Identification of the various dematiaceous fungi responsible for black grain

Identification of the various dematiaceous fungi responsible for black-grain mycetoma remains difficult with standard mycologic procedures and can be delayed up to 12 weeks. Phenotypic and biochemical methods cannot distinguish E jeanselmei from E oligosperma. In addition, E spinifera may be confused with E jeanselmei due to similar early colony morphologic characteristics. Sequencing of the rDNA ITS1-5.8S-ITS2 gene has proven to be a useful molecular tool for reliable and rapid identification of most black-grain mycetoma agents.
Subcutaneous phaeohyphomycosis can occur in both immunocompetent and immunosuppressed patients, the latter being at greater risk of treatment failure and subsequent dissemination of the infection. Owing to the rarity of this infection, there are no clinical trials to guide the management of subcutaneous phaeohyphomycosis caused by E jeanselmei. The optimal antifungal agent in the treatment of E jeanselmei is still unknown. Historically, itraconazole and voriconazole have demonstrated the most consistent in vitro activity against Exophiala species. Severe infections due to E jeanselmei, including pneumonia, fungemia, central bcr-abl inhibitor infection, endocarditis, and peritonitis, have been reported in immunocompromised patients. Antifungal therapy is recommended until all signs and symptoms of infection have resolved. In Taiwan, three reported cases of subcutaneous phaeohyphomycosis caused by Exophiala jeanselmei were identified in the literature, and all three involved immunocompromised patients. Two cases were treated successfully with itraconazole plus either debridement or cryotherapy. Our case showed that surgical excision alone was sufficient to treat and cure subcutaneous phaeohyphomycosis caused by E jeanselmei in an immunocompetent host. Although cryotherapy seems effective for treatment of subcutaneous phaeohyphomycosis, it is unknown whether cryotherapy alone is a sufficient treatment in immunocompetent hosts.
In summary, E jeanselmei should be suspected in immunocompetent patients presenting with chronic subcutaneous lesions. The diagnosis can be made by histopathologic and microbiologic evaluation of tissue and exudate. Surgical excision alone appears to be an efficacious therapy in immunocompetent hosts.

Hereditary periodic (recurrent) fever syndromes are a group of autoinflammatory diseases characterized by recurrent episodes of unprovoked inflammation without high-titer autoantibodies or autoreactive T cells. Seven diseases exhibit Mendelian patterns of inheritance with identified single gene defects. They include familial Mediterranean fever (FMF), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), pyogenic sterile arthritis, pyoderma gangrenosum and acne (PAPA), hypergammaglobulinemia D with periodic fever syndrome (HIDS) and three overlapping conditions, the cryopyrinopathies, with common cryopyrin abnormalities: familial cold autoinflammatory syndrome (FCAS); Muckle-Wells syndrome (MWS); and neonatal-onset multisystem inflammatory disease (NOMID), also known as chronic infantile neurologic cutaneous articular syndrome (CINCA). Hereditary periodic fevers are characterized by recurrent flares of systemic inflammation presenting as sudden episodes of fever associated with a dramatic elevation of acute phase reactants and a number of clinical manifestations, such as rash, serositis (peritonitis, pleuritis), lymphadenopathy, and arthritis. Disease flares are usually separated by symptom-free intervals of variable duration, characterized by complete well-being, normal growth, and complete normalization of acute phase reactants. The clinical spectrum of these disorders is also extremely variable.
TRAPS is characterized by periodic fever (duration of more than 1 week), migratory cutaneous rash, conjunctivitis, periorbital edema, lymphadenopathy, abdominal pain, myalgia, arthralgia, and serositis. It is an autosomal dominant disease that is related to heterozygous mutations in the tumor necrosis factor (TNF) receptor super family 1A (TNFRSF1A) gene encoding TNF receptor type 1 (TNFR1). Levels of acute-phase reactants are elevated during attacks and also during asymptomatic periods. TRAPS is the second most common disease among hereditary periodic fever syndromes following FMF. Although people of any ethnicity may be affected, there have been few reports to date with regards to patients from Asia. Here, we presented the case of a 14-year-old Asian girl with recurrent fever and abdominal pain for 2 years who was finally diagnosed with TRAPS, and had good response to etanercept, an anti-TNF-α agent.