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Detailed information of CHRONIC MUCOCUTANEOUS CANDIDIASIS
CHRONIC MUCOCUTANEOUS CANDIDIASIS
DEFINITION:
A group of immunodeficiency disorders primarily of T cell
function characterized by persistent Candida infection of the mucous
membranes, skin, scalp, and nails.
EPIDEMIOLOGY:
- incidence: ?
- age of onset:
- depends on type - first few months of life -> 20 years
- risk factors:
- familial - sporadic (Types 1 and 2)
- autosomal recessive (Types 3a, 4 and 5)
- autosomal dominant (Type 3b)
PATHOGENESIS:
- defect -> ? autoimmune disorder against lymphoid tissue and
endocrine organs -> failure of immune system to mount a T cell
attack and sometimes B cell attack against Candida antigen - the
endocrinopathies are not a result of candida infection of the
involved endocrine organs but more likely involve the formation
of autoantibodies
TYPES:
CLINICAL FEATURES:
- onset from first few months to 20 years
- most commonly present on oral mucous membranes
- frequently fingernails and toenails
- less commonly skin of face, hands, and feet
- very rarely systemic candidiasis
- may also involve:
- mouth/esophagus - dysphagia
- larynx - hoarseness, nasal speech
2. Type 1 - Early-Onset
- most severe and typical form
- oral with extensive skin and scalp involvement and one
or more nails of hands or feet involved
- candida granuloma
- large granulomatous or horny masses
- endocrinopathies
- common (in 50% of cases)
- usually hypothyroidism; rarely hypoparathyroid and
- Addison's Disease
3. Type 2 - Late-Onset
- mildest form
- limited to paronychia or buccal mucosa
- endocrinopathies rare
4. Type 3 - Familial
- a. mild-moderate candida; endocrinopathies rare
- b. moderate-severe candida; endocrinopathies variable
5. Type 4 - Candida-Endocrinopathy Syndrome
- mild-moderate candida
- endocrinopathies
- usually hypoparathyroidism
- may preced candidiasis
2. Endocrine Manifestations
- may occur at any time
- may be preceded by a viral infection
- Addison's Disease
- hypoparathyroidism
- hypothyroidism
- diabetes mellitus
- pernicious anemia
- eye disorders (cataracts, keratoconjunctivitis, band
kera-topathy) may be associated with hypoparathyroidism
3. Other Manifestations
- acute and chronic hepatitis and cirrhosis
INVESTIGATIONS:
- normal or slight decrease in T cell number
- diminished proliferative response to Candida antigen
- normal " phytohemagglutinin
- deficiency of migration inhibition factor (MIF)
- delayed hypersensitivity skin test to Candida is negative
and those to other antigens less vigorous
2. Humoral-Mediated
- usually normal or elevated immunoglobulins
- some may have an isolated IgA deficiency and/or deficient
IgG response to diphtheria immunization
- autoantibodies to various endocrine organs
- normal antibody response to Candida and other antigens
3. Endocrine
- serum values for the various endocrinopathies
4. Liver Function Tests
- for hepatitis and cirrhosis
2. Imaging Studies
- chronic pulmonary infiltrates and fibrosis
MANAGEMENT:
- nystatin (Mycostatin), clotrimazole (Canesten)
2. Oral
3. Intravenous
- miconazole
- watch transaminase levels
- amphotericin B
- chills/fever - premedicate with gravol, hydrocortisone,
meperidine
- nephrotoxic - tubular defects (hypokalemia, decreased
GFR)
2. Experimental
- transfer factor, levamisole, thymosin, lymphocyte infusions
- bone marrow transplant, fetal thymus transplant
3. Supportive
- Paediatrics - chronic disease, psychological support for
disfiguring skin manifestations
- Endocrine - management of various Endocrinopathies
- Dermatology - management of Candidiasis
- Prognosis - early-onset - death in 3rd decade
- late-onset - normal life span
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Pediatric Database - CHRONIC MUCOCUTANEOUS CANDIDIASIS
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