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Majocchi's granuloma is a skin condition characterized by deep, pustular plaques, and is a form of
tinea corporis Tinea corporis is a fungal infection of the body, similar to other forms of tinea. Specifically, it is a type of dermatophytosis (or ringworm) that appears on the arms and legs, especially on glabrous skin; however, it may occur on any superficial ...
. It is a localized form of fungal folliculitis. Lesions often have a pink and scaly central component with
pustules A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this ...
or folliculocentric papules at the periphery. The name comes from Domenico Majocchi, who discovered the disorder in 1883. Majocchi was a professor of
dermatology Dermatology is the branch of medicine dealing with the skin.''Random House Webster's Unabridged Dictionary.'' Random House, Inc. 2001. Page 537. . It is a speciality with both medical and surgical aspects. A dermatologist is a specialist medica ...
at the
University of Parma The University of Parma ( it, Università degli Studi di Parma, UNIPR) is a public university in Parma, Emilia-Romagna, Italy. It is organised in nine departments. As of 2016 the University of Parma has about 26,000 students. History During the ...
and later the
University of Bologna The University of Bologna ( it, Alma Mater Studiorum – Università di Bologna, UNIBO) is a public research university in Bologna, Italy. Founded in 1088 by an organised guild of students (''studiorum''), it is the oldest university in continu ...
. The most common
dermatophyte Dermatophyte (from Greek '' derma'' "skin" ( GEN ''dermatos'') and ''phyton'' "plant") is a common label for a group of fungus of ''Arthrodermataceae'' that commonly causes skin disease in animals and humans. Traditionally, these anamorphic (a ...
is called ''
Trichophyton rubrum ''Trichophyton rubrum'' is a dermatophytic fungus in the phylum Ascomycota. It is an exclusively clonal, anthropophilic saprotroph that colonizes the upper layers of dead skin, and is the most common cause of athlete's foot, fungal infectio ...
.''


Symptoms and signs

Majocchi's granuloma often presents as pink scaly patches with pustules at the periphery. It is most common on skin exposed to mechanical abuse—wear and tear—such as the upper and lower extremities. Patients experience papules, pustules, or even plaques and nodules at the infection site. The white to red papules and pustules often have a perifollicular location. Hair shafts can be easily removed from the pustules and papules. Itching is 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 Erythema (from the Greek , meaning red) is redness of the skin or mucous membranes, caused by hyperemia (increased blood flow) in superficial capillaries. It occurs with any skin injury, infection, or inflammation. Examples of erythema not asso ...
and scaling. In rare circumstances, the lesions may have keloidal features.


Causes

Majocchi's granuloma is caused by a common group of fungi called dermatophytes. Unlike traditional ''
tinea corporis Tinea corporis is a fungal infection of the body, similar to other forms of tinea. Specifically, it is a type of dermatophytosis (or ringworm) that appears on the arms and legs, especially on glabrous skin; however, it may occur on any superficial ...
'' (commonly known as ringworm) that resides in the top layer of the skin, Majocchi's granuloma contains
dermatophyte Dermatophyte (from Greek '' derma'' "skin" ( GEN ''dermatos'') and ''phyton'' "plant") is a common label for a group of fungus of ''Arthrodermataceae'' that commonly causes skin disease in animals and humans. Traditionally, these anamorphic (a ...
s that invade the hair follicle and/or dermis. The invasion of the hair follicule leads to the clinically evident papules and pustules at the periphery. The most common form, 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 Keratin () is one of a family of structural fibrous proteins also known as ''scleroproteins''. Alpha-keratin (α-keratin) is a type of keratin found in vertebrates. It is the key structural material making up scales, hair, nails, feathers, ho ...
production, the rate of
epidermal The epidermis is the outermost of the three layers that comprise the skin, the inner layers being the dermis and hypodermis. The epidermis layer provides a barrier to infection from environmental pathogens and regulates the amount of water relea ...
turnover, the degree of hydration and
lipid Lipids are a broad group of naturally-occurring molecules which includes fats, waxes, sterols, fat-soluble vitamins (such as vitamins A, D, E and K), monoglycerides, diglycerides, phospholipids, and others. The functions of lipids includ ...
composition of the ''stratum corneum'', CO2 levels, and the presence or absence of hair. Keratin and/or necrotic material can be introduced into the dermis with an infectious organism to exacerbate the problem. Majocchi granuloma also can occur as a result of the use of potent
topical steroid Topical steroids are the topical forms of corticosteroids. Topical steroids are the most commonly prescribed topical medications for the treatment of rash, eczema, and dermatitis. Topical steroids have anti-inflammatory properties and are classifie ...
s on unsuspected
tinea Dermatophytosis, also known as ringworm, is a fungal infection of the skin. Typically it results in a red, itchy, scaly, circular rash. Hair loss may occur in the area affected. Symptoms begin four to fourteen days after exposure. Multiple ar ...
.


Mechanisms

Historically, many types of dermatophytes were known to cause the condition. '' Trichophyton violaceum'' used to be one of the most common species of dermatophytes to cause this disease. Today, however, ''Trichophyton rubrum'' is the main culprit in most cases. These fungi ar
keratinophilic
and colonize or infect the superficial
keratin Keratin () is one of a family of structural fibrous proteins also known as ''scleroproteins''. Alpha-keratin (α-keratin) is a type of keratin found in vertebrates. It is the key structural material making up scales, hair, nails, feathers, ho ...
ized 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 an
immunocompetent In immunology, immunocompetence is the ability of the body to produce a normal immune response following exposure to an antigen. Immunocompetence is the opposite of immunodeficiency (also known as ''immuno-incompetence'' or being ''immuno-compro ...
host and therefore the infection incidence is higher in immune compromised individuals. The two forms of MG are: * small
perifollicular
papular A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this ...
form, which is a localized dermal infection that usually occurs in healthy individuals * deep subcutaneous plaque/nodular lesion form that occurs in immunosuppressed hosts. ''
Tinea corporis Tinea corporis is a fungal infection of the body, similar to other forms of tinea. Specifically, it is a type of dermatophytosis (or ringworm) that appears on the arms and legs, especially on glabrous skin; however, it may occur on any superficial ...
'' is the name of the subset of this disease that 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.


Diagnosis

Primary diagnosis starts with a thorough physical exam and evaluation of medical history. Often, the condition is readily apparent to a medical practitioner and no further testing is required. If not readily apparent, a
skin biopsy Skin biopsy is a biopsy technique in which a skin lesion is removed to be sent to a pathologist to render a microscopic diagnosis. It is usually done under local anesthetic in a physician's office, and results are often available in 4 to 10 days. ...
test or fungal culture may be ordered. This
pathological Pathology is the study of the causes and effects of disease or injury. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in th ...
examination of the skin biopsy helps to arrive at the correct diagnosis via a fungal culture (
mycology Mycology is the branch of biology concerned with the study of fungi, including their genetic and biochemical properties, their taxonomy and their use to humans, including as a source for tinder, traditional medicine, food, and entheogen ...
). In severe or recurrent cases, further workup may be required. This disease commonly affects both
immunocompetent In immunology, immunocompetence is the ability of the body to produce a normal immune response following exposure to an antigen. Immunocompetence is the opposite of immunodeficiency (also known as ''immuno-incompetence'' or being ''immuno-compro ...
and
immunocompromised Immunodeficiency, also known as immunocompromisation, is a state in which the immune system's ability to fight infectious diseases and cancer is compromised or entirely absent. Most cases are acquired ("secondary") due to extrinsic factors that a ...
hosts. However, immunocompromised individuals have a higher risk.


Treatment

Oral
antifungal medications An antifungal medication, also known as an antimycotic medication, is a pharmaceutical fungicide or fungistatic used to treat and prevent mycosis such as athlete's foot, ringworm, candidiasis (thrush), serious systemic infections such as cryp ...
are the standard of care. Due to the location of the dermatophytes within the hair follicle, treatment with topical antifungals is often unsatisfactory. In patients with ''
tinea pedis Dermatophytosis, also known as ringworm, is a fungal infection of the skin. Typically it results in a red, itchy, scaly, circular rash. Hair loss may occur in the area affected. Symptoms begin four to fourteen days after exposure. Multiple a ...
'' or
onychomycosis Onychomycosis, also known as tinea unguium, is a fungal infection of the nail. Symptoms may include white or yellow nail discoloration, thickening of the nail, and separation of the nail from the nail bed. Toenails or fingernails may be affected, ...
, re-inoculation and recurrence is common. In individuals with recurrent outbreaks, inoculation sources should be identified and treated appropriately. Historical therapies include oral
potassium iodide Potassium iodide is a chemical compound, medication, and dietary supplement. It is a medication used for treating hyperthyroidism, in radiation emergencies, and for protecting the thyroid gland when certain types of radiopharmaceuticals are u ...
, mildly filtered local
X-radiation An X-ray, or, much less commonly, X-radiation, is a penetrating form of high-energy electromagnetic radiation. Most X-rays have a wavelength ranging from 10 picometers to 10 nanometers, corresponding to frequencies in the range 30  ...
, and topical applications of Asterol as a fungicide in both
tincture A tincture is typically an extract of plant or animal material dissolved in ethanol (ethyl alcohol). Solvent concentrations of 25–60% are common, but may run as high as 90%.Groot Handboek Geneeskrachtige Planten by Geert Verhelst In chemistr ...
and ointment forms. In modern medicine, systemic antifungals, such as
griseofulvin Griseofulvin is an antifungal medication used to treat a number of types of dermatophytoses (ringworm). This includes fungal infections of the nails and scalp, as well as the skin when antifungal creams have not worked. It is taken by mouth. C ...
, ketoconazole, and
itraconazole Itraconazole, sometimes abbreviated ITZ, is an antifungal medication used to treat a number of fungal infections. This includes aspergillosis, blastomycosis, coccidioidomycosis, histoplasmosis, and paracoccidioidomycosis. It may be given by mo ...
, are the standard. Therapy extends over at least 4–8 weeks, and treatment continues until all lesions are cleared. Currently, no data about relapse rates or the complications of not treating Majocchi granuloma exist.


Research

The review article, "Majocchi’s granuloma: a symptom complex caused by fungal pathogens" concludes that the Tzanck smear method is a rapid and easy diagnostic test. In addition, histopathologic examinations reveal granulomatous folliculitis in patients with MG. It found that systemic antifungals given at an adequate dose and for an appropriate duration are the drugs of choice; in general,
topical A topical medication is a medication that is applied to a particular place on or in the body. Most often topical medication means application to body surfaces such as the skin or mucous membranes to treat ailments via a large range of classes ...
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 ineffective. Drugs were suspended after 15 days of use and followed by cutaneous biopsy and
histopathological Histopathology (compound of three Greek words: ''histos'' "tissue", πάθος ''pathos'' "suffering", and -λογία ''-logia'' "study of") refers to the microscopic examination of tissue in order to study the manifestations of disease. Spec ...
examination. Mycological examination showed the presence of hyphae and spores compatible with MG. The patient was treated with
griseofulvin Griseofulvin is an antifungal medication used to treat a number of types of dermatophytoses (ringworm). This includes fungal infections of the nails and scalp, as well as the skin when antifungal creams have not worked. It is taken by mouth. C ...
for 8 weeks and went into remission. The article "Tinea Corporis Gladiatorum Presenting as a Majocchi Granuloma" discussed the importance of differential diagnosis. It includes a case report involving a 20-year-old male H 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. A skin biopsy showed an acute deep folliculitis compatible with a Majocchi granuloma, but fungal stainings with a Grocott stain was negative. This was the first reported case that showed 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.


See also

* Domenico Majocchi * List of cutaneous conditions


References


External links

{{Medical resources , DiseasesDB = 33948 , ICD10 = , ICD9 = , ICDO = , OMIM = , MedlinePlus = , eMedicineSubj = article , eMedicineTopic = 1092601 , MeshID = Mycosis-related cutaneous conditions