You are in: eMedicine Specialties > Pediatrics: General Medicine > Infectious Disease SporotrichosisArticle Last Updated: Nov 15, 2007AUTHOR AND EDITOR INFORMATIONAuthor: William P Baugh, MD, Assistant Clinical Professor of Dermatology, University of California Irvine School of Medicine and Western School of Medicine; Medical Director, Full Spectrum Dermatology; Consulting Staff, Department of Dermatology, St Jude Medical Center William P Baugh is a member of the following medical societies: American Academy of Dermatology, American Society for Laser Medicine and Surgery, and Christian Medical & Dental Society Coauthor(s): Cynthia L Chen, BA, Clinical Assistant, Full Spectrum Dermatology; Brad S Graham, MD, Consulting Staff, Dermatology Associates of Tyler, East Texas Medical Center; Trinity Mother Francis Hospital Editors: Gary J Noel, MD, Department of Pediatrics, Clinical Associate Professor, Weill Medical College of Cornell University; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Larry I Lutwick, MD, Director, Division of Infectious Diseases, Veterans Affairs New York Harbor Health Care System, Professor, Department of Internal Medicine, State University of New York at Downstate; Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine; Russell W Steele, MD, Professor and Vice Chairman, Department of Pediatrics, Head, Division of Infectious Diseases, Louisiana State University Health Sciences Center Author and Editor Disclosure Synonyms and related keywords: Sporothrix schenckii, S schenckii, dimorphic fungal infection, lymphocutaneous sporotrichosis, fixed cutaneous sporotrichosis, disseminated sporotrichosis, Schenck disease, laryngeal and respiratory tract sporotrichosis, cutaneous sporotrichosis, lymphangitic cutaneous sporotrichosis, cellulitic sporotrichosis, mycetomalike sporotrichosis, systemic sporotrichosis, acquired immunodeficiency syndrome, AIDS, erythema nodosum, polyarteritis nodosum INTRODUCTIONBackgroundSporotrichosis is caused by the acquisition of Sporothrix schenckii, a dimorphic, saprophytic, and geophilic fungus. Although this fungal infection has been reported in temperate and tropical climates around the world, the fungus is less common in semiarid environments. S schenckii usually grows amidst decaying vegetable matter and in the soil as a saprophyte. Because sporotrichosis may be difficult to initially diagnose, a differential diagnosis should always be generated during the clinical evaluation (see Differentials). The association between sporotrichosis and acquired immunodeficiency syndrome (AIDS) was first reported in 1985. Although it is not considered an AIDS marker, it is included in a list of AIDS-related conditions. In immunocompromised patients, especially those with an impaired cell-mediated immunity, it can become an opportunistic infection with a possible life-threatening course. PathophysiologyThe fungus is most commonly acquired by traumatic implantation into the skin, causing a local pustule or ulcer with nodules developing proximally along the draining lymphatics. Once implanted, this saprophytic fungus can grow in human tissues. When recognized by the immune system, an inflammatory response occurs. Physical signs and symptoms relate to the location and degree of inflammation that ensues. Primary organ systems involved in sporotrichosis include the skin and the lungs, although infections at other sites have been reported. Little systemic illness usually occurs, unless the fungus is inhaled or acquired by a patient who is immunocompromised. Inhalation may cause a granulomatous pneumonitis. In a host who is immunocompromised, disseminated infection can occur from skin involvement or from primary pulmonary infection. For instance, one case has been reported of laryngeal and respiratory tract sporotrichosis after steroid inhaler use.1 Clinically, sporotrichosis may manifest as either an acute or a chronic subcutaneous mycotic infection. Although the acute phase is most common, chronic nodular lymphangiitis also reportedly develops in some cases. A minor puncture wound or splinter is sufficient to inoculate the fungus into the tissue. FrequencyUnited StatesSporotrichosis is an uncommon fungal infection of unknown frequency. InternationalDistribution is global, but incidence is unknown. Deep mycoses have mainly been reported in the tropics and subtropics. A recent endemic of lymphocutaneous sporotrichosis were reported in Mortality/MorbiditySporotrichosis is usually associated with minimal morbidity. Increased morbidity and, rarely, mortality may occur if the diagnosis is delayed, if the fungus infects patients who are immunologically compromised, or if inadequate or inappropriate therapy is rendered. RaceSporotrichosis has no racial predilection. SexSporotrichosis occurs in men, women, and children. Men have a higher risk of acquiring this fungus because they have greater environmental exposure from outdoor occupations. AgeSporotrichosis may occur in people of any age. Yet, in children, sporotrichosis tends to present more frequently with a solitary ulcerative nodule. This is in contrast to adults in which a classic lymphocutaneous form is more common. CLINICALHistoryTo evaluate a patient with possible sporotrichosis, investigate the history of risk factors for acquiring the fungus. Several predisposing factors may place a person at increased risk for developing sporotrichosis. Contact with certain plants known to harbor this fungus (eg, roses, sphagnum moss, salt-marsh hay, prairie hay) places patients at increased risk for the disease. The risk of this contact-acquired infection is increased among people in certain occupations, such as farmers or florists. Typical introduction of S schenckii into the skin has been described as occurring via a thorn or wood splinter; infections have also been reported in medical technicians who were exposed to tissue or culture specimens of S schenckii. Certain diseases, such as diabetes mellitus and alcoholism, also predispose a patient to develop localized disorders. In certain settings, patients who are immunocompromised are at risk for developing disseminated sporotrichosis. PhysicalOverall, this fungal infection most commonly affects the dorsum of the hands or fingers. Various primary lesions have been described, ranging from an erythematous papule or pustule to an ulcerating nodule. Sporotrichosis can be divided clinically into 2 main categories: cutaneous and systemic.
CausesSporotrichosis is typically acquired by inoculation of the fungus into the skin during contact with certain plants or animals. Classic scenarios include skin puncture by a splinter or rose thorn. Bites or scratches from sick animals such as cats, dogs, birds, and armadillos represent another source of infection. DIFFERENTIALSAtypical Mycobacterial Infection Blastomycosis Leishmaniasis Lymphangitis Tularemia
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| Drug Name | Potassium iodide (SSKI) |
|---|---|
| Description | For simple cutaneous lesions, the least expensive medication for treatment is a saturated solution of potassium iodide. This approach is commonly used in developing countries because of its low cost. SSKI can be administered on average for approximately 4-6 wk, but as long as 6 months. However, prolonged use should be undertaken with caution (see interactions below). The mechanism of action is unknown. Ineffective for systemic disease. |
| Adult Dose | 1 mL (5-10 gtt) PO tid with meals; increase by 1 mL/wk, reaching (not to exceed) 4-6 mL/d (40-60 gtt/d) by the end of 4-6 wk; longer courses may be needed based on clinical response |
| Pediatric Dose | Administer as in adults, with understanding that lower doses are usually more effective; should be effective at lower doses than in adults, but treatment is not usually well-tolerated because of adverse effects |
| Contraindications | Documented hypersensitivity; renal failure; hyperkalemia; pulmonary edema; tuberculosis; goiter; hypothyroidism |
| Interactions | Reported to interact with a number of different medications; because of potential for inducing elevated serum potassium levels, use cautiously with other medications that may cause hyperkalemia (eg, potassium-sparing diuretics, ACE inhibitors, potassium supplements) because these may result in very high levels of serum potassium, causing cardiac dysrhythmias and/or cardiac arrest; use with caution when prescribing with other antithyroid drugs because its ability to inhibit thyroid hormone secretion may result in significant hypothyroid states; coadministration with lithium may cause additive hypothyroid effects Use of SSKI for more than 1 mo can lead to interruption of thyroid hormone production, called Wolff-Chaikoff effect, because of excess iodide; patients with defective or absent autoregulatory mechanisms can become hypothyroid; with prolonged therapy, evaluation of TSH and T3 is advised |
| Pregnancy | D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus |
| Precautions | Caution in hyperkalemic states, cardiac disease, and acute bronchitis; prior to treatment, explain potential consequences of nausea, salivary gland swelling and salivation, fever, rash, and metallic taste |
| Drug Name | Itraconazole (Sporanox) |
|---|---|
| Description | DOC for cutaneous sporotrichosis. A fungistatic azole with broad spectrum of activity because of its inhibition of enzyme 14-alpha-demethylase, which is needed by the fungus for cell wall synthesis. Particularly effective for lymphocutaneous forms of sporotrichosis but may be used for fixed cutaneous and systemic forms. The caps and PO solution are not interchangeable (PO solution exhibits higher bioavailability). |
| Adult Dose | Average adult dose is 100 mg PO bid for 4-6 wk; take with food; 200 mg PO qd has also been reported successful in osteoarticular sporotrichosis In patients with AIDS, 300 mg/d for 6 mo, then 200 mg/d for 4 mo, then 100 mg/d for 2 mo |
| Pediatric Dose | 100 mg cap PO qd or 5 mg/kg qd; open and place in soft food (eg, applesauce); continue treatment for at least 1 wk following clinical resolution, typically 4-6 wk |
| Contraindications | Documented hypersensitivity; concomitant use with cisapride, terfenadine, and astemizole because of a drug-drug metabolism interaction causing potentially lethal cardiac effects |
| Interactions | Potent inhibitor or CYP450 3A4, thus, other medications metabolized via this system may accumulate, resulting in elevated levels and adverse effects (eg, alprazolam, midazolam, cisapride, terfenadine, astemizole, simvastatin, cyclosporine, tacrolimus); medications known to induce cytochrome P450 system (eg, rifampin) may lower levels of itraconazole, making drug ineffective; antacids may reduce absorption of itraconazole; conversely, acidic or carbonated beverage consumption may facilitate absorption; may increase digoxin levels |
| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus |
| Precautions | Caution with hepatitis or liver failure history (regularly monitor LFT results, although elevated LFT results are uncommon); other typical adverse effects include headache, pruritus, nausea, rhinitis, rash, and dyspepsia; PO solution contains hydroxypropyl-beta-cyclodextrin (solubilizing agent), which causes pancreatic hyperplasia and neoplasia at high doses in rats |
| Drug Name | Amphotericin B (Fungizone) |
|---|---|
| Description | DOC for disseminated or meningeal forms of systemic sporotrichosis. Some providers even consider this DOC for lymphocutaneous forms of sporotrichosis. Polyene antibiotic produced by a strain of Streptomyces nodosus; can be fungistatic or fungicidal. Binds to sterols, such as ergosterol, in the fungal cell membrane, causing intracellular components to leak with subsequent fungal cell death. |
| Adult Dose | 0.5 mg/kg/d slow IV infusion (over 4 h); not to exceed a total cumulative dose of 1-2 g |
| Pediatric Dose | Administer as in adults |
| Contraindications | Documented hypersensitivity |
| Interactions | Antineoplastic agents may enhance the potential of amphotericin B for renal toxicity, bronchospasm, and hypotension; corticosteroids, digitalis, and thiazides may potentiate hypokalemia; the risk of renal toxicity is increased with cyclosporine |
| Pregnancy | B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals |
| Precautions | Nephrotoxicity manifests as hypokalemia and renal tubular acidosis; regular renal function monitoring is recommended; fever and chills are not uncommon after first few administrations of drug; rare acute reactions may include hypotension, bronchospasm, arrhythmias, and shock |
| Drug Name | Terbinafine (Daskil, Lamisil) |
|---|---|
| Description | A fungicidal allylamine antifungal agent. Considered a third-line agent against sporotrichosis. Blocks ergosterol synthesis by inhibiting squalene epoxidase. Effective against S schenckii and other fungi and fungal infections, including most dermatophytes, Aspergillus species, blastomycosis, histoplasmosis, and Scopulariopsis brevicaulis. Terbinafine is well absorbed PO and has a long half-life. No elixir form is available; 250-mg tab is not scored and cannot be easily pulverized for use in children and is not palatable. |
| Adult Dose | 250 mg PO qd |
| Pediatric Dose | 20-40 kg: 125 mg PO qd/qod >40 kg: Administer as in adults |
| Contraindications | Documented hypersensitivity |
| Interactions | May decrease cyclosporine effects; toxicity of terbinafine may increase with rifampin and cimetidine |
| Pregnancy | B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals |
| Precautions | Pancytopenia, neutropenia, erythema multiforme, Steven-Johnson syndrome, acute generalized exanthematous pustulosis, taste disturbances, dyspepsia, and cholestatic liver disease; use with caution in patients with liver and/or renal dysfunction; LFT results should be monitored q6wk if long-term therapy is anticipated; monitor CBC count in patients with known or suspected immunodeficiency who are treated >6 wk |
| Drug Name | Fluconazole (Diflucan) |
|---|---|
| Description | A broad-spectrum azole antifungal agent. Considered a third-line agent for sporotrichosis treatment. Effective for a variety of fungi, including dermatophytes, candidal species, S schenckii, and some molds. It inhibits the enzyme 14-alpha-demethylase, preventing fungal cell wall formation. |
| Adult Dose | 800 mg PO qd for 4-6 wk |
| Pediatric Dose | 5-6 mg/kg/d PO for approximately 4-6 wk |
| Contraindications | Documented hypersensitivity |
| Interactions | Levels may increase with hydrochlorothiazide; fluconazole levels may decrease with long-term coadministration of rifampin; coadministration of fluconazole may decrease phenytoin clearance; may increase concentrations of theophylline, tolbutamide, glyburide, and glipizide; effects of anticoagulants may increase with fluconazole coadministration; increases in cyclosporine concentrations may occur when administered concurrently; moderately inhibits CYP450 3A3/4 isoenzymes |
| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus |
| Precautions | May cause GI upset, headache, rash, exfoliative dermatitis, and drug-induced hepatitis; discontinue if patient develops rash, elevated LFT results, or cholestasis |
Prognosis for patients with sporotrichosis depends on its clinical type (eg, fixed cutaneous, localized cutaneous, lymphocutaneous, disseminated), associated underlying diseases, and the patient's immune response to this fungus.
| Media file 1: Sporotrichosis with cutaneous necrosis and lymphangitic (sporotrichoid) spread. A 28-year-old white man presented for evaluation of a poorly healing, asymptomatic, round plaque acquired on the dorsum of his left hand. The lesion had been present for approximately 3 weeks. | |
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| Media file 2: Glucose-peptone agar culture plates revealing colony growth of Sporothrix schenckii. The left plate reveals older colonies as dark brown or black, and the right plate reveals younger white colonies with a brown center, characteristic of this fungus. | |
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| Media file 3: Microscopic examination of a blue dye preparation from the colony surface reveals elongated septate hyphae with groups of microconidia in a flowerlike arrangement. | |
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| Media file 4: A well-circumscribed, moderately elevated, erythematous plaque with central ulceration is found on the dorsum of this patient's left hand. Potassium chloride (KOH) stain was negative for fungal elements. | |
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| Media file 5: A 2 X 2 cm, dome-shaped, well-circumscribed, erythematous plaque is shown proximal to the left ring finger. The lesion was draining a serosanguineous fluid. No purulence was noted. | |
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| Media file 6: Biopsy rarely reveals the 6-mcg cigar-shaped yeast within tissue macrophages as shown in this histologic section. This is the morphology that Sporothrix schenckii assumes at 37°C. | |
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| Media file 7: Moist cream-colored colonies with a central, dark, leathery, and wrinkled surface growing at 25°C is highly suggestive of Sporothrix schenckii. | |
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| Media file 8: A fresh agar slant of Sporothrix schenckii reveals moist, white-to-creamcolored, yeastlike colonies. | |
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| Media file 9: Cutaneous, ulcerating, painless nodule on the hand and a classic sporotrichoid lymphangitic pattern spreading proximally up the arm. | |
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Article Last Updated: Nov 15, 2007