Practice Essentials
Majocchi granuloma can be defined as a deep folliculitis due to a cutaneous dermatophyte infection. [1] Majocchi granuloma is most commonly due to Trichophyton rubrum infection. Majocchi granuloma tends to occur in young women who frequently shave their legs, although Majocchi granuloma also is seen in men. [2] Majocchi granuloma also commonly occurs as a result of the use of potent topical steroids on unsuspected tinea. Majocchi granuloma is also known as granuloma trichophyticum.
Many species of dermatophytes can cause Majocchi granuloma. Today, Majocchi granuloma is usually due to T rubrum [3, 4] ; however, Trichophyton violaceum was the most common organism identified historically. Other causes of Majocchi granuloma include Trichophyton mentagrophytes and Epidermophyton floccosum. [5]
Also see the Medscape article Tinea Corporis.
Signs and symptoms
Majocchi granuloma or granuloma trichophyticum may develop on any hair-bearing area, but most often, the scalp, face, [6] forearms, hands, and legs are involved. It may sometimes involve the pubic area. [7] A superficial perifollicular form of Majocchi granuloma on the scrotum, caused by T rubrum, has been described. [8]
Majocchi granuloma may begin as solitary or multiple well-circumscribed oval patches or as indistinct scaling ones. Majocchi granuloma evolves into perifollicular papulopustules and nodules with or without background erythema and scaling.
A plaque may demonstrate keloidal features, but these findings are unusual. Nodules are often clustered, but they can be solitary as well. Pressure does not result in pus exudation.
Unlike a kerion, granuloma trichophyticum does not become clinically suppurative until late in its course, unless secondarily impetigo develops.
If the cutaneous features of Majocchi granuloma are associated with the use of topical steroids, they may be affected by the complications of topical steroid therapy, including poikiloderma with atrophy and telangiectasia, papular rosacea, or a hypopigmented patch suggestive of indeterminate leprosy.
Majocchi granuloma may rarely resemble Kaposi sarcoma, as it does in patients with AIDS or lymphocytoma cutis. In such cases, Majocchi granulomas are painful and appear as blue-red papules and nodules on an erythematous base. [9]
Majocchi granuloma may appear as a persistent cutaneous plaque in wrestlers and may be considered a type of tinea corporis gladiatorum. [10]
Also see Presentation.
Diagnostics
Majocchi granuloma seen together with severe dermatophytosis suggests assessment of immune function may be beneficial, possibly leading to a discussion of AIDS. [11]
A potassium hydroxide (KOH) preparation of scales and pustules usually reveals no hyphal elements. Samples from a contiguous dermatophyte infection, if present, may stain positive. Gram stains, calcofluor stains, scale cultures, and exudate or tissue biopsy samples may reveal hyphae when the KOH test result is negative. In general, tissue homogenate cultures are more sensitive than special stains.
Majocchi granuloma is essentially a deep suppurative and granulomatous folliculitis. The earliest sign is hyphal invasion in the cornified keratinocytes of the hair follicle, which produces a suppurative folliculitis with the rupture of the hair follicle and the spillage of its contents into the dermis. This rupture causes a granulomatous dermal response. Such nodules may heal with fibrosis. Periodic acid-Schiff or Gomori methenamine-silver stains may reveal fungal hyphae in the tissue, surrounded by a foreign body granulomatous reaction. Use of optical brighteners may enhance detection of fungal elements in the deep dermis and may be more sensitive than with periodic acid-Schiff (PAS) staining. [12]
Management
Also see Medication.
Systemic antifungal treatment is preferred in both patients who are immunocompetent and in those who are immunocompromised. Treatment should last at least 4-6 weeks. Oral terbinafine has been used worldwide for Majocchi granuloma. [5, 7, 10, 13, 14, 15] The administration of systemic terbinafine for 6 weeks is the best treatment option in a patient with a transplanted kidney and Majocchi granuloma.
Oral antifungals are usually necessary because topical agents alone are not effective. For example, systemic antifungal medication is the best option for patients who are immunocompromised.
Physicians should avoid the use of compound products containing a potent topical steroid. Physicians should not use potent topical steroids to treat possible dermatophytic infections. Combination products such as betamethasone dipropionate with clotrimazole 1% cream should be used with care or not at all. The authors do not favor the use of such products in children younger than 12 years. If such medications are used in adolescents, the authors suggest doing so for only 2 weeks. Similarly, physicians should apply topical steroids with occlusion only when they are confident that the eruption is not a dermatophytosis, because this treatment may predispose the patient to Majocchi granuloma.
To the authors' knowledge, no specific data about treating immunocompromised patients with Majocchi granuloma exist. The treatment of secondary bacterial infections and the removal of any exacerbating factors (eg, topical steroid use, occlusion) are indicated.
Photodynamic therapy employing 5-aminolevulinic acid may be an effective therapeutic option for selected patients. [16]
The best objective measure in Majocchi granuloma is to observe the patient clinically. Culture and KOH specimens may be useful, but relapse occurs if the use of occlusion and topical steroids continues or is reinitiated.
The avoidance of occlusion, topical steroids use, and leg shaving may prevent Majocchi granuloma.
Background
In 1883, Professor Domenico Majocchi (1849-1929) first described this disorder, he called granuloma tricofitico. [17] He is also credited with describing a type of chronic pigmented purpura: purpura annularis telangiectodes, which is commonly known as Majocchi disease. Majocchi, an important figure in Italian academic dermatology, was a professor of dermatology first at the University of Parma and later at the University of Bologna.
Pathophysiology
The pathophysiology of the fungal infection and defense mechanisms against superficial dermatomycosis has been studied. [18] Two series of experimental infections of T mentagrophytes were made on the forearm of a male volunteer with topical steroid ointment and vehicle alone. Steroid ointment suppressed the immune reactions locally to produce little inflammatory reaction with abundant fungal elements (so-called atypical tinea) and a mixed cell granuloma.
While inflammatory tinea capitis or kerion is the result of a hypersensitivity reaction to a dermatophytic infection, Majocchi granuloma usually begins as a suppurative folliculitis and may culminate in a granulomatous reaction. [19] Nineteen cases of kerion of the scalp in children were evaluated. Histopathological findings demonstrated a spectrum from suppurative folliculitis to dense granulomatous infiltrates without a clear relationship with the clinical features.
Widespread trichophytic granulomas may occur in patients receiving immunosuppressive therapy for leukemia or lymphoma, autoimmune diseases, and post–organ transplantation. [20, 21, 22, 23] However, these dermatophyte infections may also occur in patients with atopic dermatitis, probably because of their immunological susceptibility. [24]
Etiology
Majocchi granuloma is a foreign body granuloma most commonly caused by T rubrum.T violaceum was the most common organism identified historically.
Other causes of Majocchi granuloma include T mentagrophytes, [25] Trichophyton tonsurans, [21, 26] and E floccosum. [27] The fungal infections may be due to or linked with a widespread contiguous dermatophytosis, immunosuppression, and/or the use of topical steroids.
Epidemiology
To the authors' knowledge, no specific data on the incidence and prevalence of Majocchi granuloma exist. A patient was described in Brazil. [2]
Prognosis
Majocchi granuloma cure is expected with appropriate systemic antifungal therapy. To the authors' knowledge, no data about relapse rates or the complications of not treating Majocchi granuloma exist.
Patient Education
Patients should be educated about the cause of Majocchi granuloma, as well as the predisposing and exacerbating factors.
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Pseudomonas folliculitis. Courtesy of Hon Pak, MD.
