Neonatal Eruptions Not to Miss!



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Neonatal Eruptions Not to Miss! Rash neonatales poco communes, para tener en cuenta Miriam Weinstein MD FRCPC University of Toronto Hospital for Sick Children Toronto, Canada

Disclosure No Disclosures relevant to this presentation

Objectives Be able to recognize worrisome rashes in neonates Be able to differentiate these rashes from common, benign conditions

Pustular Eruptions in the Neonate

Candida Infections Candida: Congenital Candida acquired in utero or during birth Uncommon Birth to 1 st week Neonatal Acquired postnatal Very common After first week

Congenital Candidiasis Onset from birth to first week Risk Factors: Prematurity Maternal history of vaginal candidiasis Eruption: Generalized red papules with pustules and scale Often palms and soles involved Systemic: baby unwell, temp instability DX: KOH for yeast cells and culture TX: Systemic antifungals

Erythema Toxicum Neonatorum Red macules, papules and pustules Palms and soles not involved between 24-48 hours of age Lesions last 1-2 days, new crops q few days Common in term infants; rare in prems Dx: clinical; smear of pustule: eosinophils Tx: none

Transient Neonatal Pustular Melanosis 3 lesion types: 1. Pustules with no erythema 2. Ruptured pustules with peripheral scale 3. Hyperpigmented macules Unknown etiology More common in term infants More common in dark-skinned infants Lesions always present at birth Dx: Clinical; smear of pustule shows PMNS Tx: none

Neonatal Acne Neonatal Cephalic Pustulosis Papules and pustules on red base Mostly cheeks Also rest of face and chest, scalp, eyelids, neck No comedones Mean onset: 2-3 weeks (can rarely start at 1 week) Asymptomatic?malassezia furfur as cause Can use: topical imidazole or low-potency steroids Can be difficult to tell apart from miliaria rubra

Infantile acne Acne (true acne) at 2-3 months comedones, papules, pustules, nodules Mostly on face Usually resolves in first 2-3 years of life Androgen cause increased sebaceous activity Treat as in adolescent acne (all off-label): Topicals: benzoyl peroxide, antibiotics, retinoids Oral: antibiotics (erythromycin), severe: isotretinoin

Acropustulosis of Infancy?etiology Itchy, tense, vesicopustules on hands/feet Hard to DDX from scabies (need scraping) Scabies < than 4 weeks of age is rare Can present at birth or first weeks, months Crops every 2-4 weeks, lasts 5-10 days Remits in 1-2 years Treatment: topical steroids

Vesicular Eruptions in the Neonate

Herpes Simplex Virus in the Newborn Congenital HSV (4%) Acquired in utero (ascending infection or viremia) Perinatal HSV (80-90%) From transmission during delivery 3 types: 1. Mucocutaneous 2. Disseminated 3. CNS Postnatal HSV (10%) From infected contact

Staphylococcal Scalded Skin Syndrome Abrupt onset Usually infants or small children Bullous eruption from S. aureus toxins Tender, superficial vesicle & erosions Often present only with collarette of scale Folds, face (crusts around mouth, eyes, nose) Early presentation: tender erythema Pain is common Fever and systemic symptoms uncommon

Staphylococcal Scalded Skin Syndrome Source of Staph is NOT found in the bullae Exotoxin enters blood & causes remote bullae Sources to consider Umbilicus Nares Rectum Conjunctiva Bullous impetigo Similar but Staph is at SAME site as bulla

Staph Scalded Skin Treatment Swab possible sites for Staph Cloxacillin (bactericidal for Staph) IV +/- Clindamycin (anti-toxin) IV Pain control Compresses to healing skin Switch to oral when well Dispigmentation after rash-resolves

Rare Causes of Neonatal Vesicles Epidermolysis Bullosa Rare, genetic, lifelong blistering disorder Vesicles and bullae Tense or flaccid Presents in early infancy

Rare Causes of Neonatal Vesicles Epidermolysis Bullosa 3 types: Based on location of abnormal protein Simplex Junctional Herlitz Non-Herlitz Dystrophic Dominant Recessive

Epidermolysis Bullosa: Genetics 10 genes responsible for most EB forms Many different mutations EB-Simplex usually AD EB-Junctional usually AR EB-Dystrophic: AR and AD variants

Incontinentia Pigmenti X-linked dominant Lethal in males Some affected males reported Post-zygotic mosaicism XXY NEMO gene Needed for activation of NF-kB (transcription factor) Affects derivatives of the neuroectoderm Skin, neuro, ocular, dental, bone, hair, nail

Incontinentia Pigmenti Stage 1: erythema, vesicles, pustules Stage 2: verrucous, hyperkeratosis Stage 3: HYPERpigmentation Stage 4: HYPOpigmentation, atrophy

Incontinentia Pigmenti Stage 1 Within first few weeks of life Vesicles on red base Crusts when vesicles open Spare face Respects midline Often linear Clears by 4 months

Stage 2 Incontinentia Pigmenti Other Stages Hyperkeratotic papules as vesicles heal Distal limbs, gone by 6 months Stage 3 Hyperpigmented streaks in Blaschkoid pattern Fades and disappears by end of 2 nd decade Stage 4 Hypopigmented streaks

Non-cutaneous findings Ocular findings (many) Neonatal period: Neovascularization Needs assessment q monthly +/- treatment Dental (almost all) Nail dystrophy Scarring alopecia Sweating abnormality Neurologic

Dermatitic (eczematous) Eruptions in the Neonate

Dermatitis Atopic Dermatitis Seborrheic Dermatitis Irritant Contact Dermatitis Rare LCH Malabsorption Omenn syndrome

Seborrheic dermatitis From 3 weeks of age until 6 months Doesn t predict seb derm when older Yeast (Malassezia furfur) may play role Diaper area, scalp, brows, folds Many use term to describe cradle cap Thick, greasy scale with no inflammation Retention hyperkeratosis or cradle cap Thick, greasy scale and dermatitis Seborrheic Dermatitis

Seborrheic Dermatitis Treatment: Mix corticosteroid and antifungal 1% Hydrocortisone powder in antifungal cream Resolves without recurrences in many Persists in some children

Irritant Contact Dermatitis Very common 3-18 months Inner, upper thigh, buttock, spares creases Sharply marginated erythema, no satellites Can get glazed look and erosions Specific rare subtype on labia: Jacquet s Erosive, punched-out ulcers

Irritant Contact Dermatitis Contributing factors: Irritation from urine and stool Candida Friction from wet diaper rubbing on skin Friction from diaper wipes Irritation from diaper wipes Underlying skin disease favoring folds E.g., seborrheic dermatitis, psoriasis

Irritant Contact Dermatitis How? occlusive diaper causes over hydrated skin Hydrated skin enables skin friction against diaper High ph activates enzymes that damage epidermis Diarrhea exacerbates ICD

Irritant Contact Dermatitis: Treatment Frequent diaper change Gentle cleansing Sitz bath is best No harsh rubbing Do not need to remove all barrier cream Gently and completely dried Barrier creams: Thick zinc oxide 1% hydrocortisone TID if needed Add topical antifungal if secondary candida

Systemic Disease Presenting as Dermatitis

Zinc Deficiency Etiology: Acrodermatitis enteropathica AR Affects intestinal absorption and transport of zinc Impaired zinc excretion in breast milk Clinical: Diarrhea Skin eruption (with alopecia) Irritability Collect blood in special tubes Rapid improvement with zinc supplement

Langerhans Cell Histiocytosis Seborrheic dermatitis-like eruption Affects scalp, groin, axillae, chest Yellow, scale on red base with petechiae Non-pruritic Usually skin biopsy needed to confirm Work-up and follow by oncology

Malabsorption Syndromes Widespread dermatitis Associated signs: FTT Edema Diarrhea Irritability

Omenn Syndrome Part of SCID Dermatitis or exfoliative erythroderma Associations: Infections Eosinophilia Lymphadenopathy and HSM Alopecia Diarrhea FTT

Papulosquamous Eruptions

Neonatal Lupus Discrete, round-oval scaly papules Raccoon pattern Appears up to 6 weeks, resolve ~6 months Maternal antibodies cross placenta Anti-Ro +/- Anti-La Mother often asymptomatic 2% of exposed fetuses

Neonatal Lupus Can have associated heart disease Typically heart block (1 st, 2 nd, 3 rd ) Cardiomyopathy +/-Hepatic or Hematologic disease

Neonatal Lupus Cutaneous NL self-resolves, may leave scar Importance is in recognizing this sign May predict cardiac NL in baby Marker for risk of cardiac NL in future baby

Neonatal Lupus Once baby diagnosed Work-up including heme/liver parameters Mother should have Examination for rheumatologic disease Antibodies checked Followed during subsequent pregnancies Risk of next baby having NL elevated Cutaneous Heart Block Hepatic involvement/hematologic Izmirly et al, Arthritis and Rheumatism, April 2010

Other Neonatal Eruptions

Harlequin Ichthyosis Severe, often fatal for of ichthyosis (AR)?abnormal transport of lipids (ABCA12) Hard, thick plates of skin with fissures Ectropion and eclabium common Impaired thermoregulation & chest expansion Infection common Systemic retinoids may help Shed firm skin but severe ichthyosis persists

Collodion Membrane Baby s skin has shiny covering Membrane peels within 2 weeks Complications similar to Harlequin Possible outcomes: Ichthyosis (several types) Netherton s syndrome Gaucher disease Normal skin

Subcutaneous Fat Necrosis Nodules/plaques in neonatal period Hard, indurated, not warm Usually self-limited Healthy term delivery with obstetrical trauma Meconium aspiration, asphyxia, hypothermia, hypoxemia etc.

Subcutaneous Fat Necrosis Pathophysiology:? cold or stress injures immature fat? causes solidification and necrosis Develops granulmoatous infiltrate Non-renal calcium absorption increases

Subcutaneous Fat Necrosis Hypercalcemia is most concerning association Monitor calcium for several weeks to months Warn parents of signs to watch for: FTT, irritability, lethary,anorexia, vomiting, hypotonia, sz, cardiac arrythimias, rena. fialure Other lab abnormalities rare Often biopsy needed to make a definitive diagnosis Outcome: Normal skin Scar/atrophy Ulceration Soft tissue calcification

Summary Use morphologic clues, history and exam to distinguish benign common eruptions from rare, serious eruptions and those that suggest systemic disease