Are You Confident of the Diagnosis?
Majocchi’s granuloma is a rare clinical variant of tinea corporis. Tinea corporis is a dermatophyte infection of the skin and is generally restricted to the stratum corneum. Majocchi’s granuloma is a follicular and perifollicular dermatophyte infection of the deep dermis, and may be found in immunocompetent and immunocompromised people.
What you should be alert for in the history
In general, the patient will be an immunocompetent person with a history of chronic use of high-potency topical corticosteroids in a nondiagnosed dermatophyte infection. Another key to the diagnosis is to ask about previous shaving or waxing leading to the dissemination of the lesions. This occurs because the infected hair shafts facilitate the dermatophyte to penetrate the follicular wall.
Characteristic findings on physical examination
Patients will complain about papules, pustules, or even plaques and nodules at the infection site (Figure 1). The papules will be pink-red, and, along with the pustules, will be located in a perifollicular location. Hair shafts can be easily removed from the pustules and papules. Classically, patients will complain about many treatment attempts with poor results. Itching is very common.
Plaques, nodules, or abscesses are seen more often on the face, scalp, hands, or forearms of immunocompromised hosts. In these cases, it’s important to ask about associated diseases, such as lymphoma, AIDS, lymphopenia, and others, and dialysis, high doses of oral corticosteroids, and other immunosuppressive medications.
Expected results of diagnostic studies
The diagnosis of Majocchi’s granuloma can only be made in confidence by the demonstration of the fungus on potassium hydroxide (KOH) examination, histopathological examinations, or by culture. Histopathology demonstrates hyphae or arthrospores within expanded hair follicles, among keratin from the fragmentized hair. In the perifollicular area there will be a dermal infiltrate consisting of lymphoplasmacytic cells, and focal collections of epithelioid giant cells, neutrophils, and mild interstitial edema.
Afterwards, it’s mandatory to try to identify the agent. Direct smear exams (KOH) are often negative, but in many cases the culture in a Sabouraud agar can be enlightening (Figure 2, Figure 3). Evaluating the hair that is removed from the center of a papule or pustule with a KOH examination may be more sensitive.
We should differentiate this infection from bacterial folliculitis and other fungal infections with nodules and plaques such as chromoblastomycosis and sporotrichosis and even leishmaniasis. Other diagnoses that should be excluded are nontuberculous mycobacterial infections.
Who is at Risk for Developing this Disease?
Majocchi’s granuloma is more prevalent in tropical regions. It is related to occlusion, chronic use of high-potency topical corticosteroids, and to superficial trauma, and it is found in people who have chronic dermatophytosis, including onychomycosis or tinea pedis. Usually this variant can be detected in people who shave or wax their legs or other parts of the body.
What is the Cause of the Disease?
Any dermatophyte can cause Majocchi’s granuloma; however, the most common agent is Trichophyton rubrum (Figure 4). Rarely, other agents such as Trichophyton mentagrophytes, Trichophyton epilans, Trichophyton violaceum, Trichophyton tonsurans, Microsporum canis, Microsporum audouinii, Microsporum gypseum and Microsporum ferrugineum can cause the disease.
Other nondermatophytic fungi may cause infection in immunocompromised hosts. Majocchi’s granuloma results from the rupture of a dermatophyte-infected infundibulum, as a result of trauma.
Recently, a case of Majocchi’s granuloma related to the use of adalimumab (tumor necrosis factor alpha [TNF-alpha] inhibitor) was described.
Systemic Implications and Complications
When plaques, nodules, or abscesses are widespread and seen on the face, scalp, hands, or forearms in patients proven to have Majocchi’s granuloma, the possibility of an immunocompromised state should be considered. In such cases, it is important to ask about associated diseases, such as lymphomas, AIDS, lymphopenia, and others, and dialysis and high doses of oral corticosteroids.
Treatment options are summarized in Table I.
It is imperative to discontinue the use of topical steroids.
Optimal Therapeutic Approach for this Disease
Majocchi’s granuloma should be treated by oral antifungal agents. Topical medications are not as effective because the infection occurs deep in the dermis.
Itraconazole could be used in pulse therapy of 3 cycles of 200 mg, twice daily for 1 week with 2 weeks off between the pulses.
Other options are: terbinafine 250 mg daily or cotrimoxazole 480 mg daily, for at least 3 weeks, or griseofulvin 1g per day divided twice daily, given for 2 months.
Stop the use of all topical steroids.
Cryotherapy has been used as an adjuvant treatment in cases unresponsive to treatment with conventional antifungal medications.
We must explain to patients and their families that superficial trauma, such as scratching the fungal infection site (e.g., tinea pedis or corporis), may produce this disease and should be avoided. The lesions should remit after oral treatment, but rarely do so after topical treatment alone. For this reason, systemic treatment is preferred despite the greater risk of side effects.
Unusual Clinical Scenarios to Consider in Patient Management
In immunocompromised hosts, other nondermatophytic fungi could be implicated in Majocchi’s granuloma. There is a report of Aspergillus fumigatus as the agent in a patient with AIDS and a systemic fungal infection.
What is the Evidence?
Saadat, P, Kappel, S, Young, S, Abrishami, M. “Aspergillus fumigatus Majocchi’s granuloma in a patient with acquired immunodeficiency syndrome”. Clin Exp Dermatol. vol. 33. 2008. pp. 450-3. (Majocchi’s granuloma is usually caused by T. rubrum in immunocompetent and immunocompromised hosts. This text shows a case of Majocchi’s granuloma caused by Aspergillus fumigatus in a patient with AIDS.)
Saadat, P, Sobera, JO, Elewski, BE, Bolognia, JL, Jorizzo, JL, Rapini, RP. “Fungal disease”. vol. Vol 1. 2008. pp. 1135-62. (Majocchi’s granuloma is a clinical variant of tinea corporis caused by T. rubrum. It’s common in women with tinea pedis or onychomycosis who used to shave their legs.)
Gill, M, Sachdeva, B, Gill, PS, Arora, B, Deep, A, Karan, J. “Majocchi’s granuloma of the face in an immunocompetent patient”. J Dermatol. vol. 34. 2007. pp. 702-4. (Majocchi’s granuloma results of a rupture of infected infundibulum in a trauma. The most common site for Majocchi’s granuloma is the anterior aspect of the legs and the face is a rare site in an immunocompetent host. The authors didn’t find any risk factor for the disease but they thought maybe the use of a veil by the patient was a contribution.)
Gupta, KA, Groen, K, Woestenborghs, R, De Doncker, P. “Itraconazole pulse therapy is effective in the treatment of Majocchi’s granuloma: a clinical and pharmacokinetic evaluation and implications for possible effectiveness in tinea capitis”. Clin Exp Dermatol. vol. 23. 1998. pp. 103-8. (Topical agents are usually ineffective in Majocchi’s granuloma because the infection occurs in a deep location (dermis). In this text, the clinical and pharmacokinetic effects of pulse therapy treatment with itraconazole, 200 mg, twice a day for 1 week, with 2 weeks off was evaluated. The treatment was shown to be effective.)
Gupta, AK, Prussick, R, Sibbald, RG, Knowles, SR. “Terbinafine in the treatment of Majocchi’s granuloma”. Int J Dermatol. vol. 34. 1995. pp. 489-90. (Terbinafine 250 mg daily during 14 days in one case, and during 4 weeks in another case, was effective in the treatment of Majocchi’s granuloma.)
Chou, WY, Hsu, CJ. “A Case Report of Majocchi’s Granuloma Associated with Combined Therapy of Topical Steroids and Adalimumab”. Medicine 95.2. pp. e2245
Pietrzak, A, Tomasiewicz, K, Kanitakis, J, Paszkowski, T. “Trichophyton mentagrophytes-associated Majocchi’s granuloma treated with cryotherapy”. Folia Histochem Cytobiol. vol. 50. 2012 Oct 8. pp. 486-9.
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