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Chronic mucocutaneous candidiasis: a case report

Article  in  Journal of Indian Society of Pedodontics and Preventive Dentistry · April 2004

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Chronic Mucocutaneous Candidiasis: A Case Report.
BHOWATE Ra, DUBEY Ab
ABSTRACT
Chronic mucocutaneous candidiasis is a immuno deficiency disorder
primarily due to T cell dysfunction characterized by persistent
candidal infection of mucous membrane, skin, scalp and nails.
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. This paper describes a 12-
year-old girl with candidial infection of the oral mucosa and extra oral
involvement of fingers, nails, toes and intertragus area.
Keywords: Autoimmune, Moniliasis, Polyendocrinopathy,
Thrush.
INTRODUCTION
Chronic mucocutaneous candidiasis (CMC) is the term used
to describe a rare, heterogeneous group of syndromes in
which affected individuals with congenital, endocrinological
or immunological disorders develop recurrent or persistent
mucosal, cutaneous or nail infections with Candida albicans.
Chronic mucocutaneous candidiasis is associated with
endocrine conditions like hypoparathyroidism,
1
hypothyroidism, hypoadrenalism and diabetes mellitus .
Immune defects i.e. malfunctioning of lymphocytes, low levels
2
of immunoglobulin is also a contributory factor . Rarely chronic
mucocutaneous candidiasis develops in adult life. This is
3
often as a result of a thymoma (tumor of thymus gland) and
is associated with internal disease such as myasthenia gravis,
myositis, aplastic anaemia and neutropenia. Patient presents
with a history of recurrent or persistent superficial infections
of the oral cavity (thrush), intertriginous or perioro-facial
structures. Infants often present with candidial diaper
4
dermatitis . These infections can be followed by more
extensive scaling of skin lesions, as well as thickened nails
5
and red swollen periungal tissues . The scalp may be involved
with similar hyperkeratotic plaques, which can result in
6
scarring and alopecia .
Some patients with chronic mucocutaneous candidiasis have
an associated abnormality of iron metabolism demonstrated
7
by low serum iron and decreased iron stores . These
abnormalities may be caused by decreased iron absorption.
Provisional diagnosis of chronic mucocutaneous candidiasis
a.Principal, b.Assistant Professor, Dept. of Pedodontics & Pre-
ventive Dentistry, Govt. Dental College and Hospital, Raipur,
ISSN 0970 - 4388
can be made based on clinical findings. Microscopy and
culture of skin swabs and scrapings confirms the presence
of microorganisms. Serum values for various endocrinopathies
8
formulate an important key to diagnosis .
CASE REPORT
A 12-year-old female patient reported to the Department of
Pedodontics Govt. Dental College Raipur with the complaint
of broken maxillary anterior tooth. She wanted to replace the
same with an artificial tooth. Intra oral examination revealed
carious maxillary anteriors and premolars, bilaterally. Soft
tissues examination revealed white curd like lesions scattered
all over the buccal mucosa (Fig 1), upper and lower labial
mucosa (Fig 2), palatal mucosa (Fig 3) and dorsal surface
of tongue with loss of papillae and prominent central groove
(Fig 4). The hands and feet showed scaly lesions with
involvement of intertrigenus area and nails (Fig 5 and 6). Nails
were markedly thickened, fragmented and discolored with
significant edema. Erythema of the surrounding periungal
tissue simulating clubbing was also present. Scrapping from
the lesion revealed the presence of Candida albicans.
Laboratory investigations revealed Hb 8gm%, total leukocyte
count 3500 / cu mm, ESR 35mm at the end of one hour
(Westergen method). T3, T4, TSH and liver function tests
were within normal limits. Patient was medicated with
ketoconazole 150 mg systemically along with topical
application for 12 weeks. Carious broken maxillary teeth and
root pieces were extracted and replaced with a removable
space maintainer. Patient responded well to the therapy and
oral lesions disappeared completely after six weeks. Finger
nails and toe lesions started healing after 10 weeks of therapy.
After 12 weeks, patient was asked to continue with
ketoconazole once in a week for next six months. The patient
was followed upto two years without recurrence.
DISCUSSION
Chronic mucocutaneous candidiasis is a heterogeneous
disorder of immune system characterized by infection of
mucous membrane, scalp, skin and nails. Familial CMC may
1
be associated with autoimmune polyendocrinopathy . Most
patients with CMC have a selective defect of cell-mediated
 Chronic Mucocutaneous Candidiasis

Fig 1: Clinical photograph showing candidial lesions Fig 2: Clinical photograph showing candidial lesions
on atrophic buccal mucosa. on labial mucosa.

Fig 3: Clinical photograph showing candidial lesions Fig 4: Clinical photograph showing depapillation of
on palatal mucosa with carious 11 & 21. tongue with central groove & candidial lesions.

Figs 5 and 6: Clinical photograph showing markedly thickened nails, scaly lesions of hand and feet,
erythema of surrrounding periungal tissue, simulating clubbing.
 Chronic Mucocutaneous Candidiasis
antifungal agents for repeated or prolonged courses often in
higher doses than is normally necessary for Candida
infections. Terbinafine has been used effectively in
9 4.
Ketoconazole resistant chronic mucocutaneous candidiasis .
Use of transfer factor, Levamisole, thymosin and lymphocyte
infusion is still under experimental stages. Transfer factor
and cimetidine act as immuno modulatory agents. The ultimate
judgment regarding the propriety of any specific procedure
must be made by physician in light of all circumstances
presented by individual patient.
Clinical significance of chronic mucocutaneous candidiasis
lies in the fact that it may present as an inaugural symptom
1 3): 97-103.
of auto immune polyendocrinopathy and should lead to a
familial study with an endocrinology work-up to determine
any clinical manifestations and biological evidence of auto
immunity. Care should be taken to screen patients periodically
for the emergence of endocrinopathy. Parents should be
alerted regarding chronic recurrent nature of disease and
current paucity of effective long lasting therapies.
REFERENCES
1. Rybojad M., Abimelec P., Feuilhafe M., Morel P. and Bourrat
E.: Familial CMC associated with Autoimmune
Polyendocrinopathy. Treatment with Fluconazole 3 cases.
Ann Dermatol Venerol.1999 ; 126(1): 54 - 56.
2. Kalfa V.C., Robert R.L. and Stiehm E.R.: The syndrome of
CMC with selective antibody deficiency. Ann Allergy Asthama
Immunol 2003; 90(2): 259 - 264.
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3. Rothberg M.S., Eisenbud I. and Girboff S.: Chronic
mucocutaneous candidiasis thymoma syndrome. A case
report.Oral Srg Oral Med. Oral Pathol.1989; 68(4) : 411 - 3.
Caputo R.V.: Fungal infections in children. Dermatol
Clin.1986; 4(1): 137 - 349.
5. Jorizzo J.L.: Chronic mucocutaneous candidiasis. An update
Arch. Dermatol 1982; 118(12): 963 - 965.
6. Boni R., Trueb R. M. and Wuthrich B.: Alopecia areata in a
patient with candidiasis-endocrinopathy syndrome
unsuccessful treatment trial with diphenyl cyclo propenone.
Dermatology. 1995; 191(1): 68-71.
7. Masi M., Devinci C. and Baricord O. R.: Transfer factor in
chronic mucocutaneous candidiasis. Biotherapy, 1996; 9 (1-
8. Weiner M.H. and Coats-stephen M.: Immuno diagnosis of
systemic candidiasis mannan antigenemia detected by
Radio Immunoassay in experimental and human infections.
J Infect Dis 1979; 140 (6): 989 - 993.
9. Hassan C: Terbinafine effectiveness in ketoconozole
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Reprint Requests to :
Dr. Rahul Bhowate
Principal,
Govt. Dental College & Hospital,
Raipur, Chattisgarh.