|LETTER TO EDITOR
|Year : 2020 | Volume
| Issue : 4 | Page : 351-353
Chronic primary mucocutaneous candidiasis in a child
Aastha Gupta1, Sinu Rose Mathachan1, Pooja Arora1, Purnima Malhotra2
1 Department of Dermatology, Venereology and Leprosy, Dr. Ram Manohar Lohia Hospital and Postgraduate Institute of Medical Education and Research, New Delhi, India
2 Department of Pathology, Dr. Ram Manohar Lohia Hospital and Postgraduate Institute of Medical Education and Research, New Delhi, India
|Date of Submission||16-May-2019|
|Date of Decision||18-Jun-2019|
|Date of Acceptance||26-Apr-2020|
|Date of Web Publication||30-Sep-2020|
Dr. Sinu Rose Mathachan
Department of Dermatology, Venereology and Leprosy, Dr. Ram Manohar Lohia Hospital and Postgraduate Institute of Medical Education and Research, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gupta A, Mathachan SR, Arora P, Malhotra P. Chronic primary mucocutaneous candidiasis in a child. Indian J Paediatr Dermatol 2020;21:351-3
|How to cite this URL:|
Gupta A, Mathachan SR, Arora P, Malhotra P. Chronic primary mucocutaneous candidiasis in a child. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 Oct 19];21:351-3. Available from: https://www.ijpd.in/text.asp?2020/21/4/351/296861
A 3-year-old boy born out of a nonconsanguineous marriage presented with yellowish discoloration, subungual hyperkeratosis, and onychodystrophy affecting the nails of the right index finger, right middle finger, and left big toe for the past 1 year along with paronychia of the right index and middle finger [Figure 1]. He also had multiple, small, easily dislodgable, superficial white plaques covering the tongue, hard palate, and buccal mucosa [Figure 2]. The scraping of the plaque revealed underlying erythematous surface. The corners of the mouth showed erythema with minor fissures. The child was otherwise healthy and had had no history of atopy or recurrent infections in the past. Hemogram and routine biochemical parameters were within the normal limits. Basal serum cortisol, HIV 1 and 2 serology, and thyroid function tests were normal. Immunoglobulin (Ig) screen revealed the normal levels of IgA, IgM; however, the serum IgG levels and serum IgE levels were abnormally raised, values being 2471 mg/dl (700–1600) and 3238 IU/ml (<250), respectively. KOH examination from the oral mucosal scraping and nail periodic acid Schiff revealed the presence of slender pseudohyphae [Figure 3].
|Figure 1: Yellowish discoloration, subungual hyperkeratosis, and onychodystrophy affecting the nails of the right index finger, right middle finger, and left big toe|
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|Figure 2: Superficial white plaques covering the tongue, hard palate, and buccal mucosa|
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Chronic mucocutaneous candidiasis (CMC), characterized by persistent/recurrent candidal infection affecting the skin, mucosa, and nails, occurs as a result of defective cell-mediated immunity. Predominant sites of involvement include oral and esophageal mucosa, trunk, hands, and nails. They can be classified into several subtypes based on the mode of inheritance, gene, and locus affected and are tabulated in [Table 1].
Abnormalities in cytokine levels play a central role in CMC. There is the downregulation of Th1 and Th17 cytokines (interleukin [IL-2], IL-12, interferon-γ, IL-17, and IL-22) and upregulation of Th2 cytokines (IL-4 and IL-5). The underlying immune dysregulation could be primary or secondary. Primary CMC is characterized by an early onset and can be familial, sporadic, or can have associated autoimmune/endocrinological abnormalities. Around 20% of patients with CMC can have a positive family history with endocrinopathy as the most common association. While primary CMC represents an underlying inborn defect in the immune system, the secondary causes include HIV, diabetes mellitus, immune suppressive medications, and long-term antibiotic use and occurs later in life., In general, the humoral immune system in CMC remains unaffected with normal Ig levels.,, In contrast, our patient had abnormally high levels of serum IgG and IgE, whereas IgM and IgA were normal.
The management of CMC is difficult as the disease runs a chronic course, and relapse is common following the discontinuation of therapy. Historically, three treatment strategies have been mentioned for the treatment of CMC which include antifungal drugs, immunologic reconstitution (to correct the underlying immunodeficiency), and a combination of the two. Among immunological therapies thymus transplant, leukocyte transfer, Candida-specific transfer factor have been tried. The most widely studied treatment is the use of transfer factor which is a cell-free protein extracted from the T-lymphocytes of Candida-immune donors, thereby transferring the Candida-specific cell immunity to the patient. However, the current modality of the treatment is with systemic antifungal therapy, and fluconazole has been recommended as the first-line drug for CMC. Newer antifungals that can be given as fluconazole substitute include itraconazole, voriconazole, posaconazole, and ecchinocadins such as caspofungin, micafungin, and anidulafungin. Polyenes (topical and liposomal amphotericin B) are the third-line agents considered. Novel therapies such as Janus kinase 1/2 tyrosine kinase inhibitors (ruxolitinib) have shown improvement in patients of CMC with autoimmune diseases due to STAT 1 mutations. Systemic therapy also have drawbacks such as risk of toxicity, a failure to correct the underlying immune deficiency, relapse, and antifungal resistance. Our patient was prescribed 50 mg fluconazole weekly and topical clotrimazole cream and had excellent response with complete clearance of oral thrush and near total clearance of onychodystrophy after 1 month of the treatment [Figure 4].
|Figure 4: Near total clearance of onychodystrophy after 1 month of the treatment|
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Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]