Majocchi Granuloma 

Updated: May 14, 2019
Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD 

Overview

Background

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]

In 1883, Professor Domenico Majocchi (1849-1929) first described this disorder, he called granuloma tricofitico.[6] 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.

Also see the Medscape article Tinea Corporis.

Pathophysiology

The pathophysiology of the fungal infection and defense mechanisms against superficial dermatomycosis has been studied.[7] 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.[8] 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.[9, 10, 11] However, these dermatophyte infections may also occur in patients with atopic dermatitis, probably because of their immunological susceptibility.[12]

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,[13] Trichophyton tonsurans,[10, 14] and E floccosum.[15] 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 recently 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.

 

Presentation

History

Patients may complain of nonpruritic solitary or multiple persistent papulopustules or plaques. The legs are common sites for Majocchi granuloma in young women who frequently shave. Patients may also complain of onychomycosis or tinea pedis.

Two clinical forms of Majocchi granuloma exist, follicular and subcutaneous nodular.

The follicular type is secondary to trauma or topical corticosteroid use. It commonly occurs in young women who repeatedly shave their legs. Long-standing immunosuppression with steroids certainly predisposes individuals to widespread dermatophytosis, a component of which may be follicular papules consistent with Majocchi granuloma.[16, 17]

The subcutaneous nodular type occurs in immunocompromised hosts such as persons with graft versus host disease, those who undergo bone marrow and organ transplantation, and those receiving long-term immunosuppressive medication for lymphoma, leukemia, and autoimmune diseases.[9] Whether trichophytic abscesses in neutropenic bone marrow transplant recipients are Majocchi granulomas is debatable because these patients lack specific cellular immunity. These granulomas may be widespread.[12] Pregnancy, with its inherent altered immune status, may represent a risk factor.[18]

Recurrent Majocchi granulomas may be linked with chemotherapy-induced neutropenia.[19]

Antibiotic use does not result in Majocchi granuloma because Majocchi granuloma is an atypical course of a fungal disease that may result from a modified local and/or systemic immune response or a damaged skin barrier.

The use of potent topical steroids, especially under occlusion[20] or on preexistent tinea, may predispose the patient to Majocchi granuloma.

Physical Examination

Majocchi granuloma or granuloma trichophyticum may develop on any hair-bearing area, but most often, the scalp, face,[21] forearms, hands, and legs are involved. It may sometimes involve the pubic area.[22] A superficial perifollicular form of Majocchi granuloma on the scrotum, caused by T rubrum, has been described.[23]

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.[24]

Majocchi granuloma may appear as a persistent cutaneous plaque in wrestlers and may be considered a type of tinea corporis gladiatorum.[25]

Complications

Uncommonly, scarring and alopecia may result from Majocchi granuloma. Widespread cutaneous disease and/or fungal septicemia are potential complications in patients who are immunocompromised.

 

DDx

Diagnostic Considerations

Also consider the following:

  • Tinea incognito
  • Tinea capitis [26]
  • Bacterial folliculitis (see the image below)
  • Herpetic folliculitis
  • Pseudofolliculitis barbae
  • Nodular scabies
  • Follicular mucinosis
  • Poikiloderma with atrophy
  • Telangiectasia without scaling
  • Papular rosacea
  • Indeterminate leprosy
  • Kerion

Tinea incognito may appear as eczemalike, seborrhoeic dermatitislike, pyodermalike, and folliculitis to alopecia on scalp, trunk, and limbs.[27] The latter form may be represent some patients with Majocchi granuloma.

Pseudomonas folliculitis. Courtesy of Hon Pak, MD. Pseudomonas folliculitis. Courtesy of Hon Pak, MD.

Differential Diagnoses

 

Workup

Approach Considerations

Majocchi granuloma seen together with severe dermatophytosis suggests assessment of immune function may be beneficial, possibly leading to a discussion of AIDS.[28]

Laboratory Studies

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.

Other Tests

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.

Histologic Findings

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.[29]

 

Treatment

Medical Care

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, 22, 25, 30, 31] 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.

Prevention

The avoidance of occlusion, topical steroids use, and leg shaving may prevent Majocchi granuloma.

Long-Term Monitoring

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.

 

Medication

Medication Summary

The two classes of antifungal medications most commonly used to treat dermatophyte infections are the azoles and the allylamines. Azoles inhibit lanosterol 14-alpha-demethylase, an enzyme that converts lanosterol to ergosterol, an important component of the fungal cell wall. Membrane damage leads to permeability problems and renders the fungus unable to reproduce. Allylamines inhibit squalene epoxidase, an enzyme that converts squalene to ergosterol, leading to the accumulation of toxic levels of squalene in the cell and cell death. Examples of both classes of antifungal antibiotics are available for topical and systemic administration. Another possible therapy for refractory Majocchi granuloma is voriconazole.[32]

To achieve the best results, particularly with follicular or extensive disease, the authors often recommend a combination of topical and systemic therapy.

Antifungal agents

Class Summary

The mechanism of action may involve an alteration of RNA and DNA metabolism or an intracellular accumulation of peroxide that is toxic to the fungal cell.

Terbinafine (Lamisil)

Terbinafine is an allylamine derivative that inhibits squalene epoxidase, a key enzyme in sterol biosynthesis in fungi. This inhibition results in a deficiency in ergosterol within the fungal cell wall that causes fungal cell death. Terbinafine is available by prescription only. Some clinicians reserve the use of this drug for more widespread and/or resistant infections because of its broad coverage and cost. This medication is effective and well tolerated in children.

Butenafine (Mentax)

Butenafine is a potent antifungal related to the allylamines. It damages fungal cell membranes, arresting fungal cell growth. Butenafine is available in cream form only. Use 1% cream.

Clotrimazole (Lotrimin, Mycelex)

Clotrimazole is a broad-spectrum agent that inhibits fungal growth by altering cell membrane permeability, causing fungal cell death. Reevaluate the diagnosis if no clinical improvement is seen after 4 weeks. Often, clotrimazole is a first-line topical drug. It is available by prescription only. Cream, solution/spray, and lotion forms are available. Use 1%.

Itraconazole (Sporanox)

Itraconazole has fungistatic activity. It is a synthetic triazole antifungal agent that slows fungal cell growth by inhibiting cytochrome P-450–dependent synthesis of ergosterol, a vital component of fungal cell membranes.

Absorption is improved with food and in the presence of normal gastric acidity. Patients should be cautioned against ingesting grapefruit juice while on itraconazole therapy (decreased oral bioavailability of itraconazole).