Chronic Mucocutaneous Candidiasis

presented by

Ian McColl MBChB, FACD

Gold Coast, Australia

on May 14, 2007

Consultant Dermatologist, John Flynn Medical Centre, Tugun, Queensland, Australia

 
Abstract A 5-year-old girl presented with chronic mucocutaneous candidiasis. A therapeutic suggestion was requested.
Patient
5-year-old girl
Duration
2 years
Distribution
Scalp, Lips and Nails
Clinical History

This little 5 year old came in today for treatment of her "severe psoriasis" which she has had for the last two years. Clinically this is chronic mucocutaneous candidiasis. There is no family history. She has had vaginal candidiasis before and UTIs. She is otherwise well.

Images

Laboratory Data

nil

Histopathology

nil

Diagnosis Chronic mucocutaneous candidiasis
Reasons Presented

For therapeutic suggestons

Questions

Has anyone had recent experience of treating a case? Ketoconazole orally ? toxicity? Fluconazole orally? How long for?

References  
Comments from Faculty and Members

Steven Chow FRCPI, Senior Consultant Dermatologist, Pantai Medical Centre, Kuala Lumpur, Malaysia on May 14, 2007

I have a similar patient who has been on long term itraconazole 100mg daily for the past 5 years. She has excellent tolerance of the treatment. The disease is under control and relapses when she defaults therapy. Topical treatment is of little value.

Rick Sontheimer MD, Professor of Dermatology, Department of Dermatology, University of Oklahoma Health Science Centre, Oklahoma City, OK, USA on May 14, 2007

I agree that the photographs and history are consistent with chronic mucocutaneous candidiasis. I have no recent experience in treating this disease. However, I think it would be important to subphenotype this patient if at all possible via referral to a pediatric immunodeficiency clinic. Some forms of this disease are associated with other medical problems (e.g. poly endocrinopathies). In addition, this patient and her family deserve appropriate genetic counseling.

Khalifa Sharquie MD, Professor of Dermatology, College of Medicine, University of Baghdad, Baghdad, Iraq on May 15, 2007

Although both conditions psoriasis and candidiasis can share some features but the picture is more in favour of psoriasis especially the scalp and the nail changes. So please do therapeutic antifungal trial and biopsy to confirm the diagnosis.The Presense of candida could be a secodary invader rather than as a primary cause.

Ijaz Hussain MD, Professor of Dermatology, Sheikh Zyad Medical College, RYK, Pakistan on May 17, 2007

I must congratulate Dr. Ian for such beautiful pictures. Regarding this case:

1. CMC itself is a group of multiple disorders. Primary defect as far I know is selective immunodeficiency against Candida spp. It may be associated with many other endocrinopathies, infections etc. Family history is important.
2. Since the nails are affected oral fluconazole and short duration of therapy would be insufficient as (a) Fluconazole is a less keratophilic drug, its binding with nail keratins, MIC in the nail matrix required for C. albinans would not be achieved. and (b) As in onychomycosis we require a longer duration of treatment e.g. 6 weeks at least, the duration of treatment would be longer in this particular case too.

Choon Siew Eng FRCP Senior Consultant Dermatologist, Hospital Sultanah Aminah, Johor Bahru, Malaysia on May 17, 2007

Years ago, I treated a 6-year-old boy with very similar lesions. I put him on ketoconazole for 6 months together with oral cimetidine. I had a 3-year-old girl recently in HSAJB and treated her with itraconazole. She responded well.

Laxmisha Chandrashekar MD, Lecturer in Dermatology, Christian Medical College, Vellore, India on May 18, 2007

She definitely needs HIV test and endocrine evaluation. She also needs iron studies. She may benefit from iron supplements, levamisole 75mg BD twice weekly, along with ketoconazole.

Anne Junker MD, Professor of Paediatrics (Immunology), University of British Columbia, Vancouver, BC, Canada on June 1, 2007

Poor kid! We saw a few kids in earlier days, before CMC went by the board as a "distinct" syndrome. Treatment is obviously antifungals, long term; often relapsing disease or development of antimicrobial resistance. We had one child who "grew out of" this -- lots of problems as an infant and toddler, which resolved by school age. There seems to be a view that CMC is associated with a variety of immune defects, rather than a distinct syndrome with unique abnormality in immune function. The largest association is CMC with autoimmune polyendocrinopathy type 1, due to mutations in the AIRE (autoimmune regulator) gene. These patients may have normal T cell function, but problems dealing with candida due to defective antigen presentation and downstream responses. There is also association with thymoma, but this is pretty rare in little kids.

Pramod Kumar MD, Consultant Dermatologist, Saham Hospital, Oman on June 15, 2007

I have a similar case. A automechanic presented with typical candidiasis and satellite lesions on hands,gradually increasing to dorsum of hands,wrists,elbows, knees and legs.Scalp and mucosae are still free.I treated him for Candidiasis with Fluconazole weekly.The lesions did not spread in number or size.But the hyperkeratosis would not go.I have reviewed my diagnosis to Psoriasis and put him on methotrexate weekly 15 mg and his lesions have already melted.All lab parameters were normal.In my view like guttate variety, Candidiasis can precipitate Psoriasis as is likely in this case.

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