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Year : 2019  |  Volume : 20  |  Issue : 2  |  Page : 180-182

Disseminated congenital cutaneous candidiasis caused by Candida ciferri: A rare case report

1 Department of Paediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India
2 Department of Microbiology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India
3 Department of Paediatrics, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India

Date of Web Publication29-Mar-2019

Correspondence Address:
Dr. B Puneetha
Department of Paediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_139_18

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Congenital cutaneous candidiasis (CCC) is an uncommon disease affecting newborns which manifests within first 6 days of life following an ascending infection from birth canal or as transplacental infection. Diagnosis is challenging due to its varied clinical manifestation ranging from mild asymptomatic illness to fatal disseminated disease. We describe a full-term newborn child with CCC with dissemination born to mother with vaginal candidiasis, positive for Candida ciferri by fungal culture. This highlights the importance of maternal history of vaginal discharge during pregnancy to diagnose and prevent neonatal disseminated infection which further aids for adequate early treatment to prevent subsequent mortality.

Keywords: Candida ciferri, candidiasis, congenital cutaneous, disseminated

How to cite this article:
Puneetha B, Srinivas SM, Mahantesh S, Benakappa N. Disseminated congenital cutaneous candidiasis caused by Candida ciferri: A rare case report. Indian J Paediatr Dermatol 2019;20:180-2

How to cite this URL:
Puneetha B, Srinivas SM, Mahantesh S, Benakappa N. Disseminated congenital cutaneous candidiasis caused by Candida ciferri: A rare case report. Indian J Paediatr Dermatol [serial online] 2019 [cited 2020 Sep 20];20:180-2. Available from: http://www.ijpd.in/text.asp?2019/20/2/180/255194

  Introduction Top

Congenital cutaneous candidiasis (CCC) is a rare neonatal infection presenting on the first day or within 6 days of life which represents established candidiasis following intrauterine infection.[1] Skin lesions with varied morphologies such as maculopapular, pustules, abscess, generalized erythema, burn like dermatitis, dry cracking, and/or desquamation can be present.[2],[3] Palms, soles, and nails with sparing of mucosae and napkin area are often characteristic of CCC.[1] In full-term neonates, the disease course is usually benign and self-limiting whereas in preterm or low birth weight infants (<1000 g) dissemination of infection to blood, urine, or cerebrospinal fluid (CSF) occurs and can be fatal (mortality up to 40%) in the absence of prompt systemic treatment.[2] We report a case of nonalbican Candida species causing disseminated Candida infection in a term newborn which has not been described earlier.

  Case Report Top

We describe a full term 2-day-old male child born to 23-year-old mother who presented with skin lesions on the 2nd day of life. Mother had prolonged labor with birth asphyxia in the baby, and she also gave a history of vaginal discharge since 7th month of pregnancy. On examination, the child was lethargic with feeble cry, multiple tiny vesicles with erythema over face, trunk, and extremities [Figure 1] and [Figure 2]. Lesions became pustular with crusting on the 4th day and were associated with penile swelling and discharge from eyes [Figure 3]. Investigations revealed normal hemogram, Tzanck smear was negative for multinucleated giant cells, serology for IgM antibody for herpes simplex virus-2 and varicella-zoster was negative. KOH mount from lesions showed budding yeast cells [Figure 4]. The fungal culture revealed Candida ciferri which was identified by automated VITEK 2 system with 88% probability, sensitive to Amphotericin B with MIC 0.5. Urine examination also showed budding yeast cells. The diagnosis of disseminated CCC was made, and the child was treated with intravenous amphotericin B and topical miconazole for 14 days. Lesions resolved by 20 days leaving behind postinflammatory hyperpigmentation [Figure 5].
Figure 1: Multiple tiny vesicles and erythematous papules over face and chest

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Figure 2: Multiple vesicles, pustules with crusting over face, both upper limbs and chest

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Figure 3: Multiple pustules, erythematous papules, and few vesicles over face

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Figure 4: Potassium hydroxide mount showing pseudohyphae and yeast form of candida ciferri

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Figure 5: Post inflammatory hyperpigmentation over face, trunk, upper and lower limbs following disseminated congenital cutaneous candida infection on the 20th day of treatment

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  Discussion Top

CCC is an extremely rare perinatally acquired candidal infection which was first reported by Sonnenschein et al., in 1960 and only <100 cases have been reported in literature so far.[3],[4] Clinically, it is known to produce a spectrum of disease ranging from diffuse cutaneous eruptions to severe systemic illness in neonates or even intrauterine death. The risk factors predisposing for CCC are prematurity, maternal vaginal candidiasis, preterm labor, prolonged rupture of membranes, administration of broad-spectrum antibiotics, foreign body such as intrauterine contraceptive device or cervical suture in the uterus and chorioamnionitis following amniocentesis.[2],[4]

In contrast to acquired neonatal candidiasis which presents after 1 week of life and involve predominantly flexural areas and oral cavity, CCC presents at birth or within 72 h as generalized eruption of varied morphology which has to be differentiated from other conditions such as staphylococcal infection, herpes simplex, syphilis, erythema toxicum neonatorum, transient neonatal pustular melanosis, drug eruptions, and Langerhans cell histiocytosis.[4] Hence, basic bedside laboratory investigations such as Tzanck smear, KOH mount, and gram staining are utmost helpful in accurate diagnosis especially in case of unusual presentations with overlapping clinical morphology.

Neonates with a history suggestive of risk factors and evidence of systemic involvement screening for the dissemination of infection by blood, CSF, and urine culture is important as it can be fatal. Although the course is benign and self-limiting which rarely requires systemic treatment in term or low-risk infants, prompt systemic antifungal therapy at the time of skin lesions prevents fungal dissemination and mortality.[2]

Systemic antifungal is usually recommended in the neonates with positive cultures or burn like dermatitis, amphotericin B (0.5–1 mg/kg/day) is usually used as first-line agent and Fluconazole (6–12 mg/kg/day) as an alternative therapy given for ≥14 days' duration.[3],[4],[5]

There are more than 200 Candida species, but only few species are pathogenic, Candida albicans is the most common pathogenic species. Candida species colonize the vagina of pregnant women (30%) due to increased susceptibility occurring due to immunologic alterations, increased estrogen levels, and increased vaginal glycogen production leading to either asymptomatic infection or symptomatic vulvovaginal candidiasis especially in second and third trimesters, but only <1% of them develop Candida chorioamnionitis causing ascending infection leading formation of white microabscesses on the placenta and umbilical cord of an infant which was classically described as “white dots on placenta and red dots on baby.”[1],[6],[7]C. ciferrii is an unusual, nonalbicans Candida species first described by Kreger-van Rij in 1965.[8],[9] Although this species initially thought to be a saprophyte causing superficial infections alone, it is now considered as emerging Candida pathogen as it has been reported in literature to cause invasive azole-resistant fungal infections, especially in immunocompromised individuals.[8],[10] The cause of the current trend of switching into unusual pathogens is unknown, but it is thought to be prolonged prophylactic use of azole antifungal has led to the emergence of resistant unusual species.[10]

Our case highlights the importance of identification of etiological agent and prompt screening of neonate with suspected CCC for dissemination to reduce the mortality associated with disseminated CCC. Maternal screening for candidiasis is essential to prevent perinatal transmission of infection resulting in subsequent morbidity and mortality in neonate. Although CCC in term neonates is self-limiting, one should be aware of varied clinical features and associated systemic involvement occurring due to rare pathogenic species.

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.

  References Top

Aruna C, Seetharam K. Congenital candidiasis. Indian Dermatol Online J 2014;5:S44-7.  Back to cited text no. 1
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Kaufman DA, Coggins SA, Zanelli SA, Weitkamp JH. Congenital cutaneous candidiasis: Prompt systemic treatment is associated with improved outcomes in neonates. Clin Infect Dis 2017;64:1387-95.  Back to cited text no. 2
Jagtap SA, Saple PP, Dhaliat SB. Congenital cutaneous candidiasis: A rare and unpredictable disease. Indian J Dermatol 2011;56:92-3.  Back to cited text no. 3
[PUBMED]  [Full text]  
Srinivas SM, Bhardwaj P. Congenital cutaneous candidiasis from an asymptomatic mother. Indian J Paediatr Dermatol 2014;15:49-51.  Back to cited text no. 4
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Darmstadt GL, Dinulos JG, Miller Z. Congenital cutaneous candidiasis: Clinical presentation, pathogenesis, and management guidelines. Pediatrics 2000;105:438-44.  Back to cited text no. 5
Aguin TJ, Sobel JD. Vulvovaginal candidiasis in pregnancy. Curr Infect Dis Rep 2015;17:462.  Back to cited text no. 6
Diana A, Epiney M, Ecoffey M, Pfister RE. White dots on the placenta and red dots on the baby: Congential cutaneous candidiasis – A rare disease of the neonate. Acta Paediatr 2004;93:996-9.  Back to cited text no. 7
Gunsilius E, Lass-Flörl C, Kähler CM, Gastl G, Petzer AL. Candida ciferri, a new fluconazole-resistant yeast causing systemic mycosis in immunocompromised patients. Ann Hematol 2001;80:178-9.  Back to cited text no. 8
Kreger-Van Rij NJ. Candida ciferri, a new yeast species. Mycopathol Mycol Appl 1965;26:49-52.  Back to cited text no. 9
Cheng MF, Yu KW, Tang RB, Fan YH, Yang YL, Hsieh KS, et al. Distribution and antifungal susceptibility of Candida species causing candidemia from 1996 to 1999. Diagn Microbiol Infect Dis 2004;48:33-7.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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