Majocchi’s granuloma: a symptom complex caused by fungal pathogens

Medical Mycology July 2012, 50, 449–457 Review Article Majocchi’s granuloma: a symptom complex caused by fungal pathogens MACIT İ LKIT*, MURAT DURDU† & MEHMET KARAKAއ *Division of Mycology, Department of Microbiology, ‡Department of Dermatology, Faculty of Medicine, University of Çukurova, Adana, and †Department of Dermatology, Faculty of Medicine, Baş kent University Adana Hospital, Adana,Turkey Keywords dermatophytes, immunodeficiency, tinea pedis, transplantation, Trichophyton rubrum, Tzanck smear Introduction Dermatophytic fungi compose three anamorphic (asexual, conidial, or imperfect) genera: Epidermophyton, Trichophyton, and Microsporum. Each genus includes several recognized species. These fungi are keratinophilic and colonize or infect the superficial keratinized tissues (the skin, nails, and hair) of humans and animals. The organisms are usually restricted to the non-living cornified layer of the epidermis and do not invade beyond the epidermis. In an immunocompetent host, these fungi are usually unable to penetrate into viable tissues [1]. There are four welldescribed forms of invasive dermatophytic infections: (i) Majocchi’s granuloma (MG), which is also known as nodular granulomatous perifolliculitis; (ii) deeper Received 30 November 2011; Received in final revised form 6 February 2012; Accepted 21 February 2012 Correspondence: Macit İlkit, Division of Mycology, Department of Microbiology, Faculty of Medicine, University of Çukurova, Adana, 01330, Turkey. Tel.: ⫹90 532 286 00 99; Fax: ⫹90 322 457 30 72; E-mail: © 2012 ISHAM dermatophytosis; (iii) disseminated dermatophytosis; and (iv) mycetoma and pseudomycetoma caused by dermatophytes [2–5]. MG was first described in 1883 by Professor Domenico Majocchi (1849–1929) as an intracutaneous or subcutaneous granulomatous inflammation that arose as a result of invasion by a dermatophytic fungus (T. tonsurans); he termed the condition ‘Granuloma tricofitico’ [2]. This review provides information regarding the different clinical presentations and underlying mechanisms of MG; additionally, accurate diagnostic and management strategies for microbiologists, dermatologists, and pathologists are discussed. For this review, PubMed (Medline) and Google Scholar were searched for clinical and mycologic studies published in English (prior to July 2011) using the key words ‘Majocchi’s granuloma’, ‘trichophytic granuloma’, and ‘dermatophytic granuloma’. The reports retrieved within these search criteria were reviewed for inclusion in the study. However, papers concerning other forms of invasive dermatophytic infections, such as deeper or disseminated dermatophytosis, mycetoma, and pseudomycetoma, were excluded. DOI: 10.3109/13693786.2012.669503 Majocchi’s granuloma (MG) is a well-recognized but uncommon infection of dermal and subcutaneous tissues that is caused by mold fungi. Although primarily caused by keratinophilic dermatophytes such as anthropophilic Trichophyton rubrum, species from the Aspergillus and Phoma genera have been occasionally detected as etiologic agents of MG. In both healthy individuals and immunocompromised hosts, MG often presents as nodules, plaques, and papules on areas that are prone to trauma. Although MG generally appears on the upper and lower extremities (forearms, hands, legs, or ankles), it occasionally appears on the scalp and face. The clinical, mycologic, and/or cytologic diagnosis should be confirmed by the demonstration of perifollicular granulomatous inflammation by histologic examination. This review focuses on the clinical presentation, pathogenesis, laboratory diagnostic methods (including the Tzanck smear test), etiologic agents, histopathologic characteristics, and therapeutic approaches to the treatment of MG. 450 lİlkit et al. MG: an overview Fig. 1 (a) A 46-year-old immunocompetent man diagnosed with MG with well-demarcated, non-tender, indurated erythematous plaques, pustules, and crusts on the right shin; the lesion had been present for 4 weeks at the time of the photo. (b) Lesions were completely improved with the use of oral terbinafine (250 mg/day) and topical terbinafine cream for 4 weeks. Clinical manifestations In this study, 79 (48 men, 31 women) cases of MG were reviewed (Table 1) [7–10,12,13,17,19–49]. The mean patient age was 42 years (range, 3–87 years). The mean duration of the lesions prior to diagnosis was 10 months (range, 1 week–96 months). In the related literature, most cases with MG (62%) were immunocompetent patients. In the patient cohort that was reviewed, dermatophytic MG was characterized by inflammatory nodular (60.7%), papular (17.7%), or pustular (16.4%) lesions that generally occurred on the limbs (72%). Discrete or grouped papules (0.3–0.5 cm in size) and nodules (0.5–2 cm in size) can occur on the more active border of the erythematous plaques or alone; additionally, they can rarely be keloidal or verrucous in nature. The application of pressure does not usually cause the lesions to extrude pus. Unlike kerions, MG lesions do not suppurate until late in their course, unless secondary impetigo occurs. Pustules (16.4%) and crusts (4.1%) are observed on the erythematous plaques. Red-purple or occasionally brown papular and nodular lesions may resolve spontaneously without cutaneous scarring; however, lesions may result in eventual atrophic and hypertrophic scar formation [7,50]. The features of cellulitis, such as indurated plaques without papules, nodules, or pustules, are observed in 5.4% of all cases. Although subjective complaints are usually not reported, pruritus (10.9%) and slight tenderness following the application of pressure (9.5%) have been observed. Vulvar swelling was reported in one case [30]. MG may have a variable clinical presentation, such as abscess formation, especially when occurring in an immunodeficient host [10,16]. It was also reported that MG lesions on the right ankle of a patient became worse during pregnancy [9]. Importantly, our review of the literature found that nodular lesions were reported in 65.3% of MG cases in healthy individuals, 58.1% of which were nodular lesions that were not associated with papular lesions. Nodular lesions were detected in 53.3% and 65.3% of cases in immunosuppressed hosts and healthy individuals, respectively. For papular lesions, incidence rates of 23.3% and 14.3% were reported in immunosuppressed hosts and healthy individuals, © 2012 ISHAM, Medical Mycology, 50, 449–457 MG is a fungal disease that may result from a modified local and/or systemic response or a damaged skin barrier [6–8]. Dermatophytic MG is characterized by the presence of inflammatory papular, pustular, or nodular lesions, usually on the limbs [7]. Briefly, it is a folliculitic and perifolliculitic dermatophyte infection of the dermis [9,10]. There are two forms of MG: (i) the small, perifollicular papular form, which is a localized dermal infection that usually occurs in healthy individuals (Fig. 1 (...truncated)