Author:

SeanDevanney

Signs and Symptoms

• 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

Essential Workup

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

• Culture is the gold stand ard
• Sample from aspirated material, tissue biopsy, or sputum

Diagnostic Tests & Interpretation

Differential Diagnosis

• 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
  • Sarcoidosis

Initial Stabilization/Therapy

Airway/hemodynamic stabilization for severely ill patients with extracutaneous manifestations

ED Treatment/Procedures

• Lymphocutaneous/fixed cutaneous:
  • Itraconazole (drug of choice): Efficacious, but has numerous drug-drug interactions, potential for hepatotoxicity and 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 or cavitary lesions often require surgical resection plus amphotericin B
• Osteoarticular:
  • Itraconazole: First-line therapy for >1 yr, amphotericin B if refractory
• Disseminated:
  • Amphotericin B initially
  • Itraconazole in stable, immunocompetent patients
  • HIV and sporotrichosis: Suppressive therapy with itraconazole is recommended after initial infection

Medication

• Amphotericin B: Lipid or liposomal form 3-5 mg/kg daily (preferred due to reduced nephrotoxicity); if using deoxycholate form (pt with no risk of renal dysfunction) 0.7-1 mg/kg daily and infuse over 2 hr
• Itraconazole: Lymphocutaneous: 200 mg/dose (peds: 5 mg/kg/dose) b.i.d PO for 3 d, then 200 mg b.i.d PO for 2-4 wk after lesions resolve; pulmonary/osteoarticular: 200 mg PO b.i.d for at least 12 mo
  • Check serum levels after 2 wk to ensure therapeutic concentrations
• SSKI: 5 drop (peds: 1 drop) in milk or juice t.i.d; increase by 5 drops per dose each week up to a max 40-50 drops t.i.d (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

Disposition

Follow-up Recommendations

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

• 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 first-line therapy except for disseminated disease, where amphotericin B is used initially

• AungAK, TehBM, McGrathC, et al. Pulmonary sporotrichosis: Case series and systematic analysis of literature on clinico-radiological patterns and management outcomes . Med Mycol. 2013;51(5):534-544.
• BarrosMB, de Almeida PaesR, SchubachAO. Sporothrix schenckii and sporotrichosis . Clin Microbiol Rev. 2011;24:633-654.
• FrancesconiG, ValleAC, PassosS, et al. Terbinafine (250 mg/day): An effective and safe treatment of cutaneous sporotrichosis . J Eur Acad Dermatol Venereol. 2009;23:1273-1276.
• KauffmanCA, BustamanteB, ChapmanSW, 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.
• RudramurthySM, ChakrabartiA. Sporotrichosis: Update on diagnostic techniques . Curr Fungal Infect Rep. 2017;11:134-140.

ICD9

117.1 Sporotrichosis

ICD10

• B42.0 Pulmonary sporotrichosis

• B42.1 Lymphocutaneous sporotrichosis

• B42.9 Sporotrichosis, unspecified

• B42.82 Sporotrichosis arthritis

• B42.7 Disseminated sporotrichosis

• B42.81 Cerebral sporotrichosis

• B42.89 Other forms of sporotrichosis

• B42.8 Other forms of sporotrichosis

• B42 Sporotrichosis

SNOMED

• 42094007 Sporotrichosis (disorder)

• 45263007 Pulmonary sporotrichosis (disorder)

• 80890005 cutaneous sporotrichosis (disorder)

• 29081001 Sporotrichosis of the bones (disorder)