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Chronic Mucocutaneous Candidiasis

CMC

Chronic mucocutaneous candidiasis is a heterogeneous disorder characterized by a chronic, noninvasive Candida infection of the skin, nails, and mucous membranes.


Presentation

Superficial persistent and recurrent infections caused by Candida species (most commonly Candida albicans) are the hallmark of chronic mucocutaneous candidiasis (CMC) [1]. The frequently affected areas of the body are the buccal mucosa, palate, tongue, and the oropharynx.

Most infections occur in early childhood [2]. However, adults may also be affected by CMC. Infants usually present with a diaper rash or a thrush refractory to the treatment, often progressing to more extensive disease.

Physical examination may yield definitive diagnostic clues to CMC. Oral lesions include white plaques that are adherent to the mucosa (thrush). Oropharyngeal plaques may extend to the esophagus. Angular cheilitis is present in some individuals.

Cracked and hyperkeratotic nails with prominent discoloration are commonly seen. The periungual regions are edematous and erythematous. Lesions of the skin are mostly seen on the extremities, with thickened, red plaques being a characteristic finding seen in many affected patients. Scalp lesions may be present in CMC and frequently progress to scarring alopecia.

Other infections, whether bacterial, viral, or fungal, may also be seen in a minority of individuals [3]. The invasive and systemic disease is frequently observed in infants with a low birth weight.

An important differential diagnosis is a candidal infection occurring in immunocompromised states.

Short Stature
  • Severe, early-onset, chronic mucocutaneous candidiasis was associated with bronchiectasis, oesophageal stricture, short stature and delayed puberty in a male aged 18 years.[ncbi.nlm.nih.gov]
Weight Gain
  • His son also suffered from persistent candidiasis, chronic diarrhea, poor weight gain, and pneumonia that resulted in his demise because of sepsis.[ncbi.nlm.nih.gov]
Recurrent Otitis Media
  • In this article, we describe a 33-year-old man who experienced chronic refractory candidiasis, recurrent otitis media, and pneumonia resulting in bronchiectasis, severe oral and esophageal candidiases with strictures associated with hypothyroidism and[ncbi.nlm.nih.gov]
Sputum
  • H. influenzae type D was constantly isolated from the patient's sputum.[ncbi.nlm.nih.gov]
Chronic Diarrhea
  • His son also suffered from persistent candidiasis, chronic diarrhea, poor weight gain, and pneumonia that resulted in his demise because of sepsis.[ncbi.nlm.nih.gov]
Aphthous Stomatitis
  • Other dermatoses reported in CMC include recurrent aphthous stomatitis, seborrheic dermatitis, alopecia areata, and vitiligo.[ijpd.in]
Cutaneous Manifestation
  • There are rare reports of other cutaneous manifestations. We sought to delineate the dermatological features of APECED in an Irish case series with emphasis on timing of their appearance and association with disease severity.[scienceopen.com]
  • Clinico epidemiological study of cutaneous manifestations in the neonate.. Indian J Dermatol Venereol Leprol 66 :, 26– – 28.[jmm.microbiologyresearch.org]
  • Cutaneous manifestations of HIV infection and HIV-related disorders. In: Bolognia J, Jorizzo J, Rapini R, editors. Dermatology. Second edition. New York: Elsevier; 2008. p. 1165-81. 32. Conant MA. Fungal infections in immunocompromised individuals.[revistasocolderma.org]
Skin Ulcer
  • The symptoms of this condition are hyperkeratosis, skin ulcer, dyspareunia, endocardium abnormality, vision problems, hepatitis, seizures, hematuria and meningitis In terms of the cause of chronic mucocutaneous candidiasis one finds it can be inherited[en.wikipedia.org]
  • ulcer Open skin sore 0200042 30%-79% of people have these symptoms Abnormal vagina morphology 0000142 Dyspareunia 0030016 Papule 0200034 5%-29% of people have these symptoms Abnormal endocardium morphology 0004306 Abnormality of dental enamel Abnormal[rarediseases.info.nih.gov]
Brittle Hair
  • Patients with idiopathic hypoparathyroidism frequently develop ectodermal disease, including dry, rough skin: coarse, brittle hair; and lusterless, distally split nails. All of these complaints are relatively common in a dermatologic practice.[ncbi.nlm.nih.gov]
Convulsions
  • In 1998, at the age of 32, he developed convulsions that were accompanied by impairment of consciousness, and which were temporarily treated with steroid pulsed-medication.[ncbi.nlm.nih.gov]
Onset in Infancy or Childhood
  • Chronic mucous membrane candidiasis has an onset in infancy or childhood; the primary affected site is the oral cavity; however, lesions may occur on trunk, hands, feet and scalp.[ncbi.nlm.nih.gov]

Workup

A patient presenting with clinical features suggestive of CMC should undergo thorough testing. Samples may be scraped from the infected lesions and microscopically examined in 10-20 % potassium hydroxide (KOH) to reveal the pseudohyphae and yeast cells [4]. Additional stains that may help in identification of the fungi are the Parker blue-black ink and chlorazol black E stain.

The infective candidal organisms grow well on Sabouraud agar with selective inhibiton by cycloheximide and chloramphenicol. The growth may also be attained on the dermatophyte test medium.

Skin biopsies are usually not required to make a diagnosis of CMC. They may, however, be useful in ruling out the presence of a primary dermatosis. Subcorneal pustules along with parakeratosis and hyperkeratosis are visualized on a hematoxylin and eosin stained samples. The silver stain and the periodic acid-Schiff stain may also be beneficial in identifying these lesions.

In individuals suspected of having CMC, it is imperative to perform tests to check for primary or secondary immunodeficiencies and endocrine abnormalities. A routine testing protocol should include a complete blood count, blood glucose level, human immunodeficiency virus (HIV) testing, liver function tests, serum electrolytes, plasma adrenocorticotropic hormone (ACTH), and serum cortisol level. Patients suffering from isolated immunoglobulin G, immunoglobulin A, or immunoglobulin M subclass deficiencies have also been reported [5].

Genetic testing for the AIRE gene and anti-interferon-1 antibodies are some of the novel additions to the laboratory evaluation of CMC [6] [7] [8].

Anergy
  • He had defects in cell-mediated immunity (cutaneous anergy to common antigens, but normal number of T lymphocytes and normal lymphocyte transformation with phytohemagglutinin), and there were no endocrine deficiencies.[ncbi.nlm.nih.gov]
  • Cutaneous anergy to candida antigens has been associated with defective lymphocyte function in vitro. Temporary modification of cellular immune function in one case was associated with partial clearing of skin lesions.[annals.org]
  • […] recognition receptor essential for the control of fungal infections) CARD9 (caspase recruitment domain-containing protein 9, which is an adaptor molecule important in the production of IL-17 and for protection against fungal invasion) Patients have cutaneous anergy[merckmanuals.com]
Liver Biopsy
  • Liver biopsy revealed CD68-positive cell infiltration and IL-18 expression in the sinusoidal regions.[ncbi.nlm.nih.gov]

Treatment

  • Ketoconazole is a promising new agent for treatment of chronic mucocutaneous candidiasis.[ncbi.nlm.nih.gov]

Prognosis

  • Little is known about the incidence and prognosis of CMC. Clinically, the main complications are debilitating hands (Candida granuloma) and oesophageal stricture with potential mal-digestion/-absorption.[ncbi.nlm.nih.gov]
  • In isolated CMC, the prognosis is good; however, the significant morbidity is related to chronic and persistent skin, nail, and mucous membrane candidal infections.[emedicine.com]

Etiology

  • We report two genetic etiologies of CMCD: autosomal recessive deficiency in the cytokine receptor, interleukin-17 receptor A (IL-17RA), and autosomal dominant deficiency of the cytokine interleukin-17F (IL-17F).[ncbi.nlm.nih.gov]
  • We report two genetic etiologies of CMCD: autosomal recessive deficiency in the cytokine receptor, interleukin-17 receptor A (IL-17RA), and autosomal dominant deficiency of the cytokine IL-17F.[jimmunol.org]
  • Abstract Candidiasis (moniliasis, candidosis) includes a group of fungous diseases varying in severity from commonly encountered local superficial involvement of the oral mucosa to fulminating systemic infections rapidly terminating in death. 1 The etiologic[pediatrics.aappublications.org]

Epidemiology

  • Molecular epidemiology studies of CMC infection are limited in number and scope and it is not clear whether single or multiple strains inducing CMC persist stably or are exchanged and replaced.[ncbi.nlm.nih.gov]
  • Two previous studies investigating the molecular epidemiology of CMC patients demonstrate the emergence of azole resistant C. albicans DSTs causative of infection.[journals.plos.org]
  • Gene sequencing identified mutations in signal transducer and activator of transcription 1 ( STAT1 ), leading to defective immune responses in type 1 and type 17 helper T cells. [3, 4, 5] Epidemiology Race No racial predilection is reported for chronic[emedicine.com]
  • Clinico epidemiological study of cutaneous manifestations in the neonate.. Indian J Dermatol Venereol Leprol 66 :, 26– – 28.[jmm.microbiologyresearch.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology C albicans is an opportunistic yeast that is part of the normal flora of the gastrointestinal tract, skin, and mucous membranes. The fungus can exist in the yeast, the mycelial (pseudohyphal), or the chlamydospore phase.[emedicine.com]

Prevention

  • We conclude that continuous antifungal prophylaxis associated to drug monitoring might prevent resistance to treatment; prompt diagnosis and therapy of lung disease might control long-term progression; careful monitoring of lymphopenia-related infections[ncbi.nlm.nih.gov]
  • T-cells, particularly intact T-helper 17 cell, neutrophils and the type-1 interferon cytokine networks are non-redundant for preventing invasive fungal disease.[journals.co.za]
  • It is normally found in humans and actually does no harm unless the immune system fails to prevent its excessive multiplication. So in order to keep Candida at bay, one must have healthy and strong immune system.[ic.steadyhealth.com]

References

Article

  1. Julián-González RE, Valdebrán-Canales MA, Guidos-Morales HE. Candidiasis mucocutánea crónica. Informe de un caso. Arch Argent Pediatr. 2010;108:e37–e40.
  2. Bolognia JL, Jorizzo JL, Rapini RP, et al. Dermatologia. 2. ed. Rio de Janeiro: Elsevier; 2011. pp. 804–806.
  3. Herrod HG. Chronic mucocutaneous candidiasis in childhood and complications of non­Candida infection: a report of the Pediatric Immunodeficiency Collaborative Study Group. J Pediatr. 1990;116(3):377­82.
  4. Instituto de Ciências Biológicas. Universidade Federal de Minas Gerais . Belo Horizonte: Agentes Etiológicos das Leveduroses. 2006.
  5. Patiroglu T, Tahan F. Chronic mucocutaneous candidiasis with agammaglobulinaemia. J Eur Acad Dermatol Venereol. 2007;21(6):833­4.
  6. Brännström J, Hässler S, Peltonen L, Herrmann B, Winqvist O. Defect internalization and tyrosine kinase activation in Aire deficient antigen presenting cells exposed to Candida albicans antigens. Clin Immunol. 2006;121(3):265­73.
  7. Collins SM, Dominguez M, Ilmarinen T, Costigan C, Irvine AD. Dermatological manifestations of autoimmune polyendocrinopathy­candidiasis­ectodermal dystrophy syndrome. Br J Dermatol. 2006;154(6):1088­93.
  8. Meager A, Visvalingam K, Peterson P, et al. Anti­interferon autoantibodies in autoimmune polyendocrinopathy syndrome type 1. PLoS Med. 2006;3(7):e289.

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Last updated: 2019-07-11 20:03