Girl’s papular rash will not respond to treatment

The Case

The mother of a healthy 5-year-old girl brings her to the office for evaluation of 2 itchy plaques on her right ankle that have worsened over the last 10 days despite treatment for possible tinea corporis with topical clotrimazole 1% ointment and hydrocortisone 2.5% ointment (Figure). A fungal culture is pending.

Diagnosis: Majocchi's granuloma

Discussion

Majocchi’s granuloma was first described in 1883 by Professor Domenico Majocchi (1849-1929) in Italy.^1-3 Majocchi’s granuloma is a dermatophyte infection of the dermal and subcutaneous tissue characterized by perifollicular papular, pustular, and/or nodular lesions with granuloma formation.¹ This clinical variant of tinea corporis is uncommon because of the deep involvement of keratinophilic fungi that typically inhabit superficial epidermal layers.³ The organism responsible for most Majocchi’s granuloma formation is Trichophyton rubrum, but other fungi such as Trichophyton violaceum, Trichophyton mentagrophytes, Microsporum gypseum, Microsporum canis, and Microsporum audouinii also have been implicated.^1,4

More: Acute pruritic eruption on a child's face and hands

Two forms of Majocchi’s granuloma-a perifollicular papular form and a deep nodular form-have been described in the literature.^3,5,6 Lesions typically are found on the legs, forearms, hands, scalp, or face, although they can occur on any area of skin with hair.^2,3

The small perifollicular papular form occurs in immunocompetent patients, and is often associated with physical trauma, such as shaving or scratching, that disrupts the hair follicle.⁵ This trauma breaks the skin barrier, providing superficial infections access to the subcutaneous tissue and follicle, creating a chronic inflammatory response.⁶ The deep subcutaneous nodular form can develop in immunocompromised patients, including organ transplant recipients, patients with human immunodeficiency virus (HIV) infections, or those receiving immunosuppressive treatments, such as cyclophosphamide, vincristine, or long-term steroids.² These immunocompromised patients are at risk for disseminated infection.

Topical steroids also have been implicated in the development of Majocchi’s granuloma in immunocompetent hosts.^5,6 Steroids may advance dermatophyte growth by altering local cell-mediated immunity.⁵ Steroid activity may overcome the ability of antifungal agents when used together in a combination cream or concurrently.²

Differential diagnosis

The differential diagnosis for Majocchi’s granuloma is broad, and includes bacterial folliculitis, tinea incognito, contact dermatitis, and furunculosis, among other conditions.⁶ Diagnostic methods consist of potassium hydroxide (KOH) preparation, gram stain, tissue culture, or tissue biopsy. Because of the deep nature of the infection, fungal surface cultures and KOH preparations are not always diagnostic. Histopathologic examination of biopsy samples typically shows significant inflammation of hair follicles, dermal abscesses, and perifollicular granuloma formation. More extensive abscess formation and tissue necrosis is seen in immunocompromised patients.

Management

Resolution can rarely be achieved with topical antifungal agents because of poor penetration of the subcutaneous tissue.² The standard treatment is systemic antifungal medications, such as terbinafine, itraconazole, or griseofulvin. These medications are given for 2 to 4 weeks, although a longer course may be required for griseofulvin.^2,3,6 Immunocompromised patients may require a prolonged treatment course or even surgical excision.²

Patient outcome

The patient was advised to discontinue the hydrocortisone ointment, and to continue with clotrimazole 1% ointment. Hydroxyzine was prescribed as needed for pruritus. The fungal culture obtained on initial evaluation grew no yeast or fungal elements. Because of poor response to topical antifungal treatment, she was transitioned to griseofulvin suspension. She quickly had improvement in symptoms, with complete resolution of the lesions after 30 days of treatment.

Next: Pruritic vesicles erupt on a boy's legs

The development of Majocchi’s granuloma in this patient was likely attributed to physical trauma at the site secondary to scratching, and reduced local immunity secondary to steroid use. It is important to develop suspicion for Majocchi’s granuloma when a papular or nodular rash is not responding to treatment.¹ Also, the use of antifungal treatments and topical steroids in combination should be avoided, as steroids can lead to local immunosuppression and progression of a dermatophyte infection.² Alternative antipruritic agents should be considered for patients with fungal lesions suffering from pruritus.

REFERENCES

1. Kannan IC, Santos TB, Kac BK, Soiuza AM, Cerqueira AM. Majocchi’s granuloma-case report. An Bras Dermatol. 2015;90(2):251-253.

2. Feng WW, Chen HC, Chen HC. Majocchi’s granuloma in a 3-year-old boy. Pediatr Infect Dis J. 2006;25(7):658-659.

3. Parmar NV, Asir GJ, Rudramurthy SM. Atypical presentation of Majocchi’s granuloma in an immunocompetent host. Am J Trop Med Hyg. 2017;96(1):1-2.

4. Smith KJ, Neafie RC, Skelton HG 3rd, Barrett TL, Graham JH, Lupton GP. Majocchi’s granuloma. J Cutan Pathol. 1991;18(1):28-35.

5. Li FQ, Lv S, Xia JX. Majocchi’s granuloma after topical corticosteroids therapy. Case Rep Dermatol Med. 2014;2014:507176.

6. Ilkit M, Durdu M, Karakaş M. Majocchi’s granuloma: a symptom complex caused by fungal pathogens. Med Mycol. 2012;50(5):449-457.