- Email: [email protected]
Perineal ulcers in an infant: An unusual presentation of postnatal cytomegalovirus infection
536 Case reports
J AM ACAD DERMATOL MARCH 2006
18. Jakobiec FA, Austin P, Iwamoto T, Trokel SL, Marquardt MD, Harrison W. Primary infiltrating signet ring carcinoma of the eyelids. Ophthalmology 1983;90:291-9. 19. Thomas JW, Fu YS, Levine MR. Primary mucinous sweat gland carcinoma of the eyelid simulating metastatic carcinoma. Am J Ophthalmol 1979;87:29-33.
20. Kuno Y, Numata T, Kanzaki T. Adenocarcinoma with signet ring cells of the axilla showing apocrine features: a case report. Am J Dermatopathol 1999;21:37-41. 21. Kuno Y, Tsuji T, Yamamoto K. Adenocarcinoma with signet ring cells of the axilla: two case reports and review of the literature. J Dermatol 1999;26:390-5.
Perineal ulcers in an infant: An unusual presentation of postnatal cytomegalovirus infection John G. Hancox, MD,a Avinash K. Shetty, MD,b Omar P. Sangueza, MD,c and Gil Yosipovitch, MDa,d Wake Forest, NC
Cytomegalovirus (CMV) disease can cause significant morbidity and mortality in neonates and immunosuppressed patients. Cutaneous disease is rare, even in at-risk patients. We report a case of CMV with perineal papules, erosions, and ulcers in a preterm but presumably immunocompetent patient, whose diagnosis was first suggested by skin biopsy and confirmed with serologic testing. The mode of transmission of CMV was unclear. The lesions resolved without CMV-specific therapy, and the child had no apparent systemic sequelae of infection. CMV disease should be considered in the differential diagnosis of perineal erosions and ulcers in infancy. In addition to the case, we briefly review the literature on cutaneous manifestations of CMV disease. ( J Am Acad Dermatol 2006;54:536-9.)
C
ongenital cytomegalovirus (CMV) infection is a well-recognized cause of perinatal morbidity and mortality, occurring in 0.5% to 2% of live births.1 Approximately 10% of infants with congenital CMV infection are symptomatic and may develop hepatosplenomegaly, pneumonitis, microcephaly, deafness, and thrombocytopenia.1 Longterm serious sequelae such as mental retardation, seizures, sensorineural deafness, and visual defects can occur.1,2 In contrast, perinatal/postnatal CMV infection has received considerably less attention although infection rates up to 40% have been reported in the first 6 months of life.1,3 Perinatal CMV infection, often acquired at delivery via exposure to genital secretions or transmitted through
From the Departments of Dermatology,a the Department of Pediatrics, Section of Infectious Diseases,b the Department of Pathology,c and the Neuroscience Program,d Wake Forest University School of Medicine. Funding sources: None. Conflicts of interest: None identified. Reprint requests: Gil Yosipovitch, MD, Associate Professor, Wake Forest University School of Medicine, Department of Dermatology, Medical Center Blvd, Winston-Salem, NC 27157. E-mail: [email protected]. 0190-9622/$32.00 ª 2006 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2005.04.037
breast-feeding, is usually asymptomatic with no long-term sequelae; however, serious disease has been reported in preterm infants.4,5 Numerous cutaneous findings have been described in infants with congenital CMV infection including jaundice, petechiae, purpura, and ‘‘blueberry muffin’’ lesions, which represent extramedullary (dermal) hematopoiesis and appear as violaceous, dark blue to purple papules and nodules that can last for weeks.6 Perineal ulcers caused by CMV are very rare in neonates with perinatal or postnatal CMV infection. We describe an unusual presentation for CMV infection in a preterm but otherwise immunocompetent neonate with eroded and ulcerated papulonodules on the perineal and gluteal regions. This case is interesting in terms of its cutaneous presentation, which is usually seen in patients with systemic sequelae and/or severe immunocompromise.
CASE REPORT The patient was born at 33 and 3/7 weeks’ gestation via cesarean section with a birth weight of 6 lb, 4 oz. The child’s prenatal history was significant for supraventricular tachycardia (SVT), abdominal ascites, and pericardial effusion. The mother was RPR nonreactive, hepatitis B negative,
J AM ACAD DERMATOL
Case reports 537
VOLUME 54, NUMBER 3
Fig 1. Eroded, excoriated, and umbilicated papules and nodules on the buttocks, perineum, medial thighs, and proximal scrotum.
rubella immune, and HIV negative. She received betamethasone before delivery, and had artificial rupture of membranes at delivery. The child’s Apgar scores were 4 at 1 minute, 6 at 5 minutes, and 8 at 10 minutes; because of apnea, he was intubated. Eventually, the child’s heart rate required control with propanolol. The child was breast-fed for the first 2 weeks of life. At approximately 4 weeks after delivery, the child developed a ‘‘diaper rash’’ that was initially and unsuccessfully treated with zinc oxide paste and over-the-counter steroid agents. The family reported frequent diaper changes using disposable diapers. At approximately 12 weeks of age, physical examination revealed a well-appearing infant with normal vital signs. No lymphadenopathy or hepatosplenomegaly was noted. Examination of the skin revealed erythematous, umbilicated papules and nodules on the buttocks, perineum, medial thighs, and proximal scrotum (Fig 1). Many of the lesions were eroded. A background of erythema was present. The rest of the skin examination was unremarkable. A 3-mm punch biopsy was performed; it displayed hyperkeratosis overlying pseudoepitheliomatous hyperplasia (Fig 2, A). Within the dermis, there was a moderately heavy superficial perivascular and interstitial inflammatory infiltrate composed mostly of lymphocytes, histiocytes, and scant plasma cells. In some dermal vessels, endothelial cells were enlarged and contained intranuclear inclusions (Fig 2,
Fig 2. A, Punch biopsy displaying hyperkeratosis overlying pseudoepitheliomatous hyperplasia. (Hematoxylineosoin stain; original magnification: 32.) B, Superficial perivascular and interstitial inflammatory infiltrate composed mostly of lymphocytes, histiocytes, and scant plasma cells. Enlarged endothelial cells (black arrow) of some dermal vessels contained intranuclear inclusions typical of CMV. (Hematoxylin-eosoin stain; original magnification: 320.)
B). CD1a stain was negative, ruling out Langerhans cell histiocytosis. Immunoperoxidase stain of the tissue for CMV was positive (Fig 3). Serum CMV IgM and IgG (16 AU/mL) were both positive, but urine culture was negative. Liver transaminases, albumin, alkaline phosphatase, and bilirubin were within normal limits, as was a complete blood cell count with platelets with differential. HIV testing was negative. A computed tomographic scan of the head without contrast showed no intracranial abnormality, and a chest radiograph was normal. An ophthalmologic examination elucidated no evidence
538 Case reports
J AM ACAD DERMATOL MARCH 2006
Fig 3. Immunoperoxidase stain of the tissue for CMV was positive in scattered cells. (Original magnification: 340.)
of retinal involvement or other ocular abnormality, and no hearing deficit was elicited. Serologic studies of the mother were not performed. At his 2-month follow-up, the patient’s lesions had resolved with no specific therapy, and there were no obvious physical sequelae.
DISCUSSION Although rare, cutaneous disease caused by CMV has been reported in immunocompetent patients; uncommon cutaneous presentations in infants and children with CMV infection include scleredema7 and papular acrodermatitis of childhood (GianottiCrosti syndrome).8 A wide spectrum of cutaneous manifestations of CMV infection has been reported in immunocompromised pediatric and adult patients, including transplant recipients and patients with acquired immune deficiency syndrome (AIDS).9-12 Presentations of disseminated CMV disease from the literature include perianal and rectal ulceration,13 indurated hyperpigmented nodules or plaques,14 papular and pruritic eruptions, and vesiculobullous lesions.15 CMV-associated ulcerative diaper dermatitis was reported in a 6-month-old AIDS patient. In this case, the eruption on the perineum was the initial manifestation of a disseminated and subsequently fatal infection.16 The patient in that case had vesicles, bullae, pustules, and signs and symptoms of systemic disease. Our patient had no evidence of extracutaneous sequelae, and the lesions spontaneously resolved without any specific treatment for the CMV infection. Finally, perineal ulcers have been reported in immunocompromised adults as well.10,13,17-21 The precise mode of acquisition of CMV in our patient is not clear although breast milk is a possibility. Besides breast milk, other sources of CMV include exposure to maternal cervical secretions, transmission from other people, or transmission by blood.22 Because our patient was born via cesarean section, transmission via maternal fluids
during labor is less likely. Also, the infant did not receive any blood transfusions. CMV has been shown in the breast milk of 32% to 96% of seropositive mothers with a transmission rate of 37%.23 Transmission of CMV through breast-feeding could result in symptomatic CMV infections, including sepsis-like syndromes.23 Breast milk viral load and duration of breast-feeding may influence the rate of acquiring CMV infection.24 Severe disease is uncommon in term infants when CMV is contracted by this means because the child has passive immunity by acquired maternal antibodies.25 Transfer of the majority of protective antibodies occurs at 28 weeks, and thus severely premature neonates are much more susceptible.25 Although premature, our patient was born at 33 3/7 weeks and may have acquired such antibodies, thus decreasing the risk of severe disease. Confirming the decreased risk of postdelivery infection even in preterm infants, Vollmer et al26 suggested that CMV acquired postnatally via breast milk does not have a negative affect on neurodevelopment, even in neonates born at 28 weeks gestation.26 Since the virus is ubiquitous and we do not have serologic evidence of acute CMV infection in the mother, it is very possible that our patient acquired CMV through horizontal transmission by direct contact with virus-containing secretions from older siblings at home or from day-care contacts. With regard to the infant’s arrhythmia, no reports of CMV-associated arrhythmia to our knowledge exist in the literature. CMV pericarditis has been reported, including in an infant with congenital heart disease,27 and, in a series of 26 viral myocarditis cases, CMV was detected in 1 case by polymerase chain reaction.28 Respiratory syncytial virus has been reported in association with supraventricular tachycardia, and, in those cases with structurally normal hearts, the tachycardias were self-limited.29 We doubt the supraventricular tachycardia and CMV infection are related in our patient. The differential diagnosis of erythematous, eroded and ulcerated papules and nodules in the perineum of an infant includes Jacquet’s erosive diaper dermatitis, irritant or contact dermatitis, intertrigo, candida infection, viral causes (herpes simplex virus, human papilloma virus, CMV, human immunodeficiency virus, molluscum contagiosum), Langerhans cell histiocytosis, granuloma gluteale infantum and nutritional deficiencies such as acrodermatitis enteropathica. Jacquet’s diaper dermatitis deserves mention as it displays well-demarcated punched-out ulcers and is often the result of infrequent diaper changes; therefore, it responds to excellent diapering practices.30 Because several of
J AM ACAD DERMATOL
Case reports 539
VOLUME 54, NUMBER 3
the entities in the above differential diagnosis are worsened by poor skin care, frequent diaper changes should be part of the first step in the management of any such eruption. Treatment options for perinatal/postnatal CMV infection are limited. Although ganciclovir, a guanosine analogue that selectively inhibits CMV DNA polymerase, has been used in the treatment of symptomatic congenital CMV infection,31,32 the role of ganciclovir in treating perinatal/postnatal CMVinfected infants is yet to be determined, and there are no published controlled studies. Diagnosis of CMV disease can be difficult; histopathologic evaluation is extremely valuable in difficult cases. REFERENCES 1. Stagno S. Cytomegalovirus. In: Remington JS, Klein JO, editors. Infectious diseases of the fetus and newborn infant. 4th ed. Philadelphia: WB Saunders; 1995. pp. 312-53. 2. Prober CG, Enright AM. Congenital cytomegalovirus (CMV) infections: hats off to Alabama. J Pediatr 2003;143:4-6. 3. Ahlfors K, Ivarsson SA, Harris S. Report on a long-term study of maternal and congenital CMV infection in Sweden. Review of prospective studies available in literature. Scand J Infect Dis 1999;31:443-57. 4. Forsgren M. Cytomegalovirus in breast milk: reassessment of pasteurization and freeze-thawing. Pediatr Research 2004;56:526-8. 5. Dworsky M, Yow M, Stagno S, Pass RF, Alford CA. Cytomegalovirus infection of breast milk and transmission in infancy. Pediatrics 1983;72:295-9. 6. Brough AJ, Jones D, Page RH, Mizukami I. Dermal erythropoiesis in neonatal infantsea manifestation of intrauterine viral disease. Pediatrics 1967;40:627-35. 7. Heilbron B, Saxe N. Scleredema in an infant. Arch Dermatol 1986;122:1417-9. 8. Taieb A, Plantin P, Du Pasquier P, Guillet G, Maleville J. Gianotti-Crosti syndrome: a study of 26 cases. Br J Dermatol 1986;115:49-59. 9. Lee JY. Cytomegalovirus infection involving the skin in immunocompromised hosts. A clinicopathologic study. Am J Clin Pathol 1989;92:96-100. 10. Pariser RJ. Histologically specific skin lesions in disseminated cytomegalovirus infection. J Am Acad Dermatol 1983;9:937-46. 11. Bournerias I, Boisnic S, Patey O, Deny P, Gharakhanian S, Duffo B, et al. Unusual cutaneous cytomegalovirus involvement in patients with acquired immunodeficiency syndrome. Arch Dermatol 1989;125:1243-6. 12. Horn TD, Hood AF. Clinically occult cytomegalovirus present in skin biopsy specimens in immunosuppressed hosts. J Am Acad Dermatol 1989;21(4 Pt 1):781-4. 13. Nakoneczna I, Kay S. Fatal disseminated cytomegalic inclusion disease in an adult presenting with a lesion of the gastrointestinal tract. Am J Clin Pathol 1967;47:124-8. 14. Feldman PS, Walker AN, Baker R. Cutaneous lesions heralding disseminated cytomegalovirus infection. J Am Acad Dermatol 1982;7:545-8.
15. Bhawan J, Gellis S, Ucci A, Chang TW. Vesiculobullous lesions caused by cytomegalovirus infection in an immunocompromised adult. J Am Acad Dermatol 1984;11(4 Pt 2): 743-7. 16. Thiboutot DM, Beckford A, Mart CR, Sexton M, Maloney ME. Cytomegalovirus diaper dermatitis. Arch Dermatol 1991;127: 396-8. 17. Horn TD, Hood AF. Cytomegalovirus is predictably present in perineal ulcers from immunosuppressed patients. Arch Dermatol 1990;126:642-4. 18. Kwan TH, Kaufman HW. Acid-fast bacilli with cytomegalovirus and herpesvirus inclusions in the skin of an AIDS patient. Am J Clin Pathol 1986;85:236-8. 19. Williams G, Stretton TB, Leonard JC. Cytomegalic inclusion disease and Pneumocystis carinii infection in an adult. Lancet 1960;2:951-5. 20. Walker JD, Chesney TM. Cytomegalovirus infections of the skin. Am J Dermatopathol 1982;4:263-5. 21. Symers WS. Generalized cytomegalic inclusion body disease associated with Pneumocystis pneumonia in adults. J Clin Pathol 1960;13:1-21. 22. de Cates, Gray J, Roberton NRC, Walker J. Acquisition of cytomegalovirus infection by premature neonates. J Infect 1994;28:25-30. 23. Hamprecht K, Maschmann J, Vochem M, Dietz K, Speer CP, Jahn G. Epidemiology of transmission of cytomegalovirus from mother to preterm infant by breastfeeding. Lancet 2001 17;357:513-8. 24. Dworsky, Yow M, Stagno S, Pass RF, Alford CA. Cytomegalovirus infection of breast milk and transmission in infancy. Pediatrics 1983;72:295-9. 25. Bryant P, Morley C, Garland S, Curtis N. Cytomegalovirus transmission from breast milk in premature babies: does it matter? Arch Dis Child Fetal Neonatal 2002;87:F75-7. 26. Vollmer B, Seibold-Weiger K, Schmitz-Salue C, Hamprecht K, Goelz R, Krageloh-Mann I, Speer CP. Postnatally acquired cytomegalovirus infection via breast milk: effects on hearing and development in preterm infants. Pediatr Infect Dis J 2004;23:322-7. 27. Campbell PT, Li JS, Wall TC, O’Connor CM, Van Trigt P, Kenney RT, et al. Cytomegalovirus pericarditis: a case series and review of the literature. Am J Med Sci 1995;309:229-34. 28. Martin AB, Webber S, Fricker FJ, Jaffe R, Demmler G, Kearney D, et al. Acute myocarditis. Rapid diagnosis by PCR in children. Circulation 1994;90:330-9. 29. Donnerstein RL, Berg RA, Shehab Z, Ovadia M. Complex atrial tachycardias and respiratory syncytial virus infections in infants. J Pediatr 1994;125:23-8. 30. Halbert AR, Chan JJ. Anogenital and buttock ulceration in infancy. Australas J Dermatol 2002;43:1-6. 31. Whitley RJ, Cloud G, Gruber W, Storch GA, Demmler GJ, Jacobs RF, et al. Ganciclovir treatment of symptomatic congenital cytomegalovirus infection: results of a phase II study. J Infect Dis 1997;175:1080-6. 32. Kimberlin DW, Lin CY, Sanchez PJ, Demmler GJ, Dankner W, Shelton M, et al. Effect of ganciclovir therapy on hearing in symptomatic congenital cytomegalovirus disease involving the central nervous system: a randomized controlled trial. J Pediatr 2003;143:16-25.
J AM ACAD DERMATOL MARCH 2006
18. Jakobiec FA, Austin P, Iwamoto T, Trokel SL, Marquardt MD, Harrison W. Primary infiltrating signet ring carcinoma of the eyelids. Ophthalmology 1983;90:291-9. 19. Thomas JW, Fu YS, Levine MR. Primary mucinous sweat gland carcinoma of the eyelid simulating metastatic carcinoma. Am J Ophthalmol 1979;87:29-33.
20. Kuno Y, Numata T, Kanzaki T. Adenocarcinoma with signet ring cells of the axilla showing apocrine features: a case report. Am J Dermatopathol 1999;21:37-41. 21. Kuno Y, Tsuji T, Yamamoto K. Adenocarcinoma with signet ring cells of the axilla: two case reports and review of the literature. J Dermatol 1999;26:390-5.
Perineal ulcers in an infant: An unusual presentation of postnatal cytomegalovirus infection John G. Hancox, MD,a Avinash K. Shetty, MD,b Omar P. Sangueza, MD,c and Gil Yosipovitch, MDa,d Wake Forest, NC
Cytomegalovirus (CMV) disease can cause significant morbidity and mortality in neonates and immunosuppressed patients. Cutaneous disease is rare, even in at-risk patients. We report a case of CMV with perineal papules, erosions, and ulcers in a preterm but presumably immunocompetent patient, whose diagnosis was first suggested by skin biopsy and confirmed with serologic testing. The mode of transmission of CMV was unclear. The lesions resolved without CMV-specific therapy, and the child had no apparent systemic sequelae of infection. CMV disease should be considered in the differential diagnosis of perineal erosions and ulcers in infancy. In addition to the case, we briefly review the literature on cutaneous manifestations of CMV disease. ( J Am Acad Dermatol 2006;54:536-9.)
C
ongenital cytomegalovirus (CMV) infection is a well-recognized cause of perinatal morbidity and mortality, occurring in 0.5% to 2% of live births.1 Approximately 10% of infants with congenital CMV infection are symptomatic and may develop hepatosplenomegaly, pneumonitis, microcephaly, deafness, and thrombocytopenia.1 Longterm serious sequelae such as mental retardation, seizures, sensorineural deafness, and visual defects can occur.1,2 In contrast, perinatal/postnatal CMV infection has received considerably less attention although infection rates up to 40% have been reported in the first 6 months of life.1,3 Perinatal CMV infection, often acquired at delivery via exposure to genital secretions or transmitted through
From the Departments of Dermatology,a the Department of Pediatrics, Section of Infectious Diseases,b the Department of Pathology,c and the Neuroscience Program,d Wake Forest University School of Medicine. Funding sources: None. Conflicts of interest: None identified. Reprint requests: Gil Yosipovitch, MD, Associate Professor, Wake Forest University School of Medicine, Department of Dermatology, Medical Center Blvd, Winston-Salem, NC 27157. E-mail: [email protected]. 0190-9622/$32.00 ª 2006 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2005.04.037
breast-feeding, is usually asymptomatic with no long-term sequelae; however, serious disease has been reported in preterm infants.4,5 Numerous cutaneous findings have been described in infants with congenital CMV infection including jaundice, petechiae, purpura, and ‘‘blueberry muffin’’ lesions, which represent extramedullary (dermal) hematopoiesis and appear as violaceous, dark blue to purple papules and nodules that can last for weeks.6 Perineal ulcers caused by CMV are very rare in neonates with perinatal or postnatal CMV infection. We describe an unusual presentation for CMV infection in a preterm but otherwise immunocompetent neonate with eroded and ulcerated papulonodules on the perineal and gluteal regions. This case is interesting in terms of its cutaneous presentation, which is usually seen in patients with systemic sequelae and/or severe immunocompromise.
CASE REPORT The patient was born at 33 and 3/7 weeks’ gestation via cesarean section with a birth weight of 6 lb, 4 oz. The child’s prenatal history was significant for supraventricular tachycardia (SVT), abdominal ascites, and pericardial effusion. The mother was RPR nonreactive, hepatitis B negative,
J AM ACAD DERMATOL
Case reports 537
VOLUME 54, NUMBER 3
Fig 1. Eroded, excoriated, and umbilicated papules and nodules on the buttocks, perineum, medial thighs, and proximal scrotum.
rubella immune, and HIV negative. She received betamethasone before delivery, and had artificial rupture of membranes at delivery. The child’s Apgar scores were 4 at 1 minute, 6 at 5 minutes, and 8 at 10 minutes; because of apnea, he was intubated. Eventually, the child’s heart rate required control with propanolol. The child was breast-fed for the first 2 weeks of life. At approximately 4 weeks after delivery, the child developed a ‘‘diaper rash’’ that was initially and unsuccessfully treated with zinc oxide paste and over-the-counter steroid agents. The family reported frequent diaper changes using disposable diapers. At approximately 12 weeks of age, physical examination revealed a well-appearing infant with normal vital signs. No lymphadenopathy or hepatosplenomegaly was noted. Examination of the skin revealed erythematous, umbilicated papules and nodules on the buttocks, perineum, medial thighs, and proximal scrotum (Fig 1). Many of the lesions were eroded. A background of erythema was present. The rest of the skin examination was unremarkable. A 3-mm punch biopsy was performed; it displayed hyperkeratosis overlying pseudoepitheliomatous hyperplasia (Fig 2, A). Within the dermis, there was a moderately heavy superficial perivascular and interstitial inflammatory infiltrate composed mostly of lymphocytes, histiocytes, and scant plasma cells. In some dermal vessels, endothelial cells were enlarged and contained intranuclear inclusions (Fig 2,
Fig 2. A, Punch biopsy displaying hyperkeratosis overlying pseudoepitheliomatous hyperplasia. (Hematoxylineosoin stain; original magnification: 32.) B, Superficial perivascular and interstitial inflammatory infiltrate composed mostly of lymphocytes, histiocytes, and scant plasma cells. Enlarged endothelial cells (black arrow) of some dermal vessels contained intranuclear inclusions typical of CMV. (Hematoxylin-eosoin stain; original magnification: 320.)
B). CD1a stain was negative, ruling out Langerhans cell histiocytosis. Immunoperoxidase stain of the tissue for CMV was positive (Fig 3). Serum CMV IgM and IgG (16 AU/mL) were both positive, but urine culture was negative. Liver transaminases, albumin, alkaline phosphatase, and bilirubin were within normal limits, as was a complete blood cell count with platelets with differential. HIV testing was negative. A computed tomographic scan of the head without contrast showed no intracranial abnormality, and a chest radiograph was normal. An ophthalmologic examination elucidated no evidence
538 Case reports
J AM ACAD DERMATOL MARCH 2006
Fig 3. Immunoperoxidase stain of the tissue for CMV was positive in scattered cells. (Original magnification: 340.)
of retinal involvement or other ocular abnormality, and no hearing deficit was elicited. Serologic studies of the mother were not performed. At his 2-month follow-up, the patient’s lesions had resolved with no specific therapy, and there were no obvious physical sequelae.
DISCUSSION Although rare, cutaneous disease caused by CMV has been reported in immunocompetent patients; uncommon cutaneous presentations in infants and children with CMV infection include scleredema7 and papular acrodermatitis of childhood (GianottiCrosti syndrome).8 A wide spectrum of cutaneous manifestations of CMV infection has been reported in immunocompromised pediatric and adult patients, including transplant recipients and patients with acquired immune deficiency syndrome (AIDS).9-12 Presentations of disseminated CMV disease from the literature include perianal and rectal ulceration,13 indurated hyperpigmented nodules or plaques,14 papular and pruritic eruptions, and vesiculobullous lesions.15 CMV-associated ulcerative diaper dermatitis was reported in a 6-month-old AIDS patient. In this case, the eruption on the perineum was the initial manifestation of a disseminated and subsequently fatal infection.16 The patient in that case had vesicles, bullae, pustules, and signs and symptoms of systemic disease. Our patient had no evidence of extracutaneous sequelae, and the lesions spontaneously resolved without any specific treatment for the CMV infection. Finally, perineal ulcers have been reported in immunocompromised adults as well.10,13,17-21 The precise mode of acquisition of CMV in our patient is not clear although breast milk is a possibility. Besides breast milk, other sources of CMV include exposure to maternal cervical secretions, transmission from other people, or transmission by blood.22 Because our patient was born via cesarean section, transmission via maternal fluids
during labor is less likely. Also, the infant did not receive any blood transfusions. CMV has been shown in the breast milk of 32% to 96% of seropositive mothers with a transmission rate of 37%.23 Transmission of CMV through breast-feeding could result in symptomatic CMV infections, including sepsis-like syndromes.23 Breast milk viral load and duration of breast-feeding may influence the rate of acquiring CMV infection.24 Severe disease is uncommon in term infants when CMV is contracted by this means because the child has passive immunity by acquired maternal antibodies.25 Transfer of the majority of protective antibodies occurs at 28 weeks, and thus severely premature neonates are much more susceptible.25 Although premature, our patient was born at 33 3/7 weeks and may have acquired such antibodies, thus decreasing the risk of severe disease. Confirming the decreased risk of postdelivery infection even in preterm infants, Vollmer et al26 suggested that CMV acquired postnatally via breast milk does not have a negative affect on neurodevelopment, even in neonates born at 28 weeks gestation.26 Since the virus is ubiquitous and we do not have serologic evidence of acute CMV infection in the mother, it is very possible that our patient acquired CMV through horizontal transmission by direct contact with virus-containing secretions from older siblings at home or from day-care contacts. With regard to the infant’s arrhythmia, no reports of CMV-associated arrhythmia to our knowledge exist in the literature. CMV pericarditis has been reported, including in an infant with congenital heart disease,27 and, in a series of 26 viral myocarditis cases, CMV was detected in 1 case by polymerase chain reaction.28 Respiratory syncytial virus has been reported in association with supraventricular tachycardia, and, in those cases with structurally normal hearts, the tachycardias were self-limited.29 We doubt the supraventricular tachycardia and CMV infection are related in our patient. The differential diagnosis of erythematous, eroded and ulcerated papules and nodules in the perineum of an infant includes Jacquet’s erosive diaper dermatitis, irritant or contact dermatitis, intertrigo, candida infection, viral causes (herpes simplex virus, human papilloma virus, CMV, human immunodeficiency virus, molluscum contagiosum), Langerhans cell histiocytosis, granuloma gluteale infantum and nutritional deficiencies such as acrodermatitis enteropathica. Jacquet’s diaper dermatitis deserves mention as it displays well-demarcated punched-out ulcers and is often the result of infrequent diaper changes; therefore, it responds to excellent diapering practices.30 Because several of
J AM ACAD DERMATOL
Case reports 539
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the entities in the above differential diagnosis are worsened by poor skin care, frequent diaper changes should be part of the first step in the management of any such eruption. Treatment options for perinatal/postnatal CMV infection are limited. Although ganciclovir, a guanosine analogue that selectively inhibits CMV DNA polymerase, has been used in the treatment of symptomatic congenital CMV infection,31,32 the role of ganciclovir in treating perinatal/postnatal CMVinfected infants is yet to be determined, and there are no published controlled studies. Diagnosis of CMV disease can be difficult; histopathologic evaluation is extremely valuable in difficult cases. REFERENCES 1. Stagno S. Cytomegalovirus. In: Remington JS, Klein JO, editors. Infectious diseases of the fetus and newborn infant. 4th ed. Philadelphia: WB Saunders; 1995. pp. 312-53. 2. Prober CG, Enright AM. Congenital cytomegalovirus (CMV) infections: hats off to Alabama. J Pediatr 2003;143:4-6. 3. Ahlfors K, Ivarsson SA, Harris S. Report on a long-term study of maternal and congenital CMV infection in Sweden. Review of prospective studies available in literature. Scand J Infect Dis 1999;31:443-57. 4. Forsgren M. Cytomegalovirus in breast milk: reassessment of pasteurization and freeze-thawing. Pediatr Research 2004;56:526-8. 5. Dworsky M, Yow M, Stagno S, Pass RF, Alford CA. Cytomegalovirus infection of breast milk and transmission in infancy. Pediatrics 1983;72:295-9. 6. Brough AJ, Jones D, Page RH, Mizukami I. Dermal erythropoiesis in neonatal infantsea manifestation of intrauterine viral disease. Pediatrics 1967;40:627-35. 7. Heilbron B, Saxe N. Scleredema in an infant. Arch Dermatol 1986;122:1417-9. 8. Taieb A, Plantin P, Du Pasquier P, Guillet G, Maleville J. Gianotti-Crosti syndrome: a study of 26 cases. Br J Dermatol 1986;115:49-59. 9. Lee JY. Cytomegalovirus infection involving the skin in immunocompromised hosts. A clinicopathologic study. Am J Clin Pathol 1989;92:96-100. 10. Pariser RJ. Histologically specific skin lesions in disseminated cytomegalovirus infection. J Am Acad Dermatol 1983;9:937-46. 11. Bournerias I, Boisnic S, Patey O, Deny P, Gharakhanian S, Duffo B, et al. Unusual cutaneous cytomegalovirus involvement in patients with acquired immunodeficiency syndrome. Arch Dermatol 1989;125:1243-6. 12. Horn TD, Hood AF. Clinically occult cytomegalovirus present in skin biopsy specimens in immunosuppressed hosts. J Am Acad Dermatol 1989;21(4 Pt 1):781-4. 13. Nakoneczna I, Kay S. Fatal disseminated cytomegalic inclusion disease in an adult presenting with a lesion of the gastrointestinal tract. Am J Clin Pathol 1967;47:124-8. 14. Feldman PS, Walker AN, Baker R. Cutaneous lesions heralding disseminated cytomegalovirus infection. J Am Acad Dermatol 1982;7:545-8.
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