Australas J. Dermatol 1992; 33: 179-180
AUSTRALIAN DERMATOPATHOLOGY SOCIETY DIGITAL DILEMMA KURT GEBAUER
Royal Perth Hospital A sixty year old male caucasion presented with a ten year history of a lesion on his left index finger. The lesion first developed whilst he was working as a deep sea fisherman off the east coast of Australia. It had slowly increased in size and become more nodular. There was no significant past history and he was not taking any medications. Examination showed a hyperkeratotic and indurated plaque on the left index finger. There were dermal nodules singly and in groups that extended proximally to the level of the metacarpophalangeal joint (Fig 1).
A skin biopsy was taken for histopathological examination and culture. Prepared sections show hyperkeratosis and marked irregular epidermal hyperplasia. In the dermis there are infiltrates of chronic inflammatory cells. Also present are granulomas composed of epithelioid cells and multinucleated giant cells. Some granulomas have a small amount of central suppuration. Rounded brown fungal spores are seen within giant cells and lying free in the dermis (Fig 2). They are also present in keratinous material within hair follicles. FIGURE 1—Granulomatous and
hyperkeratotic changes of finger.
FIGURE 2—Histopathological appearance.
179
KURT GEBAUER CHROMOBLASTOMYCOSIS
Cultured material grew Phialophora verrucosa. Initial management was with Itraconazole in a dose of 300 mg per day. Clinically the lesions responded rapidly, losing their hyperkeratinisation and nodularity. We were unable to increase the dose to 400 mg per day due to gastric intolerance. At all times his biochemical and haematological indices remained normal. Repeat biopsy after eight months of therapy demonstrated multiple fungi in the dermis. The fungus was cultured and found to be sensitive to Itraconazole, though not to Amphotericin B and 5-Flucytosine. The fungus was also sensitive to Ketoconazole. However, four years previously the patient had been treated with a twelve month course of Ketoconazole 200 mg daily and the lesion had increased in size during this treatment. With these results it was decided to treat clinically apparent granulomas with liquid nitrogen cryotherapy using the freeze/double freeze technique. Each granuloma was frozen for at least thirty seconds. Clinically a good response was apparent with minimal scarring. Unfortunately the patient was lost to follow-up when he returned to the Eastern States. Discussion Chromoblastomycosis is a chronic cutaneous and subcutaneous infection of the skin caused by pigmented fungi. It is believed that infection arises after traumatic implantation of fungal organisms.' These fungi are commonly found growing in soil, decaying vegetation and rotting woods. ^ Infection is most common in the tropical and subtropical climates and it occurs rarely in Australia. There is no racial predisposition. Most cases are seen in male agricultural workers between the ages of 30 and 50.'' Children rarely develop the disease. Lesions first begin as nodules that ulcerate and spread gradually, forming large exophytic, keratinous masses on the skin surface. These persist, enlarging slowly for many years and can heal with scarring and keloid formation. Lesions are nearly always localised to the skin and subcutis. There have been rare reports of haematogenous spread, lymphatic metastases and brain abscesses."'* The most common aetiological agents are species of Phialophora, Cladosporium and Fonsecaea. Wangiella dermatitidis has been isolated on a few occasions." All these fungi have been cultured from lesions presenting within Australia. The histopathological findings are of foreign body granulomas with occasional abscess
formation. The distinct round fungal spores, also known as copper penny bodies, can be easily seen in the tissues (Fig 2). Granulomas are often seen in association with marked pseudo-epitheliomatous hyperplasia.''^ When cultured on 2% glucose Sabouraud medium the colonies appear as black spots which develop into dark brown, grey or green cup shaped plaques. They can be differentiated by their type of spore formation and fine detail of spore structure.'^ If it is possible, complete surgical excision of all lesions is curative." Cryotherapy or destruction by cautery of diathermy can be used for small lesions. Amphotericin B given intravenously or intralesionally has also been reported with success. However, when used systemically fungicidal concentrations are not obtainable in vivo. Intralesional injection of Amphotericin B is very painful and not suitable for large areas. 5-Flucytosine treatment has been reported as effective, but unfortunately the responsive fungi rapidly develop resistance and therefore it is best given in association with another active agent. Oral Thiabendazole in a dosage of 25 mg per kilogram of body weight for many months has resulted in reported cure rates of 30 to 40%. Most recently Itraconazole has been recommended as the agent of choice.' In our case Itraconazole was given in higher doses than recommended for eight months. This resulted in clinical improvement of the lesions, but biopsy and culture still showed large numbers of fungal bodies in the tissue. It may be that perseverance with high doses for extended periods of time would be required to cure the infection. Acknowledgement
Colour printing in this presentation has been supported by a grant from the Australian Society of Dermatopathology. References ' Vollum DI. Chromomycosis: a review. Br JDermatol 1977; 96: 454-458. ^ Okeke CN, Gugnani HC. Studies on pathogenic dematiaceous fungi. 1. Isolation from natural sources. Mycopathologia 1986; 94: 19-25. ' McGinnis MR. Chromoblastomycosis and phaeohyphomycosis. New concepts, diagnosis and mycology. JAm Acad Dermatol 1983; 8: 1-16. ' Azulay RD, Serruya J. Hematogenous dissemination in chromoblastomycosis. Arch Dermatol 1967; 95: 57-60. ' Takase T, Baba T, Uyeno K. Chromomycosis. A case with a widespread rash, lymph node metastasis and multiple subcutaneous nodules. Mycose 1988; 31: 343-352. ' Musella RA, Collins GH. Cerebral chromoblastomycosis. J Neurosurg 1971; 35: 219-222. ' Tuffanelli L, Milburn PB. Treatment of chromoblastomycosis. J Am Acad Dermatol 1990; 23: 728-732.
180
AUSTRALIAN DERMATOPATHOLOGY SOCIETY DIGITAL DILEMMA KURT GEBAUER
Royal Perth Hospital A sixty year old male caucasion presented with a ten year history of a lesion on his left index finger. The lesion first developed whilst he was working as a deep sea fisherman off the east coast of Australia. It had slowly increased in size and become more nodular. There was no significant past history and he was not taking any medications. Examination showed a hyperkeratotic and indurated plaque on the left index finger. There were dermal nodules singly and in groups that extended proximally to the level of the metacarpophalangeal joint (Fig 1).
A skin biopsy was taken for histopathological examination and culture. Prepared sections show hyperkeratosis and marked irregular epidermal hyperplasia. In the dermis there are infiltrates of chronic inflammatory cells. Also present are granulomas composed of epithelioid cells and multinucleated giant cells. Some granulomas have a small amount of central suppuration. Rounded brown fungal spores are seen within giant cells and lying free in the dermis (Fig 2). They are also present in keratinous material within hair follicles. FIGURE 1—Granulomatous and
hyperkeratotic changes of finger.
FIGURE 2—Histopathological appearance.
179
KURT GEBAUER CHROMOBLASTOMYCOSIS
Cultured material grew Phialophora verrucosa. Initial management was with Itraconazole in a dose of 300 mg per day. Clinically the lesions responded rapidly, losing their hyperkeratinisation and nodularity. We were unable to increase the dose to 400 mg per day due to gastric intolerance. At all times his biochemical and haematological indices remained normal. Repeat biopsy after eight months of therapy demonstrated multiple fungi in the dermis. The fungus was cultured and found to be sensitive to Itraconazole, though not to Amphotericin B and 5-Flucytosine. The fungus was also sensitive to Ketoconazole. However, four years previously the patient had been treated with a twelve month course of Ketoconazole 200 mg daily and the lesion had increased in size during this treatment. With these results it was decided to treat clinically apparent granulomas with liquid nitrogen cryotherapy using the freeze/double freeze technique. Each granuloma was frozen for at least thirty seconds. Clinically a good response was apparent with minimal scarring. Unfortunately the patient was lost to follow-up when he returned to the Eastern States. Discussion Chromoblastomycosis is a chronic cutaneous and subcutaneous infection of the skin caused by pigmented fungi. It is believed that infection arises after traumatic implantation of fungal organisms.' These fungi are commonly found growing in soil, decaying vegetation and rotting woods. ^ Infection is most common in the tropical and subtropical climates and it occurs rarely in Australia. There is no racial predisposition. Most cases are seen in male agricultural workers between the ages of 30 and 50.'' Children rarely develop the disease. Lesions first begin as nodules that ulcerate and spread gradually, forming large exophytic, keratinous masses on the skin surface. These persist, enlarging slowly for many years and can heal with scarring and keloid formation. Lesions are nearly always localised to the skin and subcutis. There have been rare reports of haematogenous spread, lymphatic metastases and brain abscesses."'* The most common aetiological agents are species of Phialophora, Cladosporium and Fonsecaea. Wangiella dermatitidis has been isolated on a few occasions." All these fungi have been cultured from lesions presenting within Australia. The histopathological findings are of foreign body granulomas with occasional abscess
formation. The distinct round fungal spores, also known as copper penny bodies, can be easily seen in the tissues (Fig 2). Granulomas are often seen in association with marked pseudo-epitheliomatous hyperplasia.''^ When cultured on 2% glucose Sabouraud medium the colonies appear as black spots which develop into dark brown, grey or green cup shaped plaques. They can be differentiated by their type of spore formation and fine detail of spore structure.'^ If it is possible, complete surgical excision of all lesions is curative." Cryotherapy or destruction by cautery of diathermy can be used for small lesions. Amphotericin B given intravenously or intralesionally has also been reported with success. However, when used systemically fungicidal concentrations are not obtainable in vivo. Intralesional injection of Amphotericin B is very painful and not suitable for large areas. 5-Flucytosine treatment has been reported as effective, but unfortunately the responsive fungi rapidly develop resistance and therefore it is best given in association with another active agent. Oral Thiabendazole in a dosage of 25 mg per kilogram of body weight for many months has resulted in reported cure rates of 30 to 40%. Most recently Itraconazole has been recommended as the agent of choice.' In our case Itraconazole was given in higher doses than recommended for eight months. This resulted in clinical improvement of the lesions, but biopsy and culture still showed large numbers of fungal bodies in the tissue. It may be that perseverance with high doses for extended periods of time would be required to cure the infection. Acknowledgement
Colour printing in this presentation has been supported by a grant from the Australian Society of Dermatopathology. References ' Vollum DI. Chromomycosis: a review. Br JDermatol 1977; 96: 454-458. ^ Okeke CN, Gugnani HC. Studies on pathogenic dematiaceous fungi. 1. Isolation from natural sources. Mycopathologia 1986; 94: 19-25. ' McGinnis MR. Chromoblastomycosis and phaeohyphomycosis. New concepts, diagnosis and mycology. JAm Acad Dermatol 1983; 8: 1-16. ' Azulay RD, Serruya J. Hematogenous dissemination in chromoblastomycosis. Arch Dermatol 1967; 95: 57-60. ' Takase T, Baba T, Uyeno K. Chromomycosis. A case with a widespread rash, lymph node metastasis and multiple subcutaneous nodules. Mycose 1988; 31: 343-352. ' Musella RA, Collins GH. Cerebral chromoblastomycosis. J Neurosurg 1971; 35: 219-222. ' Tuffanelli L, Milburn PB. Treatment of chromoblastomycosis. J Am Acad Dermatol 1990; 23: 728-732.
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