Background

Coccidioidomycosis immitis is a fungal infection. It is most commonly acquired by inhalation of the arthroconidia and results in an asymptomatic respiratory infection in 60%. Of these patients, 20% will develop cutaneous disease such as erythema multiforme or erythema nodosum. In these cases, the skin changes are a hypersensitivity reaction to the fungal antigens.

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

EPIDEMIOLOGY	CHARACTERIZATION
SYNONYMS	Sonoran Fever
GEOGRAPHY	Endemic in Lower Sonoran zone in southern Arizona

 

DISEASE ASSOCIATIONS	CHARACTERIZATION
GRANULOMATOUS DERMATITIS
Interstitial granulomatous dermatitis associated with pulmonary coccidioidomycosis David J. DiCaudo, MD Suzanne M. Connolly, MD Scottsdale, Arizona	J Am Acad Dermatol 2001;45:840-5 Abstract quote Background: Coccidioides immitis is a soil-dwelling fungus found in arid regions of the Western Hemisphere. Interstitial granulomatous dermatitis is a histopathologic pattern that may be a reactive manifestation of diverse systemic diseases. Objective: Our purpose was to describe clinical and histopathologic findings in 5 patients who presented with interstitial granulomatous dermatitis associated with pulmonary coccidioidomycosis. Methods: Medical records and skin biopsy slides from 5 patients were retrospectively reviewed. Results: In each patient, edematous papules, nodules, and plaques developed abruptly during the onset of an acute febrile illness. Coccidioidomycosis was confirmed by serology. Skin biopsy specimens revealed interstitial granulomatous dermatitis with neutrophils, leukocytoclasis, and eosinophils. Fungal stains (5/5 cases) and fungal cultures (2/2 cases) revealed no organisms within the skin biopsy specimens. Conclusion: Interstitial granulomatous dermatitis may be a presenting feature of pulmonary coccidioidomycosis and may possibly represent a reactive manifestation of the infection.
SWEET SYNDROME
Sweet Syndrome (Acute Febrile Neutrophilic Dermatosis) Associated With Pulmonary Coccidioidomycosis David J. DiCaudo, MD; Karel Jill Ortiz, MD; Stephanie J. Mengden, MD; Katherine K. Lim, MD	Arch Dermatol. 2005;141:881-884. Abstract quote Background  Sweet syndrome (acute febrile neutrophilic dermatosis) may arise in association with a variety of underlying systemic diseases. Only 1 case of coccidioidomycosis-associated Sweet syndrome has previously been reported. Observations  We describe 2 patients who developed Sweet syndrome during the onset of acute pulmonary coccidioidomycosis. Systemic antifungal therapy was given in both cases. Respiratory symptoms and skin lesions resolved within 5 weeks. Conclusions  Sweet syndrome may be a presenting feature of coccidioidomycosis. Recognition of the underlying pulmonary infection is important so that inappropriate treatment with systemic corticosteroids can be avoided.

 

PATHOGENESIS	CHARACTERIZATION
DENDRITIC CELLS
Dendritic cells and pattern of cytokines in paracoccidioidomycosis skin lesions. Pagliari C, Sotto MN.	Am J Dermatopathol 2003 Apr;25(2):107-12 Abstract quote We demonstrated and quantified by immunohistochemistry epidermal Langerhans cells, CD34+ dermal dendrocytes (DDs), and cells expressing TNFalpha, interferon-gamma (IFNgamma), IL-5, and IL-10 in skin lesions of paracoccidioidomycosis (PCM). Sixty-one biopsies were classified in three groups according to the pattern of tissue response: Group 1, well-organized granuloma; Group 2, poorly organized granuloma; and Group 3, both kinds of granuloma. Langerhans cells had short and irregular dendrites in all groups and were decreased in number in Groups 1 and 2. CD34+ DDs did not differ in number from the control group. Group 1 was characterized by many cells expressing IFNgamma. Groups 2 and 3 exhibited large numbers of cells expressing IL-5 and IL-10. The data obtained suggest that well-organized granulomas reflect a better cellular immune response, and the large number of cells expressing IL-5 and IL-10 in Group 2 indicate an ineffective response in PCM skin lesions. Both kinds of granuloma in the same cutaneous lesion probably represent an intermediate response between the anergic and hyperergic poles. Group 3 also showed higher numbers of cells expressing TNFalpha when compared with the control group. Some cells expressing TNFalpha were dendritic and localized around the granuloma similar to the factor XIIIa+ DD localization that we previously described.

 

LABORATORY/ RADIOLOGIC/ OTHER TESTS	CHARACTERIZATION
Laboratory Markers
Culture	Large sporangia 30-60 um with numerous endospores

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
GENERAL	Organisms usually found in foci of suppuration and granulomas
Coccidioidomycosis: a review and update. DiCaudo DJ. Department of Dermatology and Pathology, Mayo Clinic, Scottsdale, Arizona 85259, USA.	J Am Acad Dermatol. 2006 Dec;55(6):929-42; quiz 943-5. Abstract quote Coccidioidomycosis occurs in arid and semi-arid regions of the New World from the western United States to Argentina. Highly endemic areas are present in the southwest United States. Coccidioides species live in the soil and produce pulmonary infection via airborne arthroconidia. The skin may be involved by dissemination of the infection, or by reactive eruptions, such as a generalized exanthem or erythema nodosum. Interstitial granulomatous dermatitis and Sweet's syndrome have recently been recognized as additional reactive signs of the infection. Coccidioidomycosis is a "great imitator" with protean manifestations. Cutaneous findings may be helpful clues in the diagnosis of this increasingly important disease.
VARIANTS
Valley Fever	Combination of erythema nodosum, erythema multiforme, arthritis, and episcleritis in the setting of pulmonary infection
LUNG
DISSEMINATED	Occurs more frequently in African Americans, Filippinos, Asian women, and pregnant women in last 2 trimesters
CUTANEOUS	J Am Acad Dermatol 1981;4:38-46 Pustular, crusted, granulomatous, verrucous plaques and hyperkeratotic nodules If secondary to dissemination, usually on the face and scalp Primary infection is usually by primary inoculation presenting as an ulcer or chancre with lymphadenopathy spreading in a sporotrichoid fashion
MUSCULOSKELETAL
Musculoskeletal coccidioidomycosis: unusual sites of disease in a nonendemic area. Taxy JB, Kodros S. Department of Pathology, University of Chicago, Chicago, IL 60637, USA	Am J Clin Pathol. 2005 Nov;124(5):693-6. Abstract quote   Coccidioidomycosis is a primary pulmonary infection, endemic to the southwestern United States, caused by inhalation of spores in an immunocompetent host. When systemic spread occurs, the dissemination of infection to musculoskeletal sites might account for 20% to 50% of cases. The musculoskeletal manifestations are well recognized by physicians in endemic areas. We report 2 cases encountered in metropolitan Chicago in which morphologically typical, large, yeast-like, encapsulated, endosporulating organisms were identified in tissue samples and Coccidioides immitis was cultured. One patient had a degenerative-type radiographic picture thought to be related to a sports injury. A second patient with skin lesions and a paraspinal mass required emergency decompressive spinal surgery. A history consistent with exposure to Coccidioides organisms was apparent only for the first patient. Although the diagnosis can be established morphologically by identifying the large endospores in tissue samples, the submission of samples for culture and subsequent microbiologic confirmation requires the diagnosis to be considered clinically. This report emphasizes the rarity of the organism in nonendemic areas and the redundant value of using both morphologic and microbiologic modalities.

HISTOPATHOLOGY	CHARACTERIZATION
GENERAL
Unusual forms of immature sporulating Coccidioides immitis diagnosed by fine-needle aspiration biopsy. Ke Y, Smith CW, Salaru G, Joho KL, Deen MF. Department of Pathology & Laboratory Medicine, Robert Wood Johnson Medical School-UMDNJ, New Brunswick, NJ, USA.	Arch Pathol Lab Med. 2006 Jan;130(1):97-100. Abstract quote   Coccidioidomycosis is an endemic infection acquired by inhalation of the spores (arthroconidia) of the thermally dimorphic fungus, Coccidioides immitis. The arthroconidia transform into spherical cells called mature spherules in the lung. Immature spherules and other atypical forms of immature C immitis have rarely been found in vivo. We report on a case that presented unusual forms of immature sporulating C immitis in a fine-needle aspiration specimen. A 36-year-old Chinese woman, living in New Jersey for the past 10 years, presented with fever, night sweats, hemoptysis, and an abnormal chest radiograph approximately 9 months after a brief vacation trip to the Grand Canyon in Arizona. She was treated with antibiotics for 4 weeks without improvement. Subsequent chest computed tomography showed a 3-cm cavitary lesion in the right lower lobe of the lung. Fine-needle aspiration biopsy revealed diverse morphologic forms of a fungus that was confirmed by culture as immature sporulating C immitis.

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

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
TREATMENT
Coccidioidomycosis: Case report and update on diagnosis and management. Kim A, Parker SS. Department of Dermatology, Emory University School of Medicine.	J Am Acad Dermatol 2002 May;46(5 Pt 1):743-7 Abstract quote Coccidioidomycosis is a disease known for its endemicity to the Southwest United States. However, given the current trends of more frequent tourist and business travel, an increasing number of coccidioidomycosis infections are being seen in patients in other areas of the United States. Because the majority of patients with disseminated disease experience cutaneous manifestations, dermatologists can be of primary importance in the diagnosis of this disease. We report a case of coccidioidomycosis manifesting as granulomatous cutaneous nodules on the face of an otherwise healthy white man. We attempt to provide an update on the diagnosis, management, and treatment of patients who present with cutaneous lesions caused by coccidioidomycosis infection.

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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
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Last Updated December 7, 2006

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