0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Special Feature |

Picture of the Month—Diagnosis FREE

[+] Author Affiliations

Section Editor: Samir S. Shah, MD

More Author Information
Arch Pediatr Adolesc Med. 2007;161(9):908. doi:10.1001/archpedi.161.9.908.
Text Size: A A A
Published online

DENOUEMENT AND DISCUSSION: CONGENITAL CUTANEOUS CANDIDIASIS

Histological examination showed a subcorneal pustule filled with neutrophils and a superficial inflammatory infiltrate in the dermis. A direct potassium hydroxide test, as well as a periodic acid–Schiff stain on the biopsy specimen, showed spores and pseudohyphae. Growth of Candida albicans was observed in a Sabouraud culture. Findings of the Gram stain and bacteria culture were negative. Treatment with a topical antifungal (nystatin) was initiated, which caused the skin lesions to disappear in 7 days. After 3 months of follow-up, the patient remained asymptomatic without treatment and the ungual dystrophy resolved.

Mucocutaneous Candida infections are often observed in newborn infants because up to 20% to 25% of pregnant women have vulvovaginitis caused by this yeast.1 However, few published case reports can be found in the literature on congenital cutaneous candidiasis. It is not known whether such a scarcity of reports reflects underdiagnosis or underreporting of this condition.2

Congenital cutaneous candidiasis seems to result from chorioamnionitis acquired by an ascendant route through the intact fetal membrane from the maternal vagina.1,3,4 Skin lesions are present from birth or appear during the first few hours of life; they consist of a maculopapular eruption that evolves to include vesicles and pustules. Occurrence of palmoplantar vesicles and pustules, which evolve to desquamation, is often described. Occasionally, ungual dystrophy appears as a delayed manifestation of the disease,2,5 as in our patient. Most of the patients show only cutaneous involvement; however, some patients may develop systemic disease with nonspecific signs of sepsis, hepatomegaly, sepsis, or death.6 If systemic involvement is suspected, thorax irradiation, ophthalmologic examination, and blood, urine, and cerebral spinal fluid cultures are required. Blood culture results may be negative even when systemic involvement actually occurs. Hematogenous dissemination is not frequent but it can occur among premature neonates and infants with risk factors. Prematurity is generally accepted as a factor predisposing to disseminated disease.2,6,7 Furthermore, some authors have proposed that in infants with congenital candidiasis, disseminated infection or increased risk of disseminated infection should be suspected in the setting of respiratory distress or other laboratory or clinical signs of sepsis; birth weight lower than 1500 g; treatment with broad-spectrum antibiotics; extensive instrumentation-invasive procedures; positive systemic culture results; and evidence of an altered immune response.69

Differential diagnosis must be done as with other neonatal pustulovesicular conditions.3 The diagnosis is confirmed by identification of C albicans on a direct potassium hydroxide test or positive findings of a culture in Sabouraud medium from a cutaneous pustule. If a diagnosis is still unclear, a biopsy with a periodic acid–Schiff stain could be performed.

Treatment of candidiasis in the neonate requires an effective antifungal agent and, if possible, elimination of all risk factors for ongoing candidemia. In most term infants with congenital cutaneous candidiasis, the disease is limited to the skin, umbilical cord, and placenta.10 In these patients, topical antifungal agents are indicated (nystatin or azole formulations), although spontaneous resolution has also been described.2,6 Amphotericin B (0.5-1 mg/kg/d) is the recommended antifungal agent for the treatment of candidemia and any form of invasive candidiasis in the neonate,69,11,12 although lipid-associated amphotericin B preparations (3-5 mg/kg/d) are preferred in those cases with invasive candidiasis and severe preexisting renal insufficiency. Fluconazole (6-12 mg/kg/d) can be used as alternative therapy to amphotericin B, if the Candida species is identified and the susceptibility is shown.7,10,11 Flucytosine (50-100 mg/kg/d) is used in combination with amphotericin B for central nervous system infection.7,9,11 There are no controlled clinical trials to provide the optimal length of therapy. The topical antifungal agent must be used until the lesions resolve, and the systemic therapy must be used a minimum of 21 to 28 days.7,10,11

Correspondence: Buenaventura Hernandez-Machin, MD, Hospital Universitario Insular de Gran Canaria, Avd Marítima del Sur s/n, Las Palmas de Gran Canaria 35016, Spain (buenaventurahdezm@aedv.es).

Accepted for Publication: November 16, 2006.

Author Contributions:Study concept and design: Hernandez-Machin and Hernando. Acquisition of data: Hernandez-Machin and Hernando. Analysis and interpretation of data: Hernandez-Machin, Vargas, and Hernando. Drafting of the manuscript: Hernandez-Machin. Critical revision of the manuscript for important intellectual content: Hernandez-Machin, Reyes, and Hernando. Administrative, technical, and material support: Vargas and Hernando. Study supervision: Hernandez-Machin, Reyes, and Hernando.

Financial Disclosure: None reported.

Chapel  TAGagliardi  CNichols  W Congenital cutaneous candidiasis. J Am Acad Dermatol 1982;6 (5) 926- 928
PubMed Link to Article
Almeida Santos  LBeceiro  JHernandez  R  et al.  Congenital cutaneous candidiasis. Eur J Pediatr 1991;150 (5) 336- 338
PubMed Link to Article
Rudolph  NTariq  AAReale  MRGoldberg  PKKozinn  PJ Congenital cutaneous candidiasis. Arch Dermatol 1977;113 (8) 1101- 1103
PubMed Link to Article
Hayashi  SMochizuki  TWatanabe  S Infection of human fetal membranes in vitro with Candida albicans. Mycoses 1989;32 (3) 119- 122
PubMed Link to Article
Clegg  HWProse  NSGreenberg  DN Nail dystrophy in congenital cutaneous candidiasis. Pediatr Dermatol 2003;20 (4) 342- 344
PubMed Link to Article
Cosgrove  BFReeves  KMullins  DFord  MJRamos-Caro  FA Congenital cutaneous candidiasis associated with respiratory distress and elevation of liver function tests. J Am Acad Dermatol 1997;37 (5 pt 2) 817- 823
PubMed Link to Article
Adler  ALitmanovitz  IRegev  RArnon  SBauer  SDolfin  T Breakthrough candida infection in a preterm infant with Candida albicans infection. Am J Perinatol 2005;22 (3) 169- 171
PubMed Link to Article
Johnson  DEThompson  TRFerrieri  P Congenital candidiasis. Am J Dis Child 1981;135 (3) 273- 275
PubMed
Bendel  CMRemington  JSedKlein  JOedBaker  CedWilson  Ced Candidiasis. Infectious Diseases of the Fetus and Newborn Infant 6th ed. Philadelphia, Pa Elsevier Saunders2006;1107- 1128
Ramón Faba  PGarcía Dura  EMuñoz Romero  FCañabate González  CDelgado Florencio  VNaranjo Sintes  R Candidiasis cutánea congénita: dos casos y revisión de la literatura. Actas Dermosifiliogr 1992;83 (3) 101- 103
Pappas  PGRex  JHSobel  JD Guidelines for treatment of candidiasis. Clin Infect Dis 2004;38 (2) 161- 189
PubMed Link to Article
Waguespack-LaBiche  JChen  SHYen  A Disseminated congenital candidiasis in a premature infant. Arch Dermatol 1999;135 (5) 510- 512
PubMed Link to Article

Figures

Tables

References

Chapel  TAGagliardi  CNichols  W Congenital cutaneous candidiasis. J Am Acad Dermatol 1982;6 (5) 926- 928
PubMed Link to Article
Almeida Santos  LBeceiro  JHernandez  R  et al.  Congenital cutaneous candidiasis. Eur J Pediatr 1991;150 (5) 336- 338
PubMed Link to Article
Rudolph  NTariq  AAReale  MRGoldberg  PKKozinn  PJ Congenital cutaneous candidiasis. Arch Dermatol 1977;113 (8) 1101- 1103
PubMed Link to Article
Hayashi  SMochizuki  TWatanabe  S Infection of human fetal membranes in vitro with Candida albicans. Mycoses 1989;32 (3) 119- 122
PubMed Link to Article
Clegg  HWProse  NSGreenberg  DN Nail dystrophy in congenital cutaneous candidiasis. Pediatr Dermatol 2003;20 (4) 342- 344
PubMed Link to Article
Cosgrove  BFReeves  KMullins  DFord  MJRamos-Caro  FA Congenital cutaneous candidiasis associated with respiratory distress and elevation of liver function tests. J Am Acad Dermatol 1997;37 (5 pt 2) 817- 823
PubMed Link to Article
Adler  ALitmanovitz  IRegev  RArnon  SBauer  SDolfin  T Breakthrough candida infection in a preterm infant with Candida albicans infection. Am J Perinatol 2005;22 (3) 169- 171
PubMed Link to Article
Johnson  DEThompson  TRFerrieri  P Congenital candidiasis. Am J Dis Child 1981;135 (3) 273- 275
PubMed
Bendel  CMRemington  JSedKlein  JOedBaker  CedWilson  Ced Candidiasis. Infectious Diseases of the Fetus and Newborn Infant 6th ed. Philadelphia, Pa Elsevier Saunders2006;1107- 1128
Ramón Faba  PGarcía Dura  EMuñoz Romero  FCañabate González  CDelgado Florencio  VNaranjo Sintes  R Candidiasis cutánea congénita: dos casos y revisión de la literatura. Actas Dermosifiliogr 1992;83 (3) 101- 103
Pappas  PGRex  JHSobel  JD Guidelines for treatment of candidiasis. Clin Infect Dis 2004;38 (2) 161- 189
PubMed Link to Article
Waguespack-LaBiche  JChen  SHYen  A Disseminated congenital candidiasis in a premature infant. Arch Dermatol 1999;135 (5) 510- 512
PubMed Link to Article

Correspondence

CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

See Also...
Articles Related By Topic
Related Collections