Background

This describes a group of fungal infections of the skin caused by dematiaceous (pigmented) fungi. These are localized infections often resulting from trauma with implantation of the infected material or in immunocompromised patients. It is common on the extremities starting as a scaly plaque and slowly evolving into a verrucous nodule or plaque. Rarely, dissemination may occur with lymphangitic nodules and hematogenous lesions.

The fungal species classically associated with the disease include:

Fonsecaea pedrosi (Phialophora pedrosi)
Phialophora compacta (Fonsecaea compacta)
Phialophora verrucosa
Cladosporium carrionii
Rhinocladiella aquaspersa (Acrotheca aquaspersa)

Wangiella dermatitidis (Phialophora dermatitidis) once was considered a cause but no longer. F. pedrosoi is the most commonly isolated organism but in Australia, it is C. carrionii.

Under the microscope, there is pseudoepitheliomatous hyperplasia and granulomas of both tuberculoid and suppurative types. Intraepidermal microabscesses are present. Throughout the dermis, there are numerous chronic inflammatory cells and scattered sclerotic bodies. In long standing lesions, a squamous cell carcinoma may develop.

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	Chromoblastomycosis

 

DISEASE ASSOCIATIONS

CHARACTERIZATION

Chromoblastomycosis: A review of 100 cases in the state of Rio Grande do Sul, Brazil Renan Minotto, etal.

J Am Acad Dermatol 2001;44:585-92. Abstract quote Background: If not diagnosed earlier, chromoblastomycosis has a chronic evolutional course that may cause several problems, such as difficulty in managing therapy because of the recrudescent character of the disease, potential association with the growth of epidermoid carcinoma in affected regions, and poor quality of life and work incapacity to the patient. Although infrequent, new cases are reported in the state of Rio Grande do Sul every year, ratifying the necessity for further studies on this disease. Objective: The purpose of this study was to review clinical features and response to therapy in patients with chromoblastomycosis and present data on the demography and history of this disease in the state of Rio Grande do Sul, Brazil. Methods: We reviewed case records of 100 patients with skin lesions caused by chromoblastomycosis, who were treated between 1963 and 1998. The cases were confirmed by the histopathologic and mycologic analyses made by the Dermatology Service of the Universidade Federal do Rio Grande do Sul at the Santa Casa de Misericórdia Hospital. Results: There was a predominance of male patients (4:1) and of white farmers whose ages ranged from 50 to 59 years, with lesions on their lower limbs. Most of them were from the northern regions of the state. The average time between the appearance of the disease and medical diagnosis was 14 years. The verrucous type proved to be the most frequently reported lesion (53%). Thorn wounds were associated with the disease in 16% of the cases. Lesions uncommon to some parts of the body were also reported. In two of the cases, cutaneous lesions caused by paracoccidioidomycosis and chromoblastomycosis were found in the same patient. Epidermoid carcinoma was found in the same parts of the body affected by chromoblastomycosis. Eumycotic mycetoma and chromoblastomycosis were associated. Fonsecaea pedrosoi was found in 96% of the cases, and Phialophora verrucosa in 4% of the cases. Conclusion: In our study, we observed a predominance of cases in the regions of Missões and Alto Uruguay, followed by the upper and lower northeastern slopes and the lowlands. Severe cases of chromoblastomycosis with intense skin involvement (eg, lesions with carcinoma) were observed. Statistical analysis showed recrudescence of the disease in 43% of cases despite the treatment used.

 

PATHOGENESIS

CHARACTERIZATION

Antigen distribution and antigen-presenting cells in skin biopsies of human chromoblastomycosis. Sotto MN, De Brito T, Silva AM, Vidal M, Castro LG. Department of Dermatology, and Institute of Tropical Medicine, University of Sao Paulo Medical School, Sao Paulo, Brazil.

Cutan Pathol. 2004 Jan;31(1):14-8 Abstract quote. BACKGROUND: Chromoblastomycosis is a chronic, suppurative, granulomatous mycosis usually confined to skin and subcutaneous tissues. The host defense mechanisms in chromoblastomycosis have not been extensively investigated. The purpose of the present study was to determine the distribution and pathways of the fungal antigen(s) and the possible role of the different immunocompetent cells in antigen processing in skin lesions. METHODS: The distribution of Fonsecaea pedrosoi antigen(s) in human skin was studied in 18 biopsies from 14 patients with chromoblastomycosis. A purified polyclonal immune serum raised in rabbits against metabolic antigen(s) of F. pedrosoi was used to detect yeast antigen(s) by immunohistochemical procedures. Double immunolabeling was performed with yeast antigen(s) and Langerhans' cells [labeled with anti-S100 protein monoclonal antibody (MoAb)], yeast antigen(s) and factor XIIIa+ dermal dendrocytes (immunolabeled with anti-factor XIIIa polyclonal antibody), and yeast antigen(s) and macrophages (labeled with CD 68 monoclonal antibody). RESULTS: The F. pedrosoi antigen(s) accumulated in the skin macrophages and, in a few instances, in factor XIIIa+ dendrocytes and Langerhans' cells. CONCLUSIONS: The data obtained suggest that chiefly macrophages, also Langerhans' cells and factor XIIIa+ dermal dendrocytes, function as antigen-presenting cells in chromoblastomycosis.

 

LABORATORY/RADIOLOGIC/ OTHER

CHARACTERIZATION

Humoral immune response in chromoblastomycosis during and after therapy. Esterre P, Jahevitra M, Andriantsimahavandy A. Parasitology Unit, Institut Pasteur de Madagascar, Antananarivo 101, Madagascar.

Clin Diagn Lab Immunol 2000 May;7(3):497-500 Abstract quote A longitudinal study was carried out in Madagascar, the most important focus of chromoblastomycosis (P. Esterre, A. Andriantsimahavandy, E. Ramarcel, and J. L. Pecarrere, Am. J. Trop. Med. Hyg. 55:45-47, 1996), to investigate natural immunity to this disease. Sequential blood samples were obtained before, during, and at the end of a successful therapeutic trial with terbinafine, a new antifungal drug. Using enzyme-linked immunosorbent assay and immunoblot methods, detailed analyses of antibody concentration and antigen mapping were conducted for 136 serum samples and tentatively correlated to epidemiological and pathobiological data. Two different cytoplasmic antigens, corresponding to the two fungal species involved (Fonsecaea pedrosoi and Cladophialophora carrionii), were used to analyze the distribution of different classes of immunoglobulins. This was done with respect to the origin of the isolates, clinical and pathobiological. Although strong individual variations were noticed, some major antigens (one of 18.5 kDa specific for F. pedrosoi and two of 23.5 and 33 kDa, respectively, specific for C. carrionii) corresponded to high antibody prevalence and concentration. As some antigenic components were also detected by immunoglobulin M (IgM) and IgA antibodies, the role that these specific antibodies could play in the immune response is discussed.

 

CLINICAL APPEARANCE AND VARIANTS

CHARACTERIZATION

Chromoblastomycosis: a retrospective study of 325 cases on Amazonic Region (Brazil). Silva JP, de Souza W, Rozental S. Departamento de Farmacia, Universidade Federal do Para, Brazil.

Mycopathologia 1998-99;143(3):171-5 Abstract quote A retrospective study of 325 cases of chromoblastomycosis diagnosed in the last 55 years in the Amazon region was carried out by the main Mycology services of the state of Para, Brazil (Department of Tropical Pathology--UFPA and Mycology Department of the Evandro Chagas Institute/FNS). The data obtained showed that: (a) the main age group affected by the diseases range from 41 to 70 years-old, (b) 86.1% of the patients were agricultural-workers, (c) 93.2% of them were males and (d) 80.7% showed lesions on the lower limbs (feet and legs). The diagnosis of 62% of the cases was confirmed by laboratory studies considering the tissue form in histopathological analysis. In 24% of patients (78 cases), the etiological agent was isolated and identified through culture. Fonsecaea pedrosoi was present in 77 cases and Phialophora verucosa in only one case.

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL
An unusual dematiaceous fungal infection of the skin caused by Fonsecaea pedrosoi: a case report and review of the literature. Hamza SH, Mercado PJ, Skelton HG, Smith KJ. University of Alabama at Birmingham, Pathology, and University of Alabama at Birmingham, Dermatology, Birmingham, AL, USA.	J Cutan Pathol. 2003 May;30(5):340-3. Abstract quote BACKGROUND: A case of an unusual dematiaceous fungal infection of the skin in a 43-year-old man with diabetes mellitus treated with steroids for reactive airway disease is presented. He developed chromoblastomycosis in the left wrist and was treated with antifungals and multiple surgical excisions. RESULTS: Histologic examination of the excised tissue revealed widespread suppurative granulomatous inflammation in the dermis and subcutaneous tissue. Thick-walled internally septated brown fungal cells were found both inside multinucleated giant cells and extracellularly. Non-to-lightly pigmented septate hyphal elements, however, were also identified with special stains and, in retrospect, on one of the routinely stained sections. In culture, the organism was reported to initially grow as soft white colonies that soon turned to black and velvety. CONCLUSIONS: The two unusual features of this case include the controversial report of the organism's initial growth in culture as soft white colonies and the presence of hyphal elements in addition to the sclerotic bodies in the dermis and subcutaneous tissue. This has not been reported before in human cases of dermal infection by Fonsecaea pedrosoi.

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSIS
Chromoblastomycosis: clinical and mycologic experience of 51 cases. Bonifaz A, Carrasco-Gerard E, Saul A. Dermatology Service and Mycology Department, General Hospital of Mexico, Mexico City, Mexico.	Mycoses 2001;44(1-2):1-7 Abstract quote This is a study of 51 cases of chromoblastomycosis detected in a 17-year period, all of which were clinically and mycologically proven by direct examinations, cultures and biopsies. The therapeutic results of the various treatments used are reported. Most cases were males (36 of 51; 70%), the mean age was 35 years and farmers predominated (74%); the most frequent lesions were in the lower limbs (54%). Major clinical presentations were nodular (41%) and verrucous (26%). The principal aetiologic agent isolated was Fonsecaea pedrosoi (90%). Overall results of the various treatments were as follows: 31% were cured, 57% improved and 12% failed. The best results were obtained with cryosurgery for small lesions, with itraconazole for large ones, and in some cases the combination of both treatments.
TREATMENT
Itraconazole pulse therapy in chromoblastomycosis. Kumarasinghe SP, Kumarasinghe MP. Consultant Dermatologist, Dermatology Unit, General Hospital, Kalutara, Sri Lanka.	Eur J Dermatol 2000 Apr-May;10(3):220-2 Abstract quote Although daily itraconazole has been used effectively in chromoblastomycosis, there is no record of pulse therapy for chromoblastomycosis. A 68-year-old woman with a history of slowly enlarging scaly plaque involving the left shoulder and lateral chest, presented to the dermatology clinic at General Hospital, Kalutara, Sri Lanka. Clinically chromoblastomycosis was suspected. Direct KOH smears showed sclerotic bodies and histology showed granulomata with characteristic brown spores. Itraconazole (Sporanox) 200 mg. b.i.d. orally was given for a week followed by 3 drug free weeks. This cycle was repeated for 6 months (i.e. 7 pulses). Clinical improvement was visible by 2 months. Scrapings and biopsy repeated 5 months after the commencement of treatment were negative for chromoblastomycosis. The lesion had clinically healed by 5 months. Examination 8 months after cessation of treatment did not show any recurrence. Itraconazole pulse therapy is cheaper than daily treatment but effective in chromoblastomycosis. The optimal dosage and end point of treatment need to be ascertained after a larger study.
Case report. A case of chromoblastomycosis effectively treated with terbinafine. Characteristics of chromoblastomycosis in the Kitasato region, Japan. Tanuma H, Hiramatsu M, Mukai H, Abe M, Kume H, Nishiyama S, Katsuoka K.	Mycoses 2000;43(1-2):79-83 Abstract quote A 38-year-old male with history of trauma in the left gluteal region 20 years ago presented with a dark red skin eruption at the traumatized area. It gradually grew to form an erythematous plaque with a well-defined border. Clinical findings and mycological cultures resulted in the diagnosis of chromoblastomycosis due to Fonsecaea pedrosoi. After initial administration of 5-fluorocytosine and local heat an almost complete cure was achieved with terbinafine combined with local heat therapy. A review is given on the chromoblastomycosis cases observed in the Kitasato region in Japan.
A case of chromomycosis treated by a combination of cryotherapy, shaving, oral 5-fluorocytosine, and oral amphotericin B. Poirriez J, Breuillard F, Francois N, Fruit J, Sendid B, Gross S, Dei-Cas E. Laboratoire de Biologie, Centre Hospitalier, Dunkerque, France.	Am J Trop Med Hyg 2000 Jul-Aug;63(1-2):61-3 Abstract quote A case of chromomycosis from Comoro Islands was first treated without success with high doses of oral amphotericin B (3 g per day). Treatment with itraconazole (400 mg per day) was also unsuccessful. Then, in vitro tests were done to study the susceptibility of this Fonsecaea pedrosoi strain to antifungal drugs. It was resistant to itraconazole, sensitive to 5-fluorocytosine, and the combination of 5-fluorocytosine with amphotericin B was synergistic. The patient was then treated with this last combination of drugs, which seemed to be effective. The patient stopped this treatment after six months, and relapse occurred two years later. The best therapeutic strategy in cases of chromomycosis seems to be a combination of two drugs chosen according to the results of prior antifungal susceptibility testing.

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Dematiaceous fungi-Group of pigmented fungi, worldwide distribution, predominately in tropical and subtropical areas. Present in soil and decaying vegetable matter. They cause two groups of infections-chromomycosis and phaeohyphomycosis.

Sclerotic bodies (Muriform cells, Copper Pennies)-An intermediate vegetative form of the fungus, arrested between yeast and hyphal morphology. These are round, thickwalled, golden brown cells, about 5-12 um in diamater, present in giant cells and occasionally in intraepidermal microabscesses.

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Last Updated 2/2/2004

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