- Email: [email protected]
Laryngeal sporotrichosis causing stridor in a young child
International Journal of Pediatric Otorhinolaryngology (2003) 67, 819 /823
www.elsevier.com/locate/ijporl
CASE REPORT
Laryngeal sporotrichosis causing stridor in a young child Nissim Khabiea, Thomas G. Boyceb, Glenn D. Robertsc, Dana M. Thompsona,* a
Department of Otorhinolaryngology, Division of Pediatric Otolaryngology, Mayo Clinic, Mayo Eugenio Litta Children’s Hospital, 200 First St. SW, Rochester, MN 55905, USA b Department of Pediatric Infectious Diseases, Mayo Clinic, Mayo Eugenio Litta Children’s Hospital, Rochester, MN, USA c Division of Clinical Microbiology, Mayo Clinic, Mayo Eugenio Litta Children’s Hospital, Rochester, MN, USA Received 18 April 2002; received in revised form 13 February 2003; accepted 19 February 2003
KEYWORDS Sporotrichosis; Fungal infection; Stridor
Summary Fungal infections of the larynx are rare entities that must be considered in the differential diagnosis of the patient who presents with laryngeal symptoms. We present an unusual case of initially recurrent and then persistent stridor in a 19month-old girl, unresponsive to 4 months of antibiotic and steroid therapy. Upon our laryngoscopic examination, the patient was noted to have an ulcerated, granulomatous process involving the larynx. She also had an erythematous papule on the left thigh. Fungal cultures of both sites grew Sporothrix schenckii . The patient was treated with systemic antifungal medications and had complete resolution of her symptoms. We discuss the pathophysiology and possible source of this unusual form of sporotrichosis, the first such case reported in a child. We emphasize the role of empiric steroid therapy in exacerbating and eventually enabling dissemination of the infection. We also review the manifestations of sporotrichosis infections of the head and neck. This case demonstrates the vital importance of careful diagnosis and proper treatment of stridor in children. – 2003 Published by Elsevier Science Ireland Ltd.
1. Introduction
Presented at the American Academy of OtolaryngologyHead and Neck Surgery Annual Meeting, Denver, CO, September 2001. *Corresponding author. Tel.: /1-507-284-8855 fax: /1-507284-3410;. E-mail address: [email protected] (D.M. Thompson).
Fungal infections of the larynx in immunocompetent children are uncommon and rarely cause stridor. In the larynx, histoplasmosis and candidiasis are the most common fungal infections. Sporotrichosis has only been described in the larynx once previously, causing hoarseness in an adult. We present a unique case of recurrent, severe stridor and respiratory compromise in a child caused by a sporotrichosis.
0165-5876/03/$ - see front matter – 2003 Published by Elsevier Science Ireland Ltd. doi:10.1016/S0165-5876(03)00089-2
820
2. Case report An otherwise healthy 19-month-old girl presented to our institution with a 4-month history of recurrent stridor. The child was in her normal state of excellent health until this presentation in early autumn, when she developed an acute onset of hoarseness followed by mild biphasic stridor. She did not have fever, cough, or a history of recent cold symptoms. She was taken to her local pediatrician who diagnosed croup and prescribed parenteral steroids. The stridor resolved but returned the next day. After recurrence of stridor following a second steroid injection, she underwent bronchoscopy, which demonstrated ‘‘tracheitis’’. She was treated with nebulized albuterol and intravenous antibiotics. Bacterial culture of the bronchial lavage fluid grew Haemophilus influenzae and Streptococcus viridans . A week after dismissal while on oral antibiotics, she returned with respiratory distress. She underwent a repeat bronchoscopy and remained intubated for 1 week. Stains for fungi demonstrated the presence of yeast-like forms, but fungal culture was negative. Bacterial culture again grew H. influenzae , and she was treated with clindamycin, clarithromycin, itraconazole, and tapering doses of prednisolone. Her stridor returned following reduction in the dose of steroids. She was readmitted to her local hospital 2 weeks later and biopsies demonstrated an inflammatory cell infiltrate of unknown etiology. She was dismissed after 1 week on a higher dose of steroids to control her symptoms. She continued to have stridor despite receiving 4 mg/kg per day of prednisolone and was referred to our institution. The child presented to us with a 4-month history of biphasic stridor, hoarseness, and occasional choking episodes. There was no history of cyanosis, apnea, or difficulty eating or drinking. Review of her most recent bronchoscopic video demonstrated supraglottic inflammatory changes on both the false cords as well as the laryngeal surface of the epiglottis. The subglottic region appeared edematous. On physical examination, the patient was cushingoid and had hoarseness and inspiratory stridor at rest. The oxygen saturation ranged from 88 to 90% while breathing room air. Her respiratory rate was normal. The lungs were clear to auscultation, and there were no retractions. Fiberoptic flexible laryngoscopy revealed an irregular laryngeal surface of the epiglottis with a cobblestone appearance; the process involved the left more than the right aryepiglottic fold. There were mild inflammatory changes of the true cords as well.
N. Khabie et al.
Microlaryngoscopy and rigid bronchoscopy was performed under general anesthesia. Evaluation revealed a white, cobblestoned, ulcerated, plaquelike exudate involving the entire supraglottis, predominantly on the epiglottis and right aryepiglottic fold and right false cord (Fig. 1). The subglottic region was mildly inflamed and narrowed, but without a firm stenotic lesion. The distal trachea was normal. The airway was sized to a 3.5 endotracheal tube. Biopsies were taken from the supraglottis and were sent for histopathological examination and culture. The patient was extubated without difficulty. Frozen-section pathology demonstrated necrotizing granulomatous inflammation, likely infectious and possibly fungal. Special stains demonstrated yeast cells, 2 /5 mm in size suggestive of Histoplasma capsulatum . The patient was started on amphotericin B for presumed laryngeal histoplasmosis and tapered off the corticosteroids. An infectious disease consultation was obtained. Further history was elicited regarding possible fungal exposures. Three days before the onset of symptoms the child had fallen face-first into the hay that lined the area containing the animals at a petting zoo. The parents grew roses at their previous residence, and the child would sometimes play with the mulch in the rose garden, but there was no history of skin trauma. The family moved from this house 3 months before onset of symptoms. Two months prior to onset, the family visited a large theme park in Florida, where she viewed the topiaries made of sphagnum moss but did not have contact with them. There was no history of exposure to armadillos or cats. On further examination, the patient was noted to have a 1-cm erythematous papule on the left thigh, reportedly present for the past 2 /3 weeks. A biopsy was performed of this lesion. Serologic testing demonstrated sporothrix agglutinins at a titer of 1:320. The skin biopsy demonstrated small budding yeast cells that were 2 /6 mm in diameter. Cells were oval to round to elongated. Typical ‘‘cigar-shaped’’ organisms characteristic of Sporothrix schenckii were seen (Fig. 2). Cultures of both the skin lesion and the larynx grew S. schenckii . To assure that there was no underlying immunocompromised state, immunologic testing was performed, including B and T cell subsets, quantitative immunoglobulins, neutrophil chemiluminescence, and HIV serology. These tests were all normal. The isolated skin lesion was the only evidence of disseminated infection; chest X-ray, ophthalmologic evaluation and computed tomography of the chest and abdomen were negative. The patient was treated for 10 days with intravenous amphotericin B, by which time her stridor and hoarseness had
Laryngeal sporotrichosis causing stridor in a young child
821
Fig. 1 Intraoperative photograph during microlaryngoscopy of the supraglottis (A) and bronchoscopy of the subglottis (B) demonstrates a white, cobblestoned, ulcerated plaque involving primarily the epiglottis, right ary epiglottic fold and right false cord as well as the cobblestone appearance of the subglottic region. Fig. 2 Photomicrograph of the skin biopsy specimen demonstrates small, round to oval, budding yeast cells 2 /6 mm in length, typical of S. schenckii . Fig. 3 Repeat examination after the initiation of antifungal therapy demonstrates near total resolution of the infectious process.
resolved. A repeat laryngoscopy at that time demonstrated near complete resolution of findings (Fig. 3). She was dismissed on a 3-week tapering dose of prednisolone and a 12-month course of oral itraconazole. She remains asymptomatic 12 months after completion of therapy.
3. Discussion This is the first reported case of stridor caused by sporotrichosis of the larynx. There is only one other case of laryngeal sporotrichosis in the literature, reported in a woman who worked in a sphagnum
822
moss plant. She developed longstanding hoarseness [1] due to S. schenckii infection of the vocal cord and was successfully treated with potassium iodide. S. schenckii is a dimorphic fungus that grows as a mold in nature and as oval or cigar-shaped yeastforms in vivo. Lymphocutaneous infection arises via traumatic inoculation of contaminated plant material into the skin. Typically occurring on the extremities, the lesions are raised, erythematous nodules that are most often painless. Infection usually remains localized to the immediate area of inoculation, although it can progress to regional lymph nodes. Disseminated or visceral involvement is rare. Transmission of the disease is typically through direct inoculation, except in pulmonary disease where inhalation of the spores or fungal hyphal fragments occurs. Outbreaks of sporotrichosis have been associated with substantial occupational or recreational exposure to moss, hay or other plant exposure [2 /4]. One report describes an outbreak occurring among workers and visitors to a haunted house containing infected hay [3]. The source of our patient’s infection remains unknown, although she had several possible exposures. The most likely explanation is that she inhaled a high inoculum of spores when falling face-first into the hay 3 days prior to the onset of her symptoms. In the head and neck, sporotrichosis is most often seen in the facial skin. Cases of sinusitis [4], endopthalmitis [5], orbital margin osteomyelitis [6], and nasal obstruction [7] due to S. schenckii have been reported. Disseminated disease is sometimes seen in chronic untreated cases and may be associated with an immunocompromised state. Although our patient proved to be immunocompetent after comprehensive testing, the question remains as to why she developed this S. schenckii infection. One explanation is iatrogenic immunodeficiency secondary to long-term, high-dose steroid treatment. Glucocorticoids suppress IL-2 production and inhibit the cell-mediated response to infection. An aggressive form of sporotrichosis has been described in a patient later found to suffer from Cushing’s syndrome [8]. The authors suggest that the excess endogenous corticosteroids in her case facilitated the progression of her disease. In the case of our patient, as she was weaned off steroids, her symptoms worsened, indicating the return of the normal inflammatory response. This led to a cycle of repeated steroid bursts to control her symptoms but which ultimately allowed proliferation of the fungal infection. Although it is possible that our patient’s initial symptoms were due to viral laryngotracheobron-
N. Khabie et al.
chitis, with the subsequent sporotrichosis due to steroid therapy, we believe this explanation is unlikely for several reasons. First, her initial presentation was not typical of croup. She had no prodrome of upper respiratory infection, no fever, and most importantly, no cough. Second, the location of her fungal infection was primarily supraglottic, whereas the symptoms of croup are caused by inflammation in the subglottis. Finally, all of her possible exposures to S. schenckii, including the exposure most likely to be the source of her infection */falling face-first into hay */ occurred prior to the initial onset of her symptoms. The pathogenic mechanism responsible for the skin lesion is intriguing. This lesion had only been present for a short time relative to the glottic process. There are reports of distant autoinoculation with sporotrichosis. This process is presumed to occur when an affected area is scratched followed by inoculating another area such as the skin [9] or the eye [10]. This type of autoinoculation assumes that the fungal elements are manually transferred after scratching an infected area, which is not possible with a supraglottic source. Aerosol contamination is unlikely, as the infectious form of the fungus is the mold form found in nature; the yeast form is not infectious and personto-person transmission does not occur by the aerosol route. In disseminated sporotrichosis, spread is thought to occur via the hematogenous route and this may be the explanation for the thigh lesion in our patient. In addition to allowing proliferation of the laryngeal infection, high-dose steroid therapy was probably responsible for enabling this secondary dissemination as well. Sporotrichosis can be treated with local or surgical methods in select cases, but pharmacological therapy is the mainstay of treatment [11]. A solution of saturated potassium iodide is effective in mild cutaneous disease. However, therapy is limited by thyroid and gastrointestinal side effects. Amphotericin B is the standard treatment for disseminated disease, but again can be limited by severe toxicities. Recently, itraconazole has been shown to be effective in treating sporotrichosis [12,13]. For severe or disseminated disease, amphotericin B is used initially followed by a prolonged course of itraconazole to prevent recurrence.
4. Conclusions This case demonstrates an unusual cause of stridor in a young child. It highlights the potential danger of empiric long-term steroid use for pre-
Laryngeal sporotrichosis causing stridor in a young child
sumed recurrent croup and emphasizes the importance of a multidisciplinary approach in obtaining the proper diagnosis for children with recurrent stridor.
[7]
References
[8]
[1] W.A. Agger, G.A. Seager, Granulomas of the vocal cords caused by Sporothrix schenckii , Laryngoscope 95 (1985) 595 /596. [2] S. Conias, P. Wilson, Epidemic cutaneous sporotrichosis: report of 16 cases in Queensland due to mouldy hay, Australas. J. Dermatol. 59 (1998) 34 /37. [3] D.P. Dooley, P.S. Botsic, M.L. Beckius, Spook house sporotrichosis */a point source outbreak of sporotrichosis associated with hay bale props in a Halloween haunted house, Arch. Intern. Med. 157 (1997) 1885 /1887. [4] M. Morgan, R. Reves, Invasive sinusitis due to Sporothrix schenckii in a patient with AIDS, Clin. Infect. Dis. 23 (1996) 1319 /1320. [5] C.D. Witherspoon, F. Kuhn, S.D. Owens, M.F. White, J.A. Kimble, Endopthalmitis due to Sporothrix schenckii after
.
[6]
[9] [10]
[11] [12]
[13]
823 penetrating ocular injury, Ann. Ophthalmol. 22 (10) (1990) 385 /388. B.W. Streeten, D.D. Rabuzzi, D.B. Jones, Sporotrichosis of the orbital margin, Am. J. Ophthalmol. 77 (1974) 750 /755. B.M. Clay, V.K. Anand, Sporotrichosis: nasal obstruction in an infant, Am. J. Otolaryngol. 17 (1996) 75 /77. S. Kim, M.H. Rusk, W.D. James, Erysipeloid sporotrichosis in a woman with Cushing’s disease, J. Am. Acad. Dermatol. 40 (1999) 272 /274. H.U. Kim, S.K. Yun, S.W. Ihm, Cutaneous sporotrichosis and distant autoinoculation, J. Dermatol. 22 (1995) 72 /73. D. Vieira-Dias, C.M. Sena, F. Orefice, M.A. Tanure, J.S. Hamdan, Ocular and concomitant cutaneous sporotrichosis, Mycoses 40 (5 /6) (1997) 197 /201. R.E. Winn, A contemporary view of sporotrichosis, Curr. Top. Med. Mycol. 6 (1995) 73 /94. P.K. Shrakey-Mathis, C.A. Kauffman, J.R. Graybill, D.A. Stevens, J.S. Hostetler, G. Cloud, et al., Treatment of sporotrichosis with itraconazole, Am. J. Med. 95 (3) (1993) 279 /285. J. Ramirez, R.P. Byrd, T.M. Roy, Chronic cavitary pulmonary sporotrichosis: efficacy of oral itraconazole, J. Kentucky Med. Assoc. 96 (1998) 103 /105.
www.elsevier.com/locate/ijporl
CASE REPORT
Laryngeal sporotrichosis causing stridor in a young child Nissim Khabiea, Thomas G. Boyceb, Glenn D. Robertsc, Dana M. Thompsona,* a
Department of Otorhinolaryngology, Division of Pediatric Otolaryngology, Mayo Clinic, Mayo Eugenio Litta Children’s Hospital, 200 First St. SW, Rochester, MN 55905, USA b Department of Pediatric Infectious Diseases, Mayo Clinic, Mayo Eugenio Litta Children’s Hospital, Rochester, MN, USA c Division of Clinical Microbiology, Mayo Clinic, Mayo Eugenio Litta Children’s Hospital, Rochester, MN, USA Received 18 April 2002; received in revised form 13 February 2003; accepted 19 February 2003
KEYWORDS Sporotrichosis; Fungal infection; Stridor
Summary Fungal infections of the larynx are rare entities that must be considered in the differential diagnosis of the patient who presents with laryngeal symptoms. We present an unusual case of initially recurrent and then persistent stridor in a 19month-old girl, unresponsive to 4 months of antibiotic and steroid therapy. Upon our laryngoscopic examination, the patient was noted to have an ulcerated, granulomatous process involving the larynx. She also had an erythematous papule on the left thigh. Fungal cultures of both sites grew Sporothrix schenckii . The patient was treated with systemic antifungal medications and had complete resolution of her symptoms. We discuss the pathophysiology and possible source of this unusual form of sporotrichosis, the first such case reported in a child. We emphasize the role of empiric steroid therapy in exacerbating and eventually enabling dissemination of the infection. We also review the manifestations of sporotrichosis infections of the head and neck. This case demonstrates the vital importance of careful diagnosis and proper treatment of stridor in children. – 2003 Published by Elsevier Science Ireland Ltd.
1. Introduction
Presented at the American Academy of OtolaryngologyHead and Neck Surgery Annual Meeting, Denver, CO, September 2001. *Corresponding author. Tel.: /1-507-284-8855 fax: /1-507284-3410;. E-mail address: [email protected] (D.M. Thompson).
Fungal infections of the larynx in immunocompetent children are uncommon and rarely cause stridor. In the larynx, histoplasmosis and candidiasis are the most common fungal infections. Sporotrichosis has only been described in the larynx once previously, causing hoarseness in an adult. We present a unique case of recurrent, severe stridor and respiratory compromise in a child caused by a sporotrichosis.
0165-5876/03/$ - see front matter – 2003 Published by Elsevier Science Ireland Ltd. doi:10.1016/S0165-5876(03)00089-2
820
2. Case report An otherwise healthy 19-month-old girl presented to our institution with a 4-month history of recurrent stridor. The child was in her normal state of excellent health until this presentation in early autumn, when she developed an acute onset of hoarseness followed by mild biphasic stridor. She did not have fever, cough, or a history of recent cold symptoms. She was taken to her local pediatrician who diagnosed croup and prescribed parenteral steroids. The stridor resolved but returned the next day. After recurrence of stridor following a second steroid injection, she underwent bronchoscopy, which demonstrated ‘‘tracheitis’’. She was treated with nebulized albuterol and intravenous antibiotics. Bacterial culture of the bronchial lavage fluid grew Haemophilus influenzae and Streptococcus viridans . A week after dismissal while on oral antibiotics, she returned with respiratory distress. She underwent a repeat bronchoscopy and remained intubated for 1 week. Stains for fungi demonstrated the presence of yeast-like forms, but fungal culture was negative. Bacterial culture again grew H. influenzae , and she was treated with clindamycin, clarithromycin, itraconazole, and tapering doses of prednisolone. Her stridor returned following reduction in the dose of steroids. She was readmitted to her local hospital 2 weeks later and biopsies demonstrated an inflammatory cell infiltrate of unknown etiology. She was dismissed after 1 week on a higher dose of steroids to control her symptoms. She continued to have stridor despite receiving 4 mg/kg per day of prednisolone and was referred to our institution. The child presented to us with a 4-month history of biphasic stridor, hoarseness, and occasional choking episodes. There was no history of cyanosis, apnea, or difficulty eating or drinking. Review of her most recent bronchoscopic video demonstrated supraglottic inflammatory changes on both the false cords as well as the laryngeal surface of the epiglottis. The subglottic region appeared edematous. On physical examination, the patient was cushingoid and had hoarseness and inspiratory stridor at rest. The oxygen saturation ranged from 88 to 90% while breathing room air. Her respiratory rate was normal. The lungs were clear to auscultation, and there were no retractions. Fiberoptic flexible laryngoscopy revealed an irregular laryngeal surface of the epiglottis with a cobblestone appearance; the process involved the left more than the right aryepiglottic fold. There were mild inflammatory changes of the true cords as well.
N. Khabie et al.
Microlaryngoscopy and rigid bronchoscopy was performed under general anesthesia. Evaluation revealed a white, cobblestoned, ulcerated, plaquelike exudate involving the entire supraglottis, predominantly on the epiglottis and right aryepiglottic fold and right false cord (Fig. 1). The subglottic region was mildly inflamed and narrowed, but without a firm stenotic lesion. The distal trachea was normal. The airway was sized to a 3.5 endotracheal tube. Biopsies were taken from the supraglottis and were sent for histopathological examination and culture. The patient was extubated without difficulty. Frozen-section pathology demonstrated necrotizing granulomatous inflammation, likely infectious and possibly fungal. Special stains demonstrated yeast cells, 2 /5 mm in size suggestive of Histoplasma capsulatum . The patient was started on amphotericin B for presumed laryngeal histoplasmosis and tapered off the corticosteroids. An infectious disease consultation was obtained. Further history was elicited regarding possible fungal exposures. Three days before the onset of symptoms the child had fallen face-first into the hay that lined the area containing the animals at a petting zoo. The parents grew roses at their previous residence, and the child would sometimes play with the mulch in the rose garden, but there was no history of skin trauma. The family moved from this house 3 months before onset of symptoms. Two months prior to onset, the family visited a large theme park in Florida, where she viewed the topiaries made of sphagnum moss but did not have contact with them. There was no history of exposure to armadillos or cats. On further examination, the patient was noted to have a 1-cm erythematous papule on the left thigh, reportedly present for the past 2 /3 weeks. A biopsy was performed of this lesion. Serologic testing demonstrated sporothrix agglutinins at a titer of 1:320. The skin biopsy demonstrated small budding yeast cells that were 2 /6 mm in diameter. Cells were oval to round to elongated. Typical ‘‘cigar-shaped’’ organisms characteristic of Sporothrix schenckii were seen (Fig. 2). Cultures of both the skin lesion and the larynx grew S. schenckii . To assure that there was no underlying immunocompromised state, immunologic testing was performed, including B and T cell subsets, quantitative immunoglobulins, neutrophil chemiluminescence, and HIV serology. These tests were all normal. The isolated skin lesion was the only evidence of disseminated infection; chest X-ray, ophthalmologic evaluation and computed tomography of the chest and abdomen were negative. The patient was treated for 10 days with intravenous amphotericin B, by which time her stridor and hoarseness had
Laryngeal sporotrichosis causing stridor in a young child
821
Fig. 1 Intraoperative photograph during microlaryngoscopy of the supraglottis (A) and bronchoscopy of the subglottis (B) demonstrates a white, cobblestoned, ulcerated plaque involving primarily the epiglottis, right ary epiglottic fold and right false cord as well as the cobblestone appearance of the subglottic region. Fig. 2 Photomicrograph of the skin biopsy specimen demonstrates small, round to oval, budding yeast cells 2 /6 mm in length, typical of S. schenckii . Fig. 3 Repeat examination after the initiation of antifungal therapy demonstrates near total resolution of the infectious process.
resolved. A repeat laryngoscopy at that time demonstrated near complete resolution of findings (Fig. 3). She was dismissed on a 3-week tapering dose of prednisolone and a 12-month course of oral itraconazole. She remains asymptomatic 12 months after completion of therapy.
3. Discussion This is the first reported case of stridor caused by sporotrichosis of the larynx. There is only one other case of laryngeal sporotrichosis in the literature, reported in a woman who worked in a sphagnum
822
moss plant. She developed longstanding hoarseness [1] due to S. schenckii infection of the vocal cord and was successfully treated with potassium iodide. S. schenckii is a dimorphic fungus that grows as a mold in nature and as oval or cigar-shaped yeastforms in vivo. Lymphocutaneous infection arises via traumatic inoculation of contaminated plant material into the skin. Typically occurring on the extremities, the lesions are raised, erythematous nodules that are most often painless. Infection usually remains localized to the immediate area of inoculation, although it can progress to regional lymph nodes. Disseminated or visceral involvement is rare. Transmission of the disease is typically through direct inoculation, except in pulmonary disease where inhalation of the spores or fungal hyphal fragments occurs. Outbreaks of sporotrichosis have been associated with substantial occupational or recreational exposure to moss, hay or other plant exposure [2 /4]. One report describes an outbreak occurring among workers and visitors to a haunted house containing infected hay [3]. The source of our patient’s infection remains unknown, although she had several possible exposures. The most likely explanation is that she inhaled a high inoculum of spores when falling face-first into the hay 3 days prior to the onset of her symptoms. In the head and neck, sporotrichosis is most often seen in the facial skin. Cases of sinusitis [4], endopthalmitis [5], orbital margin osteomyelitis [6], and nasal obstruction [7] due to S. schenckii have been reported. Disseminated disease is sometimes seen in chronic untreated cases and may be associated with an immunocompromised state. Although our patient proved to be immunocompetent after comprehensive testing, the question remains as to why she developed this S. schenckii infection. One explanation is iatrogenic immunodeficiency secondary to long-term, high-dose steroid treatment. Glucocorticoids suppress IL-2 production and inhibit the cell-mediated response to infection. An aggressive form of sporotrichosis has been described in a patient later found to suffer from Cushing’s syndrome [8]. The authors suggest that the excess endogenous corticosteroids in her case facilitated the progression of her disease. In the case of our patient, as she was weaned off steroids, her symptoms worsened, indicating the return of the normal inflammatory response. This led to a cycle of repeated steroid bursts to control her symptoms but which ultimately allowed proliferation of the fungal infection. Although it is possible that our patient’s initial symptoms were due to viral laryngotracheobron-
N. Khabie et al.
chitis, with the subsequent sporotrichosis due to steroid therapy, we believe this explanation is unlikely for several reasons. First, her initial presentation was not typical of croup. She had no prodrome of upper respiratory infection, no fever, and most importantly, no cough. Second, the location of her fungal infection was primarily supraglottic, whereas the symptoms of croup are caused by inflammation in the subglottis. Finally, all of her possible exposures to S. schenckii, including the exposure most likely to be the source of her infection */falling face-first into hay */ occurred prior to the initial onset of her symptoms. The pathogenic mechanism responsible for the skin lesion is intriguing. This lesion had only been present for a short time relative to the glottic process. There are reports of distant autoinoculation with sporotrichosis. This process is presumed to occur when an affected area is scratched followed by inoculating another area such as the skin [9] or the eye [10]. This type of autoinoculation assumes that the fungal elements are manually transferred after scratching an infected area, which is not possible with a supraglottic source. Aerosol contamination is unlikely, as the infectious form of the fungus is the mold form found in nature; the yeast form is not infectious and personto-person transmission does not occur by the aerosol route. In disseminated sporotrichosis, spread is thought to occur via the hematogenous route and this may be the explanation for the thigh lesion in our patient. In addition to allowing proliferation of the laryngeal infection, high-dose steroid therapy was probably responsible for enabling this secondary dissemination as well. Sporotrichosis can be treated with local or surgical methods in select cases, but pharmacological therapy is the mainstay of treatment [11]. A solution of saturated potassium iodide is effective in mild cutaneous disease. However, therapy is limited by thyroid and gastrointestinal side effects. Amphotericin B is the standard treatment for disseminated disease, but again can be limited by severe toxicities. Recently, itraconazole has been shown to be effective in treating sporotrichosis [12,13]. For severe or disseminated disease, amphotericin B is used initially followed by a prolonged course of itraconazole to prevent recurrence.
4. Conclusions This case demonstrates an unusual cause of stridor in a young child. It highlights the potential danger of empiric long-term steroid use for pre-
Laryngeal sporotrichosis causing stridor in a young child
sumed recurrent croup and emphasizes the importance of a multidisciplinary approach in obtaining the proper diagnosis for children with recurrent stridor.
[7]
References
[8]
[1] W.A. Agger, G.A. Seager, Granulomas of the vocal cords caused by Sporothrix schenckii , Laryngoscope 95 (1985) 595 /596. [2] S. Conias, P. Wilson, Epidemic cutaneous sporotrichosis: report of 16 cases in Queensland due to mouldy hay, Australas. J. Dermatol. 59 (1998) 34 /37. [3] D.P. Dooley, P.S. Botsic, M.L. Beckius, Spook house sporotrichosis */a point source outbreak of sporotrichosis associated with hay bale props in a Halloween haunted house, Arch. Intern. Med. 157 (1997) 1885 /1887. [4] M. Morgan, R. Reves, Invasive sinusitis due to Sporothrix schenckii in a patient with AIDS, Clin. Infect. Dis. 23 (1996) 1319 /1320. [5] C.D. Witherspoon, F. Kuhn, S.D. Owens, M.F. White, J.A. Kimble, Endopthalmitis due to Sporothrix schenckii after
.
[6]
[9] [10]
[11] [12]
[13]
823 penetrating ocular injury, Ann. Ophthalmol. 22 (10) (1990) 385 /388. B.W. Streeten, D.D. Rabuzzi, D.B. Jones, Sporotrichosis of the orbital margin, Am. J. Ophthalmol. 77 (1974) 750 /755. B.M. Clay, V.K. Anand, Sporotrichosis: nasal obstruction in an infant, Am. J. Otolaryngol. 17 (1996) 75 /77. S. Kim, M.H. Rusk, W.D. James, Erysipeloid sporotrichosis in a woman with Cushing’s disease, J. Am. Acad. Dermatol. 40 (1999) 272 /274. H.U. Kim, S.K. Yun, S.W. Ihm, Cutaneous sporotrichosis and distant autoinoculation, J. Dermatol. 22 (1995) 72 /73. D. Vieira-Dias, C.M. Sena, F. Orefice, M.A. Tanure, J.S. Hamdan, Ocular and concomitant cutaneous sporotrichosis, Mycoses 40 (5 /6) (1997) 197 /201. R.E. Winn, A contemporary view of sporotrichosis, Curr. Top. Med. Mycol. 6 (1995) 73 /94. P.K. Shrakey-Mathis, C.A. Kauffman, J.R. Graybill, D.A. Stevens, J.S. Hostetler, G. Cloud, et al., Treatment of sporotrichosis with itraconazole, Am. J. Med. 95 (3) (1993) 279 /285. J. Ramirez, R.P. Byrd, T.M. Roy, Chronic cavitary pulmonary sporotrichosis: efficacy of oral itraconazole, J. Kentucky Med. Assoc. 96 (1998) 103 /105.