• Kauffman CA, Bustamante B, Chapman SW, et al. Clinical practice guidelines for the management of sporotrichosis: 2007 update by the Infectious Diseases Society of America.
  Clin Infect Dis
  . 2007;45:1255–1265.
  
• Tiwari A, Malani AN. Primary pulmonary sporotrichosis: Case report and review of the literature.
  Infect Dis Clin Prac.
  2012;20:25.