bulla and erosions in neonates

Bullae and erosions in neonates : 

Bullae and erosions in neonates By: Dr. Deepak Kumar

Neonatal skin : 

Neonatal skin

More common disorders : 

More common disorders Miliaria crystallina Bullous impetigo Thermal or chemical burns Epidermolysis bullosa Incontinentia pigmenti Bullous ichthyosiform erythroderma Mastocytosis

Rare disorders : 

Rare disorders Neonatal herpes simplex Fetal varicella syndrome (FVS) Herpes zoster Congenital syphilis Passively transferred Pemphigus vulgaris Herpes gestationis Bullous pemphigoid Extensive congenital erosions and vesicles healing with reticulate scarring

Rare disorders : 

Rare disorders Congenital erythropoietic porphyria AEC syndrome Sucking blisters Congenital absence of skin Porphyrias and transient porphyrinaemia Langerhans’ cell histiocytosis

Miliaria crystallina : 

Miliaria crystallina Three forms of miliaria Miliaria crystallina Miliaria rubra (prickly heat) Miliaria profunda Due to Obliteration or Disruption of the eccrine sweat duct Differ in clinical form Due to the different levels of obliteration

Miliaria crystallina : 

Miliaria crystallina Obstruction is within stratum corneum Vesicle is subcorneal Often seen in febrile illnesses Associated with profuse sweating Common in infants Due to delay in patency in sweat ducts Presents as Crops of clear, thin-walled, superficial vesicles 1–2 mm in diameter, Without associated erythema

Miliaria crystallina : 

Miliaria crystallina Treatment The only really effective treatment for miliaria Avoidance of further sweating Oral ascorbic acid 500 mg b.d Diminish the severity of miliaria Calamine lotion Bland emollient

Miliaria crystallina : 

Miliaria crystallina

Bullous impetigo : 

Bullous impetigo Caused by Phage group II strains of S. Aureus Present as Bullae with Thin, delicate walls and narrow, red areola Contain clear fluid Sites Perineum Periumbilical area Neck creases

Bullous impetigo : 

Bullous impetigo Untreated generalized bullous impetigo Associated with Significant mortality Complications Lung abscess Staphylococcal pneumonia Osteomyelitis

Bullous impetigo : 

Bullous impetigo Treatment Topical Mupirocin Fusidic acid Neomycin + Bacitracin Oral antibiotic Flucloxacillin Erythromycin Tetracycline

Thermal burns : 

Thermal burns Burn An injury caused by Pathological flux of energy within tissue Resulting in disruption of functional integrity Source of energy Thermal Chemical Electrical Radiation

Thermal burns : 

Thermal burns

Thermal burns : 

Thermal burns A radiant warmer is a bed that can help keep the babies warm when they cannot control their own body temperature.

Thermal burns : 

Thermal burns

Thermal burns : 

Thermal burns

Thermal burns : 

Thermal burns Superficial partial thickness Deep partial thickness

Thermal burns : 

Thermal burns Full thickness

Thermal burns : 

Thermal burns Area of burn Lund-browder chart

Thermal burns : 

Thermal burns First aid and prehospital management Stop the burning process Primary survey Airway Breathing Circulation Cervical spine immobilization Secondary survey Wrap in clean dressings No topical antimicrobial

Thermal burns : 

Thermal burns Assessment in the hospital Depth of burn Area of burn Fluid resuscitation Children: 3–4 mL Hartmann’s solution/kg body weight/% BSA burn Half volume in first 8 h post-burn Remaining half over the subsequent 16 h of the first day Maintenance requirements for the first 24 h 1000 mL for the first year of life, Plus 100 mL for each subsequent year of life up to 5 years

Thermal burns : 

Thermal burns Urine output of More than 0.5 ml/kg/h in an adult 1.0 ml/kg/h in a child Escharotomy Circumferential deep dermal or full thickness burn Limbs Chest Neck Skin will lose its normal flexibility

Thermal burns : 

Thermal burns

Thermal burnsmanagement : 

Thermal burnsmanagement Small and superficial Deroofing blisters Cleansing with saline Non-adherent dressing Deep partial thickness burn Full thickness burns Surgery Debridement Graft

Epidermolysis bullosa : 

Epidermolysis bullosa A group of genetically determined Skin fragility disorders Characterized by blistering Skin Mucosae Following mild mechanical trauma

Epidermolysis bullosasimplex : 

Epidermolysis bullosasimplex

Epidermolysis bullosasimplex : 

Epidermolysis bullosasimplex

Epidermolysis bullosasimplex : 

Epidermolysis bullosasimplex Weber cockayny

Epidermolysis bullosa simplex : 

Epidermolysis bullosa simplex Dowling meara

Epidermolysis bullosa.. junctional : 

Epidermolysis bullosa.. junctional

Epidermolysis bullosajunctional : 

Epidermolysis bullosajunctional

Epidermolysis bullosajunctional : 

Epidermolysis bullosajunctional Herlitz

Epidermolysis bullosajunctional : 

Epidermolysis bullosajunctional Non-herlitz

Epidermolysis bullosadystrophic : 

Epidermolysis bullosadystrophic

Epidermolysis bullosadystrophic generalized : 

Epidermolysis bullosadystrophic generalized

Epidermolysis bullosadystrophic : 

Epidermolysis bullosadystrophic

Epidermolysis bullosadystrophic : 

Epidermolysis bullosadystrophic Recessive generalized DEB

Epidermolysis bullosadystrophic : 

Epidermolysis bullosadystrophic Recessive localized DEB

Epidermolysis bullosaTreatment : 

Epidermolysis bullosaTreatment No specific treatment for any form of EB Mainstay of management Protection and avoidance Of provoking factors

Epidermolysis bullosaTreatment : 

Epidermolysis bullosaTreatment Management of neonates and infants In specialist neonatal or paediatric unit Expert nursing care Nursed on thick foam pads Covered by a silk sheet Erosions cleaned with normal saline Non adherent dressings Topical antibiotic

Epidermolysis bullosaTreatment : 

Epidermolysis bullosaTreatment Severe generalized recessive DEB Special precautions Adhesive tapes Sphygmomanometer cuffs Tourniquets Systemic treatment Corticosteroid Phenytoin Vitamin E Minocycline Ciclosporin Retinoic acid

Epidermolysis bullosaTreatment : 

Epidermolysis bullosaTreatment Severe generalized recessive DEB Skin grafting Pain management Anaemia

Epidermolysis bullosaTreatment : 

Epidermolysis bullosaTreatment Epidermolysis bullosa simplex Reduce Heat Humidity Well fitting well-ventilated shoes Cotton socks 20% aluminium chloride hexahydrate

Incontinentia pigmenti : 

Incontinentia pigmenti Hereditary syndrome Mutation in chromosome Xq28 Cutaneous lesions Vesicular Verrucous Pigmented Associated with developmental defects of Eye Skeletal system Central nervous system 95% of cases are females Lethal in males

Incontinentia pigmenti : 

Incontinentia pigmenti Pathology Bullae Beneath the horny layer Within a spongiotic epidermis Dermis Non-specific inflammatory changes With a cellular infiltrate Eosinophils Lichenoid papules Hyperkeratosis Acanthosis Oedema of the basal layer Upper dermis Macrophages laden with melanin

Incontinentia pigmenti : 

Incontinentia pigmenti Skin changes Often present at birth Usually develop before end of first week Three clinical stages Bullae Papular and warty lesions Pigmentation

Incontinentia pigmenti : 

Incontinentia pigmenti Clear tense bullae Often in linear groups On the limbs in recurrent crops Followed by Smooth red nodules or plaques Often irregularly linear On the limbs and trunk Bluish-purple in colour May ulcerate Linear warty lesions Dorsa of hands and feet

Incontinentia pigmenti : 

Incontinentia pigmenti Pigmentation Blue-grey or slate to brown (characteristic) Bizarre ‘splashed’ or ‘chinese figure’ distribution (diagnostic)

Incontinentia pigmenti : 

Incontinentia pigmenti 25% of cases Patches of cicatricial alopecia At or near the vertex Present from birth or develop in infancy Acute inflammatory skin changes Peripheral eosinophilia Uncommon after the age of 6 months

Incontinentia pigmenti : 

Incontinentia pigmenti Dental defects Delayed dentition Partial anodontia Cone- or peg-shaped teeth Ocular defects(30% cases) Blindness Strabismus Cataract Uveitis Optic atrophy Retinal vascular abnormalities

Incontinentia pigmenti : 

Incontinentia pigmenti CNS disorders (25% cases) Mental retardation Slow motor development Spastic tetraplegia and diplegia Microcephaly Epilepsy Diagnosis Combination of Bullae with Linear nodular or warty lesions In a female infant is pathognomonic No treatment Control secondary infection

Bullous ichthyosiform erythroderma : 

Bullous ichthyosiform erythroderma Rare autosomal dominant disorder Of keratinization In early phase Associated with blistering Incidence Less than 1 in 100,000 Genetic defect In K1 or K10

Bullous ichthyosiform erythroderma : 

Bullous ichthyosiform erythroderma Histopathology Epidermal Acanthosis and hyperkeratosis Granular layer, and in spinous layers Multiple perinuclear vacuoles Large clumped keratohyaline granules Split with oedema in granular layer Basal keratinoyctes are normal and Dermis Mild upper dermal lymphocytic infiltrate Also known as Epidermolytic hyperkeratosis

Bullous ichthyosiform erythroderma : 

Bullous ichthyosiform erythroderma Presents with Epidermolysis (fragile skin) Gradual evolution of hyperkeratosis Mild generalized erythroderma At birth Flaccid blisters Peeling and superficial erosions At sites of minor trauma or friction In the first few hours of life

Bullous ichthyosiform erythroderma : 

Bullous ichthyosiform erythroderma Superficial erosions Heal rapidly without scarring Easy blistering ceases In the first few months Blistering at sites of trauma Nappy area, thighs, knees) Continues through childhood Hyperkeratosis Early childhood Around the anterior neck Flexures, abdominal wall Infragluteal folds and scalp

Bullous ichthyosiform erythroderma : 

Bullous ichthyosiform erythroderma Yellow–brown, waxy Ridged or corrugated scale In skin creases

Bullous ichthyosiform erythroderma : 

Bullous ichthyosiform erythroderma Keratolytic preparations Salicylic acid Reduce hyperkeratosis Topical calcipotriol Reduction in scaling Retinoid therapy Acitretin

Mastocytosis : 

Mastocytosis A rare condition characterized by Too many mast cells That are functionally normal Usually presents in skin But may affect Bone marrow Gastrointestinal tract

Mastocytosis : 

Mastocytosis Clinical classification of mastocytosis I Indolent mastocytosis A Cutaneous Urticaria pigmentosa Mastocytoma Telangiectasia macularis eruptiva perstans (TMEP) Diffuse (erythrodermic) cutaneous mastocytosis B Systemic (extracutaneous mast cells in at least one organ) II Mastocytosis with an associated haematological disorder A Myeloproliferative disorders B Myelodysplastic disorders III Aggressive mastocytosis with lymphadenopathy and eosinophilia IV Mast cell leukaemia

Mastocytosis : 

Mastocytosis In neonates Solitary mastocytoma Diffuse cutaneous mastocytosis

Mastocytosismastocytoma : 

Mastocytosismastocytoma Red or pink Nodules or plaques 3–4 cm in diameter Usually solitary Blister on friction Involute over the first few years

Diffuse cutaneous mastocytosis : 

Diffuse cutaneous mastocytosis Mast cells Infiltrate entire skin diffusely Skin thickened Doughy consistency Blistering common Pruritus intense Pigmentation absent High risk of systemic disease May resolve spontaneously

Neonatal herpes simplex : 

Neonatal herpes simplex Cause:HSV 1 & 2 Contact with infected genital tract During delivery Lesions on skin Occurs on day 2 & 20 Most commonly affected Scalp & face Oral erosions HSV 2 infection is fatal Early recognition & Rx

Fetal varicella syndrome : 

Fetal varicella syndrome A rare disorder Adults have antibody to varicella If mother had chickenpox (no antibody) Between 7th and 20th wk pregnancy Spontaneous abortion Child born with FVS

Fetal varicella syndrome : 

Fetal varicella syndrome Principal clinical features of the fetal varicella syndrome. Low birth weight Cutaneous lesions Localized absence of skin (on limb) Scars (dermatomal outline) Papular lesions Hypoplasia of one or more limbs And/or malformed digits Ocular anomalies Chorioretinitis, cataracts, Microphthalmia, Horner’s syndrome CNS abnormalities Seizures, mental retardation Hydrocephalus, cortical atrophy Encephalitis, encephalomyelitis

Congenital syphilis : 

Congenital syphilis Skin is normal at birth Initial lesion:2nd & 8th week Reddish brown macule or papule (3% cases) bullous lesions Site anogenital area face palms and soles Paronychia Low birth weight

Transplacental transfer of maternal autoantibodies : 

Transplacental transfer of maternal autoantibodies Restricted to IgG antibodies 3–6 months of life Neonatal pemphigus vulgaris Cutaneous and/or mucosal Erosions or bullae Circulating IgG antibodies IMF positive in skin Resolved spontaneously Within 3 weeks

Transplacental transfer of maternal autoantibodies : 

Transplacental transfer of maternal autoantibodies Transplacental pemphigoid (herpes) gestationis 10% of infants affected Present at birth or Up to the third day of life Spontaneous regression Within 3 weeks is the rule Direct immunofluorescence Normal by end of first month

Extensive congenital erosions andvesicles healing with reticulate scarring : 

Extensive congenital erosions andvesicles healing with reticulate scarring Rare disorder of unknown aetiology Affected infants born premature At birth Extensive superficial erosions Scattered vesicles and bullae Affecting 75% of body surface Heal in first few weeks Leaving soft, reticulated scarring Loss of sweating in scarred areas Patchy alopecia Absence of nails

Extensive congenital erosions andvesicles healing with reticulate scarring : 

Extensive congenital erosions andvesicles healing with reticulate scarring (a) Extensive vesicles and erosions were noted on the skin of this child in the delivery room. (b) Erosions and crusts healed with widespread reticulated, supple scarring.

Bullous eruptions resulting fromtransient porphyrinaemia : 

Bullous eruptions resulting fromtransient porphyrinaemia Bullous photosensitivity reactions In neonates with Congenital erythropoietic porphyria Hepatoerythropoietic porphyria Erythropoietic protoporphyria Harderoporphyria Transient porphyrinaemia Without error in iron metabolism Prematurity Cholestasis Disturbed hepatocellular function Renal failure

Algorithm for evaluation of neonatal rashes : 

Algorithm for evaluation of neonatal rashes

Thank you : 

Thank you

Slide 76: 

Fig 2.56 Aplasia cutis congenita. (a) An extensive area of aplasia cutis was noted on the scalp of this newborn. (b) After 5 months the scalp was almost completely healed.When the child was 5 years old, the residual scar was repaired following tissue expansion.

Slide 77: 

Fig. 36.9 Neonatal herpes simplex virus infection. Note the clustering of the vesicles on an erythematous base.

Epidermolysis bullosa : 

Epidermolysis bullosa

Epidermolysis bullosa : 

Epidermolysis bullosa Fig 2.52 Dystrophic epidermolysis bullosa. (a) Blisters, erosions, and hundreds of milia are seen on the foot and ankle and (b) hand of this newborn. (c) In this child, severe scarring encased the fingers, resulting in syndactyly. (d) Recurrent blistering resulted in scarring and permanent nail loss in this adolescent.

Epidermolysis bullosa : 

Epidermolysis bullosa

Incontinentia pigmenti : 

Incontinentia pigmenti

Incontinentia pigmenti : 

Incontinentia pigmenti Fig 2.62 Incontinentia pigmenti. Vesicles on an erythematous base extended in a linear pattern down the (a) legs and (b) trunk of an 8-day-old girl. (c) Warty papules developed on the fingers of this 4-month-old girl in the site of earlier vesicles. (d) Progressive ‘marble cake’ hyperpigmentation was first noted on the trunk of this infant at 4 months of age. (e) Subtle scarring on the legs was the only sign of incontinenta pigmenti in the mother of the child. Note the absence of hair follicles in the linear scars. (f) This 60-year-old woman with incontinentia pigmenti has had recurrent warty subungual nodules and nail dystrophy since adolescence. A biopsy showed eosinophilic dermal inflammation and dyskeratosis

Mastocytosis : 

Mastocytosis

Mastocytosis : 

Mastocytosis Fig 2.61 Mastocytosis. (a) Brown macules and plaques typical of urticaria pigmentosa were scattered on the trunk of this infant. A Darier sign became evident in a single lesion on the back after rubbing. (b) Hyperpigmentation is prominent in this healthy black infant with urticaria pigmentosa. Note the blisters on her forehead. (c) Vesicles and crusted papules on a red urticarial base were present at birth in this asymptomatic newborn. After the crusts healed, no blisters recurred. (d) This infant with diffuse infiltrative lesions had widespread blistering and bleeding into lesions.

Epidermolysis bullosajunctional : 

Epidermolysis bullosajunctional Fig 2.51 Junctional epidermolysis bullosa.Widespread involvement was seen in this infant at birth. (a) Note the erosions and the large, intact blister over the thumb and dorsum of the hand. (b) Large, denuded areas occur over the back and buttocks.