Pulmonary phaeohyphomycosis due to Xylohypha bantiana

We encountered a rare case of pulmonary phaeohyphomycosis due to Xylohypha bantiana documented by culture. This dematiaceous (darkly pigmented) fungus is primarily neurotropic. It usually produces phaeohyphomycosis of the central nervous system but may also involve the skin and subcutaneous tissues. The patient, a 49-year-old woman with a history of steroid-treated inflammatory bowel disease, was found to have a lung nodule consisting of granulomas that contained dark hyphal fragments that stained positively with the argentaffin reaction. Surgical excision was curative and appears to be the treatment of choice.     

Cladophialophora bantiana brain abscess in a solid-organ transplant recipient: case report and review of the literature

Cerebral phaeohyphomycosis caused by Cladophialophora bantiana is a rare disease. We describe a heart and bilateral lung transplant recipient who was unsuccessfully treated for a C. bantiana brain abscess. This report compares the present case to those of other solid-organ transplant recipients with the same infection and to those of patients who did not receive transplants.     

A case of systemic lupus erythematosus complicated with psoriasis vulgaris]

A 49-years-old female admitted to our hospital because of skin eruptions on the extremities in 1985. She had suffered from polyarthralgia, skin eruptions since 1983. Physical examinations revealed discoid lesion, central nervous system involvement, and polyarthritis. Laboratory tests revealed leukopenia, thrombocytopenia, and hypocomplementemia. Antinuclear antibody, ant-DNA antibody, LE test were positive. From these findings, she was diagnosed as systemic lupus erythematosus (SLE). She developed lupus peritonitis in 1990 and 1994, which was successfully treated by steroid pulse therapy. Since then, the activity of SLE was in good control under administration of prednisolone 10 mg/day. Chilblain lupus was seen from 1993, Raynaud's phenomenon from 1996, and she further developed subcutaneous induration on her chest, back and upper extremities in 1999. Skin biopsy findings were compatible with lupus panniculitis. In 2002, erythematous patches with scales were observed on her right hand and left knee, and these skin lesions were histologically diagnosed as psoriasis vulgaris. An autoimmune response similar to SLE is speculated in psoriasis. We describe a rare case of SLE with various skin lesions including psoriasis vulgaris.     

Aspiration cytology of brain abscess from a fatal case of cerebral phaeohyphomycosis due to Ramichloridium mackenziei

Ramichloridium mackenziei is a dematiaceous fungus that usually causes cerebral phaeohyphomycosis. We describe the aspiration cytology findings of a case of cerebral abscess caused by R. mackenziei in a 66-yr-old Saudi woman who had a long standing history of diabetes mellitus and a recent diagnosis of systemic lupus erythematosus. She was on long-term corticosteroid therapy. The patient developed rapidly progressive multiple brain abscesses and died despite aspiration of the abscess and administration of intravenous amphotericin B lipid complex and voriconazole.     

Phaeohyphomycosis in a snow leopard (Uncia uncia) due to Cladophialophora bantiana

Phaeohyphomycosis caused by Cladophialophora bantiana was diagnosed in a 5-month-old snow leopard with spastic paralysis of the hind legs and inability to defaecate or urinate. At post-mortem examination, a greenish soft mass resembling an abscess was found on one side of the epidural space at the fourth lumbar vertebral body. Histological examination revealed a purulent meningitis with myelomalacia. Dematiaceous fungal hyphae, present within the inflammatory infiltrate, were identified as C. bantiana by culture and sequence analysis of the 18S ribosomal RNA gene. This neurotropic fungus rarely affects organs other than the brain in human beings and cats, and has been reported only occasionally in Europe. The case described suggests that phaeohyphomycosis due to C. bantiana infection may be recognized more frequently in the future and the possible involvement of organs other than the brain should be borne in mind.     

Brain abscess due to Cladophialophora bantiana

Cladophialophora bantiana (Xylohypha bantiana) is a dematiaceous fungus with distinct neurotropism. CNS phaeohyphomycosis due to C. bantiana is an uncommon infectious condition and is associated with high mortality. We report this rare clinical entity in a 22-year-old male, presenting as brain abscess. Etiological diagnosis was made based on fungal culture and histopathological examination. Complete surgical resection of the lesion and treatment with antifungal agents could not save the patient from this frequently fatal infection.     

Cerebral phaeohyphomycosis in an immunodeficient child treated medically with combination antifungal therapy.

Cerebral phaeohyphomycosis is a rare fungal infection with a poor prognosis when using conventional antifungal therapy in the absence of neurosurgical intervention. We present a case of a pediatric patient with inoperable Cladophialophora bantiana cerebral abscesses. To our knowledge, this child's case is the first reported to be treated with the combination of the newer triazole voriconazole and the new echinocandin caspofungin. Although our patient subsequently died, the natural rapid progression of the disease seemed to be altered by the antifungal combination alone, in the absence of surgery. Despite the fatal outcome for our patient, we encourage other clinicians to try unique medical approaches for this historically life-threatening infection when adjunctive surgery is impossible.     

Cladosporium trichoides cerebral phaeohyphomycosis in a liver transplant recipient. Report of a case

Cerebral phaeohyphomycosis (also referred to as cerebral chromomycosis), one of the diseases caused by the dematiaceous (black) fungi, is most commonly caused by Cladosporium trichoides (referred to by some as Xylohypha bantiana) and is a rare disease, with 31 culture-proven cases reported to date. Although most cases have occurred in immunocompetent hosts, recent experimental evidence suggests that host immunosuppression may predispose patients to the disease. The authors report a case of fatal cerebral phaeohyphomycosis in a liver transplant patient, the first to occur in a transplant patient of any type, to the best of the authors' knowledge. This case provides support for the hypothesis that immunosuppressed patients may be at increased risk for development of this disease.     

A case report; systemic lupus erythematosus associated with lupus laryngitis]

Systemic lupus erythematosus (SLE) is an autoimmune collagen vascular disease which produces widespread damage to multiple organs. Few studies on laryngeal involvement in SLE have been reported. We report here a case of SLE complicated by lupus laryngitis. A 27-year-old woman was diagnosed as having SLE in October 1996 based on findings of polyarthritis, lymphocytopenia, positive anti-nuclear antibody and anti-Sm antibodies. Polyarthralgia disappeared transiently, and no other clinical symptoms were found. Therefore, she was followed with no medication. She presented hoarseness with high fever and facial erythema in September 1998. Laboratory findings on admission revealed pancytopenia and hypocomplementemia. Anti-nuclear antibody, anti-Sm antibody and anti-RNP antibody were all positive with high titers. Chest X ray examination showed pleural effusion. Laryngoscopy showed a bamboo-joint-like lesion at the middle of the bilateral vocal cords. She was treated with 60 mg of prednisolone (i.v.) which resulted in improvement of hoarseness, pancytopenia, hypocomplementemia and pleuritis. Based on these findings, we diagnosed her hoarseness as the manifestation of laryngitis associated with SLE (lupus laryngitis).     

A case of systemic lupus erythematosus which Parkinsonism was induced by tacrolimus

The present case is the first report of a systemic lupus erythematosus patient which has been induced Parkinsonism with the administration of tacrolimus (TAC). A 50-year-old woman was diagnosed as lupus nephritis on September 2003. The patient had been prescribed initially 40 mg/day of prednisolone, then cyclosporine was added on May 2005. One year later, she developed severe headache, so cyclosporine was stopped, and she was prescribed tacrolimus on February 2007. However her severe headache had been disappeared, she experienced rigidity and tremor around September 2007. The Dopamine-transporter-imaging examination reavealed that she had Parkinson's disease. The gene analysis on the genetic background showed her case was the sporadic type? Parkinson's disease. Washing out of Tacrolimus, her Parkinsonism was partially improved. This fact suggested that her Parkinsonism was drug-induced type Parkinsonism. In lupus nephritis patients who have been treated with TAC, a very careful observation should be considered because neurological disorders inducing Parkinsonism may occur.     

Successful bosentan therapy in a case of pulmonary arterial hypertention developed during immunosuppressive therapy for lupus nephritis

We report a 43-year-old female who developed pulmonary arterial hypertension (PAH) during intensive immunosuppressive therapy for systematic lupus erythematosus (SLE). She was diagnosed as SLE at the age of 32 years based on serological and hematological abnormalities, oral ulcers, and facial erythema. She experienced frequent flare-ups of disseminated discoid lupus between the ages of 33 and 36 years and developed immune thrombocytopenia at the age of 39 years. In 2007 when she was 43 years old, she developed lupus nephritis (LN) with elevated serum anti-double stranded DNA antibodies and urine protein of less than 1 g/day. Combination therapy for the LN with 35 mg/day prednisolone and intravenous cyclophosphamide (IVCY) led to renal remission. After the seventh monthly session of IVCY, she developed dyspnea on exertion. PAH was diagnosed based on enlarged main pulmonary arteries on the chest x-ray, right ventricular outflow and a peak tricuspid regurgitant pressure gradient exceeding 45 mmHg on echocardiography, an elevated plasma brain natriuretic peptide (BNP) level of 260 pg/ml, the exclusion of pulmonary thromboembolism, and no lung fibrosis. The PAH was treated successfully with bosentan. At present the tricuspid regurgitation has disappeared, and the plasma BNP level has normalized.     

A case report of hypertrophic pachymeningitis associated with systemic lupus erythematosus, showing a headache and hearing loss resembling intracranial hypotension]

We report a case of systemic lupus erythematosus (SLE) complicated with hypertrophic pachymeningitis. A 34-year old woman who was diagnosed as SLE in 1985 was admitted to our hospital for a high grade fever and a headache. Laboratory findings showed increased titer of anti-double strand DNA antibody and decreased number of platelets. She complained a severe headache and hearing loss which were worsened by head-up position, resembling the symptoms of intracranial hypotension. MRI findings revealed thickened dura and she was diagnosed as hypertrophic pachymeningitis. Both clinical symptoms and laboratory findings were resolved after methyl-prednisolone pulse therapy followed by a high dose of prednisolone. Although hypertrophic pachymeningitis is a rare complication with SLE, it should be considered in SLE patients with severe headache.     

Subcutaneous phaeohyphomycosis in transplant recipients: review of the literature and demonstration of in vitro synergy between antifungal agents.

Infection of subcutaneous tissue by black fungi (subcutaneous phaeohyphomycosis) has only been reported in six transplant patients, all of whom were solid organ recipients. These patients presented with indolent, localized infections at least 1 year after transplant, while on maintenance immunosuppressive regimens. They were cured by surgical resection, either alone or in conjunction with antifungal agents. We report a case of subcutaneous phaeohyphomycosis occurring in a bone marrow transplant recipient receiving high doses of immunosuppressive agents, in whom widespread subcutaneous infection due to Exophiala jeanselmei was not eradicated by repeated resections and therapy with amphotericin B and flucytosine. The infection was eventually cured after addition of itraconazole to the therapeutic regimen. Results of in vitro testing of the isolate for susceptibility to a combination of amphotericin B, flucytosine and itraconazole confirmed the potential role of combination antifungal therapy in the setting of refractory infection.     

Subcutaneous phaeohyphomycosis: a histopathological study of nine cases from Malawi.

The clinical and histopathological features of subcutaneous phaeohyphomycosis in nine patients from Malawi are presented. The patients had varied clinical presentations and microscopically, in each case, there was a subcutaneous abscess or cystic granuloma. In seven cases (78%) the causative fungal elements were easily identified in sections stained with haematoxylin and eosin. The fungal elements were easily identified in the other two cases with special fungus stains. The misleading terminology, which was used previously to cover this infection, is discussed. Even though this paper reports on this disease in a tropical country, subcutaneous phaeohyphomycosis occurs world wide, and these fungi should be looked for in otherwise unexplained subcutaneous abscesses or cystic granulomata.     

Cerebral phaeohyphomycosis due to Cladophialophora bantiana in a French Guianese child

We report a case of cerebral phaeohyphomycosis, a fungal brain infection due to a dark (dematiaceous) fungi in a 6-year-old French Guyanese boy. The child presented fever and drowsiness due to several paraventricular brain abscesses. Neurological surgeries were performed to reduce intracranial hypertension and to obtain abscess biopsies. Mycological cultures of intraoperative samples led to the diagnosis of cerebral phaeohyphomycosis due to Cladophialophora bantiana. The patient neurological status deteriorated and remained critical after several weeks of combination antifungal therapy with voriconazole 8 mg/kg/day, liposomal amphotericin B 10 mg/kg/day and flucytosine 200 mg/kg/day. A complete surgical resection was not possible because of multiple small abscesses. A multidisciplinary ethical staff decided on home medical care with palliative ventriculoperitoneal shunt, nasogastric feeding and analgesics. One year later, the patient's neurological condition had improved and cerebral lesions had regressed, while he had not received any antifungal treatment but only traditional medicines. Cerebral phaeohyphomycosis are rare diseases affecting immunocompromised but also apparently non-immunocompromised patients, as in this case. A complete surgical resection is not always possible and mortality rates are high in spite of treatments with a combination of antifungals. The diagnosis may be difficult because of these dematiaceous fungi's slowly growing and their potential pathogenicity for laboratory staff.     

Multifocal subcutaneous phaeohyphomycosis caused by Phialophora verrucosa

An otherwise healthy 85-year-old woman presented with purulent multifocal subcutaneous nodules on the dorsal side of the right forearm and hand. Histopathologic examination of the biopsied specimen showed a subcutaneous granuloma with central abscess and necrosis, consistent with phaeomycotic cyst. Faint brown septate hyphae and moniliform fungal elements were found in the granuloma. Culture of the discharge grew Phialophora verrucosa. This fungus has rarely been reported as a pathogen of subcutaneous phaeohyphomycosis. Subcutaneous phaeohyphomycosis usually presents as a single lesion. In this case of multifocal lesions, initial imperfect excision seems to have caused satellite lesions. To our knowledge, this is the first report of multifocal subcutaneous phaeohyphomycosis caused by P verrucosa. The disease responded to oral itraconazole administration.     

A Light and Electron Microscopical Study of Membranocystic Lesions in a Case of Lupus Erythematosus Profundus

A light and electron microscopical study on membranocys-tic lesions (MCL) in a case of lupus erythematosus profundus (LEP) is reported. The patient was a 16 year old female who presented with subcutaneous nodules on both upper arms. The light microscopic features were consistent with LEP, and the result of an immunofluorescence band test supported this diagnosis. A peculiar finding in this case was MCL in the subcutaneous tissue. Ultra-structurally, these were thin membranes without a tubular structure and tortuous thick membranes composed of minute tubules. The lesions were very similar to the fatty tissue changes in membranous lipodystrophy. On the other hand, the basement membranes of the blood vessels were thickened and multilayered, and the Iumina were narrowed by endothelial swelling and thickening of the vessel wall. Our findings suggest that the MCL in LEP result from circulatory disturbance of the fat tissue.     

A light and electron microscopical study of membranocystic lesions in a case of lupus erythematosus profundus

A light and electron microscopical study on membranocystic lesions (MCL) in a case of lupus erythematosus profundus (LEP) is reported. The patient was a 16-year-old female who presented with subcutaneous nodules on both upper arms. The light microscopic features were consistent with LEP, and the result of an immunofluorescence band test supported this diagnosis. A peculiar finding in this case was MCL in the subcutaneous tissue. Ultrastructurally, these were thin membranes without a tubular structure and tortuous thick membranes composed of minute tubules. The lesions were very similar to the fatty tissue changes in membranous lipodystrophy. On the other hand, the basement membranes of the blood vessels were thickened and multilayered, and the lumina were narrowed by endothelial swelling and thickening of the vessel wall. Our findings suggest that the MCL in LEP result from circulatory disturbance of the fat tissue.     

Kikuchi's lymphadenitis (necrotizing lymphadenitis) and systemic lupus erythematosus: a case report

A 26-year-old Indian lady was admitted for lower abdominal pain, diarrhoea, vomiting, fever and cough. The initial diagnosis was that of peritonitis secondary to ruptured or perforated viscus with lobar pneumonia. On laparotomy, she was found to have necrotizing or Kikuchi's lymphadenitis of the abdominal lymph nodes. The initial two antinuclear antibody (ANA) results came back negative. She was diagnosed to have systemic lupus erythematosus (SLE) when the third sample for ANA came back positive and the double-stranded DNA (dsDNA) antibody test was homogenously positive. This case illustrates a need to be aware that necrotizing lymphadenitis can precede the onset of systemic lupus erythematosus.     

Clinical and serological manifestations associated with interferon-α levels in childhood-onset systemic lupus erythematosus

OBJECTIVE:

To determine the serum levels of interferon alpha in childhood-onset systemic lupus erythematosus patients, their first-degree relatives and healthy controls and to evaluate the associations between serum interferon alpha and disease activity, laboratory findings and treatment features.

METHODS:

We screened consecutive childhood-onset systemic lupus erythematosus patients in a longitudinal cohort at the pediatric rheumatology unit of the State University of Campinas between 2009 and 2010. All patients demonstrated disease onset before the age of 16. Disease status was assessed according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Interferon alpha levels were measured using an enzyme-linked immunoabsorbent assay.

RESULTS:

We included 57 childhood-onset systemic lupus erythematosus patients (mean age 17.33±4.50), 64 first-degree relatives (mean age 39.95±5.66), and 57 healthy (mean age 19.30±4.97) controls. Serum interferon alpha levels were significantly increased in childhood-onset systemic lupus erythematosus patients compared to their first-degree relatives and healthy controls. Interferon alpha levels were significantly increased in patients with positive dsDNA antibodies, patients with cutaneous vasculitis, patients with new malar rash and patients who were not receiving medication. Interferon alpha levels correlated with C3 levels and systemic lupus erythematosus Disease Activity Index scores. In addition, we observed an inverse correlation between patient age and interferon alpha levels.

CONCLUSION:

Interferon alpha may play a role in the pathogenesis of childhood-onset systemic lupus erythematosus, especially in cutaneous manifestations and dsDNA antibody formation. The observation that interferon alpha levels are increased in patients who are not taking medication should be investigated in longitudinal studies to determine whether elevated interferon alpha levels may predict systemic lupus erythematosus flares.