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Background

This fungal infection is an opportunistic infection. One of the more common presentations is an infection in the nasal sinuses in patients with a diabetic ketoacidosis or diabetic coma. These organisms have a propensity to invade blood vessels causing thrombosis and infarction of the surrounding tissues.

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/Other Diagnostic Testing  
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/
Immunohistochemistry/
Electron Microscopy
 
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

DISEASE ASSOCIATIONS CHARACTERIZATION
Diabetes mellitus  
GOUT  
Cutaneous zygomycosis associated with urate panniculitis.

Vernon SE, Dave SP.

Department of Pathology, University of Miami Miller School of Medicine, Miami, FL 33136, USA.

Am J Dermatopathol. 2006 Aug;28(4):327-30 Abstract quote

Cutaneous zygomycosis is being increasingly recognized as a serious and life-threatening infection in debilitated and immunosuppressed patients, including transplant patients. The organisms are morphologically distinct but difficult to grow in cultures from clinical samples.

We report a case of cutaneous zygomycosis in a neonatal multi-visceral organ transplant patient, with subcutaneous panniculitis accompanied by extensive local acicular uric acid crystal deposition. Although the patient's serum uric acid was subsequently found to be in the normal range, transient hyperuricemia could not be excluded. Because we use a microwave-based processing system avoiding aqueous solutions, the crystals were maintained in the tissue sections and were shown by various methods to consist of monosodium urate. Early diagnosis combined with extensive debridement and prompt antifungal therapy resulted in a successful outcome.

We have coined the term "urate panniculitis" to describe this phenomenon.
Hematologic malignancies  
Malnutrition  
Immunocompromised states  
HIV

J Am Acad Dermatol 1994;30:904-908

Does not increase the risk of infection unless the patient has a history of IV drug abuse

 

PATHOGENESIS CHARACTERIZATION
Mucorales order

Pathologic Diagnosis of Fungal Infections. Chicago, Ill: American Society of Clinical Pathologists. 1987:8595.

Rhizopus
Mucor
Absidia species

 

Although classically described as broad nonbranching hyphae with branching at right angles,the zygomycetes are in fact pauciseptate rather than aseptate, having septa at irregular intervals

 

LABORATORY/
RADIOLOGIC
CHARACTERIZATION
CULTURES  
Increased culture recovery of zygomycetes under physiologic temperature conditions.

Department of Infectious Diseases, Infection Control and Employee Health, University of Houston College of Pharmacy, Houston, TX 77030, USA.

 

Am J Clin Pathol. 2007 Feb;127(2):208-12 Abstract quote

Poor recovery of Zygomycetes hyphae from tissue specimens may result from failure of current culture methods to mimic physiologic conditions found in hyphae-laden infected tissue.

We describe the use of an in vitro model simulating Zygomycetes growth under necrotic or hypoxic tissue conditions. We preconditioned hyphae of clinical Zygomycetes isolates in flasks under anaerobic conditions using Ana-Packs (Becton Dickinson Microbiology Systems, Sparks, MD) at 37 degrees C for 48 hours, thus simulating in vivo growth in an infracted hypoxic lesion, and compared the recovery of paired inocula at 37 degrees C and 25 degrees C. Incubation of stock culture isolates at 37 degrees C resulted in significantly better culture recovery (about 10-fold) when compared with incubation at 25 degrees C (P < .0001). In addition, we similarly evaluated 25, 291 consecutive clinical specimens.

Among 41 specimens, the yield of Zygomycetes cultures incubated at 37 degrees C (23/41 [56%]) was significantly higher than that incubated at 25 degrees C (9/41 [22%]; P = .0001). Overall, we found that culture recovery was significantly (254%) enhanced at 37 degrees C.

GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION
General  
VARIANTS  
Rhinocerebral
 
Pulmonary
 
Gastrointestinal
 
Disseminated
 
SKIN

Begin as erythematous patches which become purpuric and hemorrhagic and ulcerate with eschar formation

Usually secondary to disseminated disease
Primary usually from skin trauma
Burn patients at risk, especially from contaminated bandages


Mucor mycosis infection presenting as a non-healing ulcer in an immunocompromised patient.

Lenane P, Keane CO, Loughlin SO.

Dermatology Department, Mater Hospital, Eccles Street, Dublin, Ireland.

Clin Exp Dermatol. 2003 Mar;28(2):157-9 Abstract quote

Mucor mycosis is an uncommon saprophytic opportunistic fungus causing localized cutaneous infection associated with high morbidity and, on dissemination, high mortality.

We report the case of an immunocompromised patient with an aggressively progressing, painful non-traumatic ulceration, unresponsive to standard treatment.

Deep biopsies for haematoxylin and eosin staining and fungal culture revealed the characteristic broad non-septate irregular hyphae of mucor allowing introduction of the appropriate treatment.

Infection with mucor mycosis must be considered in today's medical environment as the number of immunocompromised patients increases.


Primary cutaneous mucormycosis in infants and neonates: case report and review of the literature.

Oh D, Notrica D.

University of Arizona College of Medicine, Phoenix Integrated Surgical Residency, Phoenix, AZ, USA.

 

J Pediatr Surg. 2002 Nov;37(11):1607-11. Abstract quote

A case of angioinvasive cutaneous mucormycosis in a premature infant, eventually requiring extracorporeal membrane oxygenation therapy, is described.

The fungal infection began at the site of a brachial artery catheter that had been covered with an adhesive dressing in the left antecubital fossa. The infection progressed rapidly over a 5-day period, and a left arm amputation was required. Fungal hyphae were present at the margins of resection. The patient eventually had disseminated mucormycosis and died. A second case of cutaneous mucormycosis in another premature infant also is presented. This infant had the infection at an intravenous catheter site. Rapid initiation of surgical debridement of the wound and amphotericin B therapy resulted in patient survival.

Eighteen reported cases of cutaneous mucormycosis in neonates were found and are reviewed. Prematurity, low birth weight, broad-spectrum antibiotics, corticosteroid therapy, and local trauma to the skin site were common risk factors. Only 7 of the 18 patients survived.

Therapy consisted of local debridement and intravenous amphotericin B. High index of suspicion, early diagnosis, and rapid institution of therapy can improve survival rate. The key to prevention appears to be appropriate skin care.


Primary cutaneous mucormycosis complicating the use of adhesive tape to secure the endotracheal tube.

Alsuwaida K.

University Health Network, University of Toronto, Toronto, Ontario, Canada.

Can J Anaesth. 2002 Oct;49(8):880-2. Abstract quote

PURPOSE: To report a rare case of primary cutaneous mucormycosis (PCM), complicating securing of the endotracheal tube with adhesive tape.

CLINICAL FEATURES: A 39-yr-old woman with systemic lupus erythematosus (SLE) developed four annular, punched out ulcers with a necrotic centre and elevated border in a linear distribution over the left cheek, under the tape securing the endotracheal tube. A tissue biopsy revealed broad, branching, nonseptate hyphae found in epidermis and dermis consistent with mucormycosis, best demonstrated with silver staining. Cultures were positive for Rhizopus species. Treatment with iv amphotericin B was successful.

CONCLUSION: Because of the rarity of the disease and the difficulty of culturing the causative organism, diagnosis of mucormycosis is often elusive. Tissue biopsy and microscopic visualization of nonseptate hyphae with right-angled branching are the only methods for making the diagnosis. Skin biopsy of new ulcerative or plaque-like lesions should be obtained in immunocompromised patients. Early diagnosis and prompt treatment are critical for favourable outcomes in PCM.


Primary cutaneous mucormycosis: guide to surgical management.

Losee JE, Selber J, Vega S, Hall C, Scott G, Serletti JM.

Division of Plastic Surgery, University of Rochester, Children's Hospital at Strong, Box 661, 601 Elmwood Avenue, Rochester, NY 14642, USA.

Ann Plast Surg. 2002 Oct;49(4):385-90. Related Articles, Links


Primary cutaneous mucormycosis: guide to surgical management.

Losee JE, Selber J, Vega S, Hall C, Scott G, Serletti JM.

Division of Plastic Surgery, University of Rochester, Children's Hospital at Strong, Box 661, 601 Elmwood Avenue, Rochester, NY 14642, USA.

Mucormycosis is the most acute, fulminate, and fatal of all fungal infections in humans. It presents most frequently in immunocompromised patients, but can occur in healthy patients in the presence of often-insignificant trauma. Surgical management of primary cutaneous mucormycosis is almost always required. Case reports of surgical treatment for primary cutaneous mucormycosis are reported in the literature; however, the extent of debridement required for cure is unclear and no uniform plan of treatment has been suggested.

To date, no clinical guidelines exist to assist the clinician in the surgical management of this disease. This article reviews the literature, reports on two clinical cases, and submits clinical guidelines designed to assist the clinician in the surgical management of primary cutaneous mucormycosis. Because of the infrequent and potentially fatal nature of the diagnosis, a high index of suspicion and a low threshold for wound biopsy must be maintained. Wound cultures are grossly inadequate and should not be relied on for a false sense of security.

It is recommended that, for the early diagnosis of cutaneous mucormycosis, chemotherapy and surgical debridement of grossly necrotic tissue be performed at the earliest possible time. The debrided wound is monitored for the resolution of surrounding erythema and induration before definitive reconstruction. In the case of delayed diagnosis and/or advanced or rapidly progressive disease, surgical debridement of all involved tissue, in addition to chemotherapy, is warranted.

 

HISTOLOGICAL TYPES CHARACTERIZATION
General

Arch Pathol Lab Med 125:375378, 2001

The inflammatory responses were predominantly neutrophilic (50%), predominantly granulomatous (5%), pyogranulomatous (25%), or absent (20%)

Invasive disease was characterized by prominent infarcts (94%), angioinvasion (100%), and prominent perineural invasion (90%) in biopsies that contained nerves for evaluation

At least rare hyphal septa were always seen (100%), and most branches (95%) varied from 45 to 90

Perineural invasion is a common finding in invasive zygomycosis, as are angioinvasion and infarcts
Prior to excluding the presence of these fungi in biopsies suspected to contain zygomycetes, the perineural space should be carefully examined

VARIANTS  
NECROTIZING FASCIITIS  
Zygomycotic necrotizing fasciitis in immunocompetent patients: a series of 18 cases.

1Department of Histopathology, Post-graduate Institute of Medical Education and Research, Chandigarh, India.

 

Mod Pathol. 2006 Sep;19(9):1221-6. Abstract quote

Necrotizing fasciitis is most often associated with bacterial infections. Zygomycosis is an uncommon infection causing necrotizing fasciitis.

We report 18 such cases of zygomycotic necrotizing fasciitis, of these, 15 were immunocompetent. Of the eight cases cultured, five were positive for Apophysomyces elegans. A retrospective case review conducted at a tertiary referral center, from 1998 to 2004, 18 cases of fungal necrotizing fasciitis were diagnosed based on histomorphology of fungal organisms; and in few of the cases diagnosis was supported by mycologic culture reports. Of the total of 18 cases, culture report was available in eight cases, and out of which five of them grew A. elegans. Fifteen patients were immunocompetent. Clinical presentation, mycologic findings and histopathologic results were evaluated.

A review of the literature pertaining to A. elegans infection was also done. Histopathologic examination showed broad, predominantly aseptate and occasional pauciseptate, thin-walled fungal hyphae with occasional angioinvasion. To the best of our knowledge, this is the first largest series of zygomycotic necrotizing fasciitis from India.

Herein, we present data on 18 cases of necrotizing fasciitis assosiated with zygomycosis. Most of the cases in our series were immunocompetent. Nonsuppurative necrosis with presence of typical fungal profiles was important histologic feature.

Zygomycosis must be considered in the differential diagnosis not only in immunocompromised patients but also in the absence of any underlying disorders.
SKIN

Large necrotic areas with suppuration involving the dermis and subcutaneous tissue

Hyphae common within the vessels causing thrombosis and infarction

 

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
Special stains PAS positive
GMS weakly positive

Clin Infect Dis 1992;14:126S-129S
J Am Acad Dermatol 1994;30:904-908

Macpherson and Pincus. Clinical Diagnosis and Management by Laboratory Methods. Twentyfirst Edition. WB Saunders. 2006.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.


Commonly Used Terms

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Commonly Used Terms
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Last Updated February 12, 2007

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