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Purpura annularis telangiectodes

Majocchi's disease (also known as Majocchi's Granuloma, "Purpura annularis telangiectodes,", and "Purpura annularis telangiectodes of Majocchi") is well-recognized but uncommon skin condition characterized by purple/bluish-red 1- to 3-cm annular patches composed of dark red telangiectases with petechiae. The name Majocchi's comes from the Professor Domenico Majocchi who first discovered the disorder in 1883. Domenico Majocchi was a professor of dermatology at the University of Parma and later the University of Bologna. Majocchi's disease can be defined as a infection of the dermal and subcutaneous tissues due to a fungal mold infection on the cutaneous layer of the skin. The most common dermatophyte is called Trichophyton rubrum. This disease can affect both immunocompetent and immunocompromised hosts. However, immunocompromised individuals have a higher risk.

Mechanisms

Historically, there have been many types of dermatophytes that were known to cause Majocchi's granuloma. Trichophyton violaceum used to be one of the most common species of dermatophytes to cause this disease. Today, however, Trichophyton rubrum proves to be the main culprit is most cases. 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. The fungi are usually unable to penetrate into viable tissues in a immunocompetent host and therefore many times the infection incidence is higher in immune compromised individuals. There are two forms of MG: (i) the small, perifollicular papular form, which is a localized dermal infection that usually occurs in healthy individuals and (ii) the form featuring deep subcutaneous plaques or nodular lesions that occur in immunosuppressed hosts. Tinea corporis is the name of the subset of this disease that only remains restricted to the stratum corneum. Otherwise, the atypical deeper involvement is known as Majocchi's granuloma. Because keratinophilic dermatophytes digest keratin, the introduction of keratin into the dermis may also act as a medium for continued growth of the organism.

Symptoms

In both healthy and immunocompromised hosts, Majocchi's granuloma often presents as nodules and papules on areas that are most exposed to mechanical abuse--wear and tear--such as the upper and lower extremities. Patients will complain about papules, pustules, or even plaques and nodules at the site of infection. The papules will be pink-red and will be located in a perifollicular location. Hair shafts can be easily removed from the pustules and papules. Itching is also very common. Firm or fluctuant subcutaneous nodules or abscesses represent a second form of MG that is generally observed in immunosuppressed hosts. Nodules may develop in any hair-bearing part of the body but are most often observed on the forearms, hands, and legs of infected individuals. Involvement of the scalp and face is rarely observed. Lesions start as solitary or multiple well-circumscribed perifollicular papulopustules and nodules with or without background erythema and scaling. In rare circumstances, the lesions may have keloidal features.

Diagnosis

Primary diagnosis usually starts off with a thorough physical exam and evaluation of medical history. To further investigate, a dermoscope, a diagnostic tool, is used by the dermatologist to examine the skin using a magnified lens. A complete blood count (CBC) along with other blood tests can also be done to rule out any sort of other infections. Lastly, a skin biopsy test may be ordered to arrive at a definitive diagnosis. This pathological examination of the skin biopsy helps to arrive at the correct diagnosis via a fungal culture(mycology). The biopsy is put together with clinical and microscope findings and study of the special tissues if need be. The signs and symptoms of MG are similar to many other clinical conditions and therefore it is necessary to perform all of the additional tests in order for a physician to correctly rule out all other possible diagnoses.

Causes and Prevention

The exact cause of Majocchi's granuloma is not well established however a dysfunctinoal immune system may be a causative factor. The first form of MG, the superficial perifollicular form occurs predominately on the legs of otherwise healthy young women who repeatedly shave their legs and develop hair follicle occlusions that directly or indirectly disrupt the follicle and allow for passive introduction of the organism into the dermis. Hence, the physical barrier of the skin is important because it prevents the penetration of microorganisms. Physical factors that play a major role in inhibiting dermal invasion include the interaction among keratin production, the rate of epidermal turnover, the degree of hydration and lipid composition of the stratum corneum, CO₂ levels, and the presence or absence of hair. Keratin and/or necrotic material can also be introduced into the dermis with an infectious organism to further enhance the problem. In immunocompromised individuals, the use of topical corticosteroids may lead to a dermatophyte infection due to local immunosuppression.

Treatment and Prognosis

The treatment of Majocchi's disease can be difficult because the condition can slowly progress and is chronic in nature. After a period of time, the lesions can reoccur. Even though the condition has improved, there is always the possibility of reoccurance. There are treatments available to help improve the symptoms, however, there is no absolute cure for the disease. Some of these treatments include the application of topical steroids and lotions and ultraviolet therapy. The use of narrowband UVB and psoralen plus UVA have shown to be effective treatments for some patients with pigmented purpuric dermatoses. Majocchi granuloma also commonly occurs as a result of the use of potent topical steroids on unsuspected tinea. Historically, antifungal therapy has been successful in controlling MG in most instances. Therapies that have been used, included oral potassium iodide, mildly filtered local X-radiation, and topical applications of Asterol as a fungicide in both tincture and ointment forms. In modern medicine, systemic antifungals, such as griseofulvin, ketoconazole, and itraconazole, are the pillars of therapy, as they are safe and effective. The duration of therapy should be at least 4–8 weeks, and treatment should be continued until all lesions are cleared. Currently, no data about relapse rates or the complications of not treating Majocchi granuloma exist.

Recent Research

In the review article, Majocchi’s granuloma: a symptom complex caused by fungal pathogens authors Macit Ilkit, Murat Durdu and Mehmet Karakas focus 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. They conclude that, it should be noted that the Tzanck smear method is a rapid, easily performable diagnostic test. In addition, histopathologic examinations reveal granulomatous folliculitis in patients with MG. Lastly, systemic antifungals given at an adequate dose and for an appropriate duration are the drugs of choice; in general, topical antifungals alone do not clear the fungal infections.

In Majocchi's granumloma - Case report, the authors discuss the case of a three-year-old child who presented with lesions around her jaw. It was reported that she had been using a combination of topical corticoids, anti-fungals, and antibiotics during this period. The use of these products was only worsening her folliculitis. Drugs were then suspended after 15 days of use and was followed by cutaneous biopsy and histopathological examination. Mycological examination showed the presence of hyphae and spores compatible with Majocchi's granuloma. The patient was treated with griseofulvin for 8 weeks and went into remission from this condition.

In Tinea Corporis Gladiatorum Presenting as a Majocchi Granuloma, authors Anil Kurian and Richard M. Haber discuss the importance of differential diagnosis when it comes to diagnosing patients with Majocchi's granuloma. Their paper focuses on case report involving a 20-year-old male who had been a part of schools wrestling team for the past six years. H presented with a 4-year history of follicular papules and pustules on his right forearm. This lesion had the typical clinical appearance of a Majocchi granuloma. A skin biopsy from the right forearm showed an acute deep folliculitis compatible with a Majocchi granuloma, but fungal stainings with a Grocott stain was negative. This was the first case reported in medical literature where it was shown that tinea corporis gladiatorum can present as a Majocchi granuloma. Thus, dermatologists must consider a Majocchi granuloma in the differential diagnosis of persistent skin lesions in wrestlers.