Neonatal Dermatologic Potpourri

Transcription

1 Neonatal Dermatologic Potpourri Kimberly A. Horii, MD Professor of Pediatrics Children s Mercy Hospitals & Clinics Division of Dermatology Kansas City, Missouri

2 Neonatal Dermatologic Potpourri I have no financial relationships with the manufacturers of any commercial products and/or provider of commercial services discussed in this CME activity I do not intend to discuss off label use of a commercial product/device in my presentation 2

3 Objectives After participating in the presentation, you should have an increased knowledge and enhanced competence to Recognize several dermatoses seen in the neonate Develop a brief differential diagnosis for vesiculopustular lesions in the neonate Discuss differences in premature neonatal skin 3

4 Neonatal Skin Disorders Premature Neonatal Skin Neonatal Skin Infections Anetoderma of Prematurity Developmental Defects Vesiculopustular lesions Diaper Dermatitis

5 Premature Neonatal Skin Increasingly improved survival of premature infants Stratum corneum (epidermal barrier) becomes functionally mature between 32 and 34 weeks gestation Stratum corneum of premature infants is less functionally effective than full term infants Takes at least 2-4 weeks postnatal age to develop a more competent barrier (longer for extremely premature infants)

6 Premature Neonatal Skin Premature neonates experience significant skin related morbidity due to: Insensible water loss Fluid and electrolyte imbalance Increased heat loss Physical and mechanical skin injury from multiple procedures Percutaneous absorption of topical agents Sepsis secondary to skin fragility and breakdown

7 Opportunistic Fungal Infections Premature neonates are at an increased risk of opportunistic fungal infections Immature immune function Prolonged broad spectrum antibiotic exposure Skin trauma/damage with disrupted epidermal barrier Cutaneous infections can lead to invasive infection with high morbidity & mortality Aspergillosis, Candidiasis, Zygomycetes (Rhizopus & Mucor)

8 Opportunistic Cutaneous Fungal Infections Skin lesions in premature neonates can present with Erythematous papules, pustules, or plaques Erosions & ulcers with hemorrhagic eschar or crust May also have signs of clinical instability Emergent biopsy for histology & culture Aids in diagnosis & treatment options Workup for possible systemic infection

9 Congenital Cutaneous Candidiasis Rare congenital skin infection presenting at birth or within the first 6 days of life Characteristic skin patterns Papulopustular eruption (morbilliform appearing eruption) Burn-like dermatitis or extensive erosions More common in premature neonates

10 Congenital Cutaneous Candidiasis Affected premature infants have increased morbidity and mortality due to systemic involvement and require comprehensive workup Recommend Infectious Diseases involvement Premature infants usually require systemic antifungal therapy

11 Anetoderma of Prematurity Atrophic patches & outpouchings of skin Not associated with prior trauma?relationship to monitoring leads/tape Common locations Trunk and proximal extremities Lesions appear within 2-3 months of life in extremely premature infants (24-29 weeks gestation) Over years lesions evolve into anetoderma Histology: loss of dermal elastic tissue

12 Developmental Defects Cranial Dysraphism Aplasia cutis congenita Spinal Dysraphism

13 Cutaneous Signs of Cranial Dysraphism Up to 1/3 of congenital non-traumatic scalp nodules may connect to the underlying central nervous system Cephaloceles- the term refers to a congenital herniation of intracranial structures through a skull defect Includes meningoceles and encephaloceles

14 Cutaneous Signs of Cranial Dysraphism Smaller & atretic cephaloceles may be mistaken for a variety of cutaneous lesions including: Infantile hemangiomas, hematomas, aplasia cutis, dermoid or epidermal cysts Cephaloceles can occur in frontal, parietal, or occipital scalp Recent study showed majority of cases were on the vertex Usually midline or slightly lateral to midline

15 Hair Collar Sign Evaluation of an infant with a congenital scalp nodule surrounded by a hair collar should include a complete physical exam, neurologic exam, & MRI (specifically focusing on scalp lesion) The presence of a co-existent vascular stain may increase the suspicion of associated cranial dysraphism (intracranial connection to the mass) Neurosurgical referral suggested when evidence of intracranial communication on imaging

16 Aplasia Cutis Congenita Congenital absence of skin Most common on scalp Solitary or multiple lesions Can present with an ulcer and granulation tissue or a superficial erosion Recommend wound care to area Eventually heals into scar tissue with associated alopecia

17 Aplasia Cutis Congenita May be secondary to incomplete closure of the neural tube Membranous aplasia cutis Translucent membrane with associated hair collar sign Multiple possible rare associated anomalies Majority of patients with localized lesions have a good prognosis

18 Markers of Spinal Dysraphism (Spinal Cord Abnormalities) Midline lumbosacral hemangiomas Aplasia cutis congenita Midline lipomas Midline skin tags Hypertrichosis of midline spine Deep and large sacral dimple (isolated simple dimples do not require w/u) Dimples >2.5 cm from the anal verge Asymmetrically deviated gluteal cleft Midline congenital melanocytic nevi (low risk) 2 or more findings increases risk of spinal dysraphism

19 Spinal Dysraphism Work-up Radiologic imaging provides a sensitive screening modality Imaging should be performed prior to surgical intervention MRI is the diagnostic gold standard High resolution ultrasound may be a screening alternative in infants less than 4 months of age (prior to skeletal maturation) High risk cutaneous markers are best evaluated still by MRI

20 Vesicular & Pustular Lesions in Neonates: Differential Diagnoses Non-infectious Erythema toxicum Pustular melanosis Miliaria Neonatal acne Bullous mastocytosis Incontinentia pigmenti Epidermolysis bullosa Langerhans cell histiocytosis Infectious Herpes simplex Impetigo-Staph aureus Candidiasis Scabies Group B streptococcus Pseudomonas Syphilis Aspergillus Pustular leukemoid reaction

21 Distinguishing Non-infectious From Infectious Neonatal Eruptions Smears of skin lesions Gram stain KOH Wright stain Cultures from skin lesions Bacterial Viral Fungal Skin biopsy

22 Transient Benign Lesions Erythema Toxicum Transient Neonatal Pustular Melanosis

23 Erythema Toxicum Self-limited, asymptomatic condition Begins during the first few days of life Most common eruption in the term neonate Blotchy red macules and patches with overlying papule or pustule More commonly on the trunk, buttocks, and proximal limbs Lesions wax and wane, resolve within 1 week Wright stain shows numerous eosinophils

24 Transient Neonatal Pustular Melanosis Unknown etiology Pustular skin eruption More common in darker pigmented neonates 3 stages 1 st superficial pustules present at or near birth 2 nd superficial erosions with collarette of scale 3 rd hyperpigmented macules, may last months Gram stain: neutrophils, no organism

25 Neonatal Skin Infections Impetigo neonatorum Neonatal herpes simplex virus (HSV)

26 Impetigo Neonatorum Multiple pustules most common in the diaper region and lower abdomen in neonates Gram stain: gram positive cocci in clusters Bacterial culture: Staphylococcus aureus Usually requires systemic antibiotic

27 Neonatal Herpes Simplex Infection with Herpes Simplex virus 1/3200 deliveries Retrospective study found a similar prevalence as bacterial meningitis in neonates admitted from a large ER (0.2%) Neonates whose mothers develop a primary genital HSV infection late in pregnancy are at highest risk Majority of neonates acquire infection during delivery

28 Neonatal Herpes Simplex 3 categories of neonatal HSV Skin, eye, mucous membrane (SEM) involvement (45%) Central nervous system involvement (30%) Disseminated infection (lungs, liver, CNS, SEM, adrenal) (25%) Affected neonates usually present between 5-21 days of life Skin, eye, mucous membrane involvement presents most commonly at days of age

29 Neonatal Herpes Simplex Factors associated with neonatal HSV Maternal primary HSV infection, vaginal delivery, prematurity, postnatal HSV contact, seizures, vesicular rash, hypothermia, lethargy, CSF pleocytosis, hepatosplenomegaly, elevated hepatic enzymes, maternal fever, respiratory distress, and thrombocytopenia If skin lesions are present Grouped 2-4 mm vesicles on an erythematous base which evolve into pustules and crusted erosions (common on scalp)

30 Neonatal Herpes Simplex High associated morbidity and mortality if not diagnosed/treated early Recommended work-up of suspicious skin lesions Viral culture of base of vesicle/lesion Consider HSV PCR of base of vesicle/lesion Thorough systemic workup Lumbar puncture for HSV PCR & viral culture Surface swabs sent for viral culture of mouth, conjunctiva, nasopharynx, & rectum Rule out disseminated infection Systemic acyclovir at high doses Infectious Diseases consultation

31 Diaper Dermatitis Variants of diaper dermatitis Common Irritant contact diaper dermatitis Infectious-candida, staph, strep Seborrheic dermatitis Allergic contact dermatitis Uncommon Psoriasis Zinc deficiency Langerhans Cell Histiocytosis 31

32 Irritant Contact Diaper Dermatitis Affects up to 25% of infants wearing diapers Due to increased skin hydration, exposure to chemical irritants, & friction beneath the diaper Chronic stooling can exacerbate Erythema involving the convex surfaces of the buttocks, perineum, lower abdomen, & thighs Commonly spares the skin folds Severe cases may have superficial erosions 32

33 Treatment of Irritant Contact Diaper Dermatitis Frequent diaper changes?super absorbent diapers Gentle cleansing Possible avoidance of diaper wipes Topical barriers ointments/cream Zinc oxide White petrolatum 33

34 Treatment of Infectious Diaper Dermatitis Candidal diaper dermatitis Antifungal cream (effective for yeast) or ointment Azoles (clotrimazole, miconazole, ketoconazole) Impetigo Topical or oral antibiotics Perianal Strep or Strep Intertrigo Oral antibiotic All types benefit from topical barriers 34

35 Neonatal Skin Disorders Premature Neonatal Skin Neonatal Skin Infections Anetoderma of Prematurity Developmental Defects Vesiculopustular lesions Diaper Dermatitis

36 References Johnson DE et al. Extremely preterm infant skin care. Adv Neonatal Care 2016;16(5S):S26-S32. Kalia YN et al. Development of skin barrier function in premature infants. J Invest Dermatol 1998;111: Csoma ZR et al. Iatrogenic skin disorders and related factors in newborn infants. Pediatr Dermatol 2016;33(5): Visscher MO et al. Newborn infant skin: physiology, development, and care. Clin Dermatol 2015;33: Rogdo B et al. Primary cutaneous aspergillosis in a preterm neonate. BMJ 2014 published online doi:10/1136/bcr Gallai F et al. Simultaneous primary invasive cutaneous aspergillosis in two preterm twins: case report and review of the literature. BMC Infect Dis 2017;17:535. Kucinskiene V et al. Cutaneous fungal infection in a neonatal intensive care unit patient. Pediatr Dermatol 2014;31(3): Barton M et al. Early-onset invasive candidiasis in extremely low birth weight infants: perinatal acquisition predicts poor outcome. CID 2017;64:

37 References Marcoux D et al. Deep cutaneous fungal infections in immunocompromised children. J Am Acad Dermatol 2009;61: Horii KA et al. Emerging cutaneous infections in the premature neonate. Adv Dermatol 2007;23: Darmstadt GL et al. Congenital cutaneous candidiasis: clinical presentation, pathogenesis, and management guidelines. Pediatrics 2000;105: Kaufman DA et al. Congenital cutaneous candidiasis: prompt systemic treatment is associated with improved outcomes in neonates. CID 2017;64(10): Prizant TL et al. Spontaneous atrophic patches in extremely premature infants. Arch Dermatol 1996;132: Goujon E. Anetoderma of prematurity. Arch Dermatol 2010;146(5): Bellet JS. Developmental anomalies of the skin. Semin Perinatol 2013;37: Sewell MJ et al. Neural tube dysraphism: review of cutaneous markers and imaging. Pediatr Dermatol 2015;32(2):

38 References Bessis D et al. The scalp hair collar and tuft signs. J Am Acad Dermatol 2017;76: Chien M et al. The hair-collar sign. J Pediatr 2016;168:246. Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification J Am Acad Dermatol 1986;14: Drolet B et al. Membranous aplasia cutis with hair collars. Congenital absence of skin or neuroectodermal defect? Arch Dermatol 1995;131: Patel DP et al. Aplasia cutis congenital: evaluation of signs suggesting extracutaneous involvement. Pediatr Dermatol 2018;35:e59-e61. Drolet BA et al. When good is not good enough: the predictive value of cutaneous lesions of the lumbosacral region for occult spinal dysraphism. Arch Dermatol 2004;140(9): Guggisberg D et a. Skin markers of occult spinal dyraphism in children. Arch Dermatol 2004;140(9): O Neill BR et al. Use of magnetic resonance imaging to detect occult spinal dysraphism in infants. J Neurosurg Pediatr 2017;19:

39 References Reginatto FP et al. Benign skin disease with pustules in the newborn. An Bras Dermatol 2016;91(2): Nanda S et al. Analytical study of pustular eruptions in neonates. Pediatr Dermatol 2002;19(3): Zhao CY et al. Blistering diseases in neonates. Curr Opin Pediatr 2016;28: Antaya RJ et al. Blisters and pustules in the newborn. Pediatr Ann 2010;39(10): Wagner A. Distinguishing vesicular and pustular disorders in the neonate. Curr Opin Pediatr 1997;9: Reginatto FP et al. Epidemiology and predisposing factors for erythema toxicum neonatorum and transient neonatal pustular: a multicenter study. Pediatr Dermatol 2017;34(4): Monteagudo B et al. Prospective study of erythema toxicum neonatorum. Pediatr Dermatol 2012;29(2): Koch LH et al. Congenital herpes simplex virus infection: two unique cutaneous presentations associated with probable intrauterine transmission. J Am Acad Dermatol 2009;60: Marquez L et al. A report of 3 cases and review of intrauterine herpes simplex virus infection. Pediatr Infect Dis J 2011;30:

40 References Caviness AC et al. Clinical and laboratory features of neonatal herpes simplex virus infection. Pediatr Infect Dis J 2008;27: Demeulemeester V et al. A rare infectious cause of severe neonatal skin lesions. BMJ Case Rep 2015: doi: /bcr Curfman Al et al. Initial presentation of neonatal herpes simplex virus infection. J Pediatr 2016;172: Pinninti S et al. Management of neonatal herpes simplex virus infection and exposure. Arch Dis Child Fetal Neonatal Ed 2014;99F240-F244. Kimberlin DW et al. Guidance on management of asymptomatic neonates born to women with active genital herpes lesions. Pediatrics 2013;131:e635-e646. Pinninti SG et al. Neonatal herpes simplex virus infections. Pediatr Clin N Am 2013;60: Ravanfar P et al. Diaper dermatitis. Curr Opin Pediatr 2012;24: Klunk C, Domingues E, Wiss K. An update on diaper dermatitis. Clin Dermatol 2014;32(4): Shin HT. Diagnosis and management of diaper dermatitis Pediatr Clin North Am 2014;61(2):

41 References Coughlin CC et al. Diaper dermatitis: clinical characteristics and differential diagnosis. Pediatr Dermatol 2014;31;Suppl 1: Van Gysel. Infections and skin diseases mimicking diaper dermatitis. Int J Dermatol 2016;55(suppl1):