NEONATAL DISEASES

By: Eufronia Apple G. Puzon, MD

TOPICS
Erythema Toxicum Neonatorum

Miliaria
Cutis Marmorata Hemangioma of Infancy

Urticaria Pigmentosa
Congenital Syphilis

ERYTHEMA TOXICUM NEONATORUM

Idiopathic common condition seen up to 75% of term newborns. Rarely seen in premature infants. Usually begin at 24 to 48 hours of age PE: Blotchy erythematous macules 1 to 3 cm in diameter with a 1 to 4 mm central vesicle or pustule. Trunk is the common site Spare the palms and soles

Fitzpatrick Dermatology 7th Ed. p. 937

Erythematous macules, some with a tiny central papulopustule on the arm of a 1-day-old newborn.

Wright stained smear: Numerous eosinophils

ERYTHEMA TOXICUM NEONATORUM

Differential Diagnosis: - Transient Neonatal Pustular Melanosis Miliaria

Bacterial Folliculitis
Neonatal Herpes Scabies

Benign and clears spontaneously by 2 to 3 weeks of age without residua.

Andrews' Diseases of the Skin 10th Ed. p. 140 Fitzpatrick Dermatology 7th Ed. p. 937

MILIARIA
Retention of sweat as a result of occlusion of eccrine sweat ducts. Common in hot humid climates.
Rupture of gland or duct at different levels

Occlusion from the sweat glands

Escape of sweat

MILIARIA

Andrews' Diseases of the Skin 10th Ed. p.23

FOUR TYPES:

Miliaria Crystallina

Miliaria Rubra

Miliaria Pustulosa

Miliaria Profunda

MILIARIA CRYSTALLINA
Aka Miliaria Sudamina Asymptomatic; Duration short PE:

- superficial, sub-corneal, non-inflammatory vesicles that easily rupture when rubbed with a finger.
Self-limited; No treatment required.

Andrews' Diseases of the Skin 10th Ed. p.23 Fitzpatrick Dermatology 7th Ed. p. 730

MILIARIA RUBRA

Aka Prickly Heat Obstructed sweat migrates into the epidermis and upper dermis causing pruritic inflammatory papules around the sweat pores. Sites: antecubital and popliteal fossae, trunk, inframammary areas, abdomen (waistline) and inguinal regions.

Andrews' Diseases of the Skin 10th Ed. p. 23 Fitzpatrick Dermatology 7th Ed. p. 730

MILIARIA RUBRA
PE: - discrete extremely pruritic, erythematous papulovesicles accompanied by a sensation of prickling, burning or tingling. Eruption subsides after the patient moves to a cool environment.

Andrews' Diseases of the Skin 10th Ed. p.23 Ffitzpatrick Dermatology 7th Ed. p. 730

CUTIS MARMORATA
Physiologic mottling of the skin. Transient and common finding in newborns. Aka marbled skin because of its mottled bluish discoloration. Commonly seen on lower extremities. Disappears when extremities are warmed.

Andrews' Diseases of the Skin 10th Ed. p.818

CUTIS MARMORATA
Caused by instability or immaturity of the nerve supply to the superficial capillary blood vessels in the skin.
This causes the blood vessels in some regions of the skin to dilate.

http://doctorsgates.blogspot.com/2011/03/mottled-skin-or-cutis-marmorata-in.html

HEMANGIOMA OF INFANCY
Aka Strawberry Hemangioma
Most common tumor of infancy. Females > Males 3:1 ratio

Etiology: localized proliferative process of angioblastic mesenchyme (embryonic mesenchymal tissue from which blood cells and blood vessels arise).
Course of Lesion: Initial proliferation phase lasts 3 to 9 months. Enlarge rapidly during 1st year. Regresses gradually over 2 to 6 years and is usually complete by the age of 10.

Fitzpatrick's Color Atlas & Synopsis of Clinical Dermatology 5th Ed. p. 180

HEMANGIOMA OF INFANCY
PE: - Soft, bright red to deep purple On diascopy, does not blanch completely With onset of spontaneous, white-to-gray area appears on the surface of the central part of the lesion Usually solitary and locaized or extend over an entire region. Head and neck 50%, Trunk 25%

Distribution: -

Fitzpatrick's Color Atlas & Synopsis of Clinical Dermatology 5th Ed. p. 180

HEMANGIOMA OF INFANCY
SPECIAL PRESENTATIONS 1. Deep Hemangioma - aka Cavernous Hemangioma - localized, firm rubbery mass of bluish color with telangiectases in overlying skin. - lower dermis and subcutaneous fat.

Fitzpatrick's Color Atlas & Synopsis of Clinical Dermatology 5th Ed. p. 180

HEMANGIOMA OF INFANCY
2. Multiple HIs - multiple small (<2cm), cherry red papular lesions involving skin alone ( benign cutaneous hemangiomatosis) or skin and internal organs (diffuse neonatal hemangiomatosis).

HEMANGIOMA OF INFANCY
3. Congenital Hemangiomas
- develop in utero Subdivided: Rapidly involuting congenital hemangiomas (RICH)

Noninvoluting congenital hemangiomas (NICH)

- violaceous tumors with overlying telangiectasia with large veins in periphery or as red-violaceous plaques invading deeper tissues. - NICH fast flow hemangioma requiring surgery

HEMANGIOMA OF INFANCY
Dermatopathology: Proliferation of endothelial cells in various amounts in the dermis and/or subcutaneous tissue. Diagnosis: Clinical findings and MRI Doppler and arteriography fast flow Course and Prognosis: No residual skin change at the site in most lesions (80%). Residual atrophy, depigmentation, telangiectasia and scarring (20%). Deeper lesions may not involute completely (mucous membranes)

Fitzpatrick's Color Atlas & Synopsis of Clinical Dermatology 5th Ed. p. 181

HEMANGIOMA OF INFANCY
Management: - Continuous wave or pulsed dye laser - Cryosurgery - Intralesional and systemic high dose glucocorticoids - INF- Majority of HIs active nonintervention is the best approach because spontaneous resolution gives the best cosmetic results.

URTICARIA PIGMENTOSA
Generalized Eruption, Childhood Type Form of cutaneous mastocytosis Begins during the first weeks of life PE: Rose-colored, pruritic, urticarial, slightly pigmented macules, papules or nodules.

Oval or round and vary in diameter between 5 and 15mm and may coalesce
Varies from yellowish-brown to yellowish-red Occ. lesions are a pale yellow color (Xanthelasmoidea)

Andrews' Diseases of the Skin 10th Ed. p.616

URTICARIA PIGMENTOSA
PE: Vesicle and Bulla formation prominent feature early in the disease Not evanescent Lesions persist and gradually become chamois or slatecolored

Andrews' Diseases of the Skin 10th Ed. p.616

URTICARIA PIGMENTOSA
Dariers Sign - Firmly stroked or vigorously rubbed, urticaria with a surrounding erythematous flare usually develops

* Commonly disappear within a few years, generally before puberty.

CONGENITAL SYPHILIS
Acquired in utero Infection through the placenta usually does not occur before the 4th month.

Pathogenesis:
- Lesions usually develop after 4th month of gestation. - Associated with fetal immunologic competence (depends on immune response of fetus) Adequate treatment before 16th week prevents fetal damage Untreated fetal loss up to 40%

Andrews' Diseases of the Skin 10th Ed. p.360 Fitzpatricks Color Atlas & Synopsis of Clinical Dermatology 5th Ed. p. 924

CONGENITAL SYPHILIS
CLINICAL MANIFESTATIONS: Early Congenital Syphilis within first 2 years of life Late congenital Syphilis after the age of 2 years

EARLY CONGENITAL SYPHILIS

Presents within first 2 years of life

Cutaneous manifestations 3rd week of life

Premature, often marasmic, fretful, dehydrated Face is pinched and drawn (old man or woman) Multisystem disease characteristic

Snuffles form of rhinitis, first specific finding

Andrews' Diseases of the Skin 10th Ed. p.361

EARLY CONGENITAL SYPHILIS

Cutaneous Lesions: Usually morbilliform and rarely purely papular. Bright or violaceous red later fading to coppery color.

Papules may become large and infiltrated; frequently scaling is pronounced.

There is secondary pustule formation with crusting. Predilection to face, arms, buttocks, legs, palms and soles.

EARLY CONGENITAL SYPHILIS

Syphilitic Pemphigus Bullous eruption usually on the palms and soles Uncommon lesion Present at birth or appear in the first week of life Bullae become purulent and rupture weeping erosions Desquamation is common often preceded by edema and erythema

EARLY CONGENITAL SYPHILIS

Condylomata Lata
Large, moist, hypertrophic papules found about the anus and in the other folds of the body. More common around the first year of life than in the newborn period.

EARLY CONGENITAL SYPHILIS

Bone lesions occur in 70% to 80% Epiphysitis Common

Causes pain on motion, leading to the infants refusing to move (Parrot pseudoparalysis)
Bone lesions occur at the epiphyseal ends of the long bones.

CNS involvement 86%; meningeal or meningovascular in origin (3rd to 6th month of life) Hepatomegaly, splenomegaly, enlargement of lymph nodes.

LATE CONGENITAL SYPHILIS

Inflammatory Late Congenital Syphilis lesions of the cornea, bones, and CNS are the most important. intense pericorneal inflammation and persists to diffuse clouding of the cornea w/o surface ulceration (20% to 50%) Affects the knees leads to symmetrical, painless swelling.

Interstitial Keratitis
-

Perisynovitis (Clutton joints) -

Seizures frequent symptom

LATE CONGENITAL SYPHILIS

Malformations (Stigmata) Hutchinsons teeth Malformation of the central upper incisors. Hyperplastic tooth Flattening of the nasal bones Unilateral thickening of the inner 3rd of one clavicle. Appears on the right side in right-handed persons and on the left side in left-handed persons.

Mulberry molar
Nasal Chondritis

Higoumenakis Sign

Andrews' Diseases of the Skin 10th Ed. p.362

CONGENITAL SYPHILIS
Diagnosis: Infants of women who meet the following criteria should be evaluated for congenital syphilis: 1. Maternal untreated syphilis, inadequate treatment, or no documentation of adequate treatment. 2. Treatment of maternal syphilis with erythromycin. 3. Treatment less than 1 month before delivery. 4. Inadequate maternal response to treatment. 5. Appropriate treatment before pregnancy, but insufficient serologic follow-up to document adequacy of therapy.

Andrews' Diseases of the Skin 10th Ed. p.362

CONGENITAL SYPHILIS

Management:
Therapy should be undertaken in consultation with a pediatric infectious disease specialist.

CDC TREATMENT GUIDELINES 2010

Infants with proven or highly probable disease and 1.an abnormal physical examination that is consistent with congenital syphilis; 2. a serum quantitative nontreponemal serologic titer that is fourfold higher than the mothers titer; or 3.a positive darkfield test of body fluid(s).

Recommended Evaluation:
1. CSF analysis for VDRL, cell count, and protein 2. Complete blood count (CBC) and differential and platelet count 3. Other tests as clinically indicated (e.g., long-bone radiographs, chest radiograph, liver-function tests, cranial ultrasound, ophthalmologic examination, and auditory brain stem response)

CDC TREATMENT GUIDELINES 2010

Recommended Regimens: Aqueous crystalline penicillin G 100,000150,000 units/kg/day, administered as 50,000 units/kg/dose IV every 12 hours during the first 7 days of life and every 8 hours thereafter for a total of 10 days

OR
Procaine penicillin G 50,000 units/kg/dose IM in a single daily dose for 10 days

CDC TREATMENT GUIDELINES 2010

Infants who have a normal physical examination and a serum quantitive nontreponemal serologic titer the same or less than fourfold the maternal titer and the 1. mother was not treated, inadequately treated, or has no documentation of having received treatment; 2. mother was treated with erythromycin or another nonpenicillin regimen;or 3. mother received treatment <4 weeks before delivery. Recommended Evaluation 1. CSF analysis for VDRL, cell count, and protein 2. CBC, differential, and platelet count 3. Long-bone radiographs

CDC TREATMENT GUIDELINES 2010

Recommended Regimens: Aqueous crystalline penicillin G 100,000150,000 units/kg/day, administered as 50,000 units/kg/dose IV every 12 hours during the first 7 days of life and every 8 hours thereafter for a total of 10 days

OR
Procaine penicillin G 50,000 units/kg/dose IM in a single daily dose for 10 days OR Benzathine penicillin G 50,000 units/kg/dose IM in a single dose

CDC TREATMENT GUIDELINES 2010

Infants who have a normal physical examination and a serum quantitative nontreponemal serologic titer the same or less than fourfold the maternal titer and the

1. mother was treated during pregnancy, treatment was appropriate for the stage of infection, and treatment was administered >4 weeks before delivery and 2. mother has no evidence of reinfection or relapse.

Recommended Evaluation No evaluation is required.

CDC TREATMENT GUIDELINES 2010

Recommended Regimen: Benzathine penicillin G 50,000 units/kg/dose IM in a single dose

* Another approach involves not treating the infant, but rather providing close serologic follow-up in those whose mothers nontreponemal titers decreased fourfold after appropriate therapy for early syphilis or remained stable or low for late syphilis.

CDC TREATMENT GUIDELINES 2010

Infants who have a normal physical examination and a serum quantitative nontreponemal serologic titer the same or less than fourfold the maternal titer and the 1. mothers treatment was adequate before pregnancy and 2. mothers nontreponemal serologic titer remained low and stable before and during pregnancy and at delivery (VDRL <1:2; RPR <1:4). Recommended Evaluation No evaluation is required. Recommended Regimen: No treatment is required; however, benzathine penicillin G 50,000 units/kg as a single IM injection might be considered, particularly if follow-up is uncertain.