Pediatric DermatologyPediatric Dermatology

העור גם מהווה •זירה למחלות עור

ראשוניות

העור מבטא מחלות •סיסטמיות

שכבת הקרן

אפידרמיס

דרמיס

זקיק השערה

בלוטת חלב בלוטת זיעה

העור

העור הוא האיבר הגדול בגוף• ממשקל הגוף במבוגר15-20%משקלו כ-–

הומיאוסטזיס• "מעטפת" פיברואלסטית המגינה על אברי הגוף–

מישוש•גירויים מהחוץ מועברים למערכת העצבים –

המרכזית

תרמוסטט• בלוטות הזיעה ומחזור הדם ההיקפי –

Dייצור של ויטמין •

בדיקת העור

העור!כליש לבדוק את •

הבדיקה כוללת :•ריריות–ציפורניים–שיער וקרקפת–

תאורה טובה•

תאור העור

הפריחה היא מקולו-פפולרית•

נמצא פצע בעור •

לחולה יש פריחה...•

הדרך לאבחנה היא תאור נכון של העור•

מורפולוגיה

צורת הנגעים•

מורפולוגיה

נגעים משנייםמופיעים בעקבות •

הנגע הראשוניסיבוך –נסיגה–

או מסיבה חוץ עורית•

טראומה–גרד–

נגעים ראשונייםמתפתחים באופן •

ספונטני כביטוי לתהליך פתולוגי

בעור

נגעים ראשוניים

macule patchכתם • רובד papuleפפולה •

papuletumor גידול noduleקשרית •nodusקשר •bulla בועה vesicleשלפוחית •pustuleמוגלתית •urtica, whealאורטיקה •

Maculeכתם

כתם )שטוח( על פני •העור או הרירית גודלו

סמ'. אין שינוי 1קטן מ-במרקם העור למעט הצבע.יכולים להופיע

כתמים בעלי צורות וצבעים שונים

Patch

1כתם )שטוח( גדול מ-•סמ' על פני העור או

הרירית. אין שינוי במרקם העור למעט

הצבע.

Papule

התרוממות מוצקה של • על פני האפידרמיס

העור או הרירית גודלו סמ'. יכולות 1קטן מ-

להופיע פפולות בעלות צורות צבעים ומרקמים

שונים

Plaqueרובד

פפולות מתמזגות •ליצירת רבדים, גודלם

יכול להגיע לעשרות סמ'. יכולים להופיע רבדים בעלי צורות

צבעים ומרקמים שונים

Noduleקשרית

נגע מורם ומוצק הנובע •. בדרמיסמהמצאות תסנין

התסנין יכול להיות דלקתי או נאופלסטי. לעיתים

הקשרית אינה מורמת אלא עמוקה וניתן להבחין בה

על ידי מישוש העור. לרוב הקשרית נוקשה יותר

מהעור הבריא

Nodusקשר

נגע הנוצר מהמצאות • בתת העורתסנין

)היפו-דרמיס(. התסנין יכול להיות דלקתי או

נאופלסטי. לעיתים הקשר אינו מורם אלא

עמוק וניתן להבחין בו על ידי מישוש העור.

Vesicleשלפוחית

נגע אפידרמלי מורם •המכיל נוזל שקוף, סרוטי,

המורגי ועוד. קוטרו סמ' . שלפוחית 1המרבי

רופפת מעידה על מיקום אינטר-אפידרמלי.

שלפוחית מוצקה היא לרוב סב-אפידרמלית

Bullaבועה

שלפוחית שקוטרה גדול • סמ'1מ-

Pustuleמוגלתית

נגע אפידרמלי מורם • מראשיתוהמכיל בתוכו

נוזל מוגלתי. קוטרו ממ' . נוזל 5המרבי

הפוסטולה יכול להיות מזוהם או סטרילי.

Urtica, Whealאורטיקה

בצקת בעור, בגוון לבן •עד ורוד בהיר. הגודל

והצורה משתנים. היא מתפתחת בתוך דקות,

לרוב מלווה גרד עז ונעלמת כעבור שעות

שעות( 24)לא יותר מ-מבלי להותיר סימן

נגעים משניים

scaleקשקש •erosionגלע •ulcerכיב •crustגלד •scarצלקת •

Scaleקשקש

חומר קרן המופיע על •העור כשכבה

לבנה/אפורה דקה ונוקשה, יבשה או

שמנונית. מהווה ביטוי לנשירה

רבה של תאי קרן. מעיד על הפרעה

בהתקרנות העור.

Erosionגלע

חסר בשלמות • של העור האפידרמיס

או הרירית. מתפתח לאחר טראומה

או לאחר בקיעה של שלפוחית או מוגלתית.

נרפא להותיר צלקת מבלי

Ulcerכיב

האפידרמיסחסר בשלמות •. והדרמיס

הכיבים מסווגים על פי עומקם, צורתם והרקמה

בבסיסם. נוצרים בעקבות טראומה או

כתוצאה מפריצת תסנין עמוק החוצה.

נותרת לאחר ריפוי הכיב צלקת

Crustגלד

שכבה נוקשה כקליפה •הנוצרת מקרישה של

הפרשה. גלדים מכסים גלעים וכיבים.

צבע הגלד תלוי בסוג ההפרשה

צהבהב - סרום– ירקרק - מוגלה/צהוב–חם - דם–

Scarצלקת

רקמת חיבור חדשה •במקום שהיה בו חסר של

רקמה בדרמיס או בהיפודרמיס. צלקות

יכולות להיות:שקועות )אטרופיות(

הפרטרופיות )מעל שטח העור(

קלואיד צלקת המתפשטת מעבר למקום הפגיעה

הראשונית

Configurations

גבול/ צורה של נגע בודד•

או

צורה שיוצרים מספר נגעים•

Distributionפיזור

מפושט •Widespread

Symetricסימטרי •Truncalגו •Extremitiesגפיים •Flexuralצד מכופף •Extensorצד מיישר ••Photodistributed•Intertriginous•Scattered

Haphazard

localizedממוקם ••Acral Palms and/orכפות •

Solesמרפקים ובירכיים •

Elbows and/or KneesDermatomalדרמטומלי •Lymphangiticלימפטי •

Distribution

• Flexural

Distribution

• Extensor

Distribution

• Seborrheic

Distribution

• Intertriginous

Distribution

• Photodistributed

Distribution

• Palms and /or Soles

Distribution

• Dermatomal

Distribution

• Lymphangitic

Pediatric DermatologyPediatric Dermatology

Neonatal Birth Marks

• Represent an excess of one or more of the normal components of skin

• Collections of highly differentiated cells:Blood vesselsLymph vessels Pigment cellsHair folliclesSebaceous glands Epidermis, collagen, or elastin

Dermal Melanosis (Mongolian Spots)

• Most common pigment abnormality of infants

• Present at birth or early infancy

• Blue-black or gray macules; solitary or multiple

• Millimeters to several centimeters in size

Dermal Melanosis (Mongolian Spots)

• Distribution: Usually: lower back, sacrum, buttocks; can occur elsewhere

• Consist of spindle-shaped pigment cells (melanocytes) located deep within the dermis

• Most lesions fade spontaneously by 4 years old

Café au Lait Spots

• Round, uniformly light-brown (dark brown on dark skin) macules

• From few mm to 20cm diameter

• Distribution:may occur anywhere; buttocks most common in

newborns

• Most are present at birth or develop in the first few months of life they may increase in number and size with age

Café au Lait Spots

• Increased epidermal melanin in melanocytes and keratinocytes, no melanocytic proliferation

• Rarely have medical importance unless:Number >6 Diameter>5 mm

• Associated syndromes: Neurofibromatosis (NF1, most common)

Congenital Melanocytic Nevi

• Macular, papular, or plaque pigmented lesions with wide range of brown +/- irregular black or blue spots

• Proliferative nests of melanocytes

• Present at birth or develop <1yo– Grow in proportion of child growth

• Size:– Most nevi are small: measuring <1.5 cm – Large lesions are >10 or 20 cm at their greatest

diameter

Congenital Melanocytic Nevi

• Distribution:Anywhere

• Large CMN may be associated:

Limb hypoplasia MyelomeningoceleTumors etc.

Congenital Melanocytic Nevi

• Small risk of melanoma in patients with CMN<1.5cm

• Large CMN lifetime risk for melanoma up to 8%most occurring before puberty

• Prophylactic removal of large congenital nevi within the first year of life recommended

Vascular Birth Marks

• Vascular malformations:Salmon patchPort-wine stains

• Vascular proliferation:

Hemangioma

Flat Capillary Malformations

• Salmon patch nevus flammeus light red or pink in color

• Port-wine stain deep red or purple-red

Salmon Patch (Angel Kiss, Stork Bite)

• Salmon patch:present in up to 50% neonates Stork Bite when on neckAngel Kiss when on forehead

• Present at birth, usually grow in proportion with child

• May resolve <6monthsStork Bite are more likely to persist

• Vascular malformations other than Salmon Patches do NOT usually resolve

Port-Wine Stain

• Present at birth, grows in proportion with child

• Pink or red patches that may have increased skin color, thickness or nodularity with age

• Distribution: Face or extremities, can occur anywhere, usually

unilateral

• Persists for life

Port-Wine Stain

• Sturge-Weber Syndromeoccur in up to 20% of infants with PWS over V1onset <2yo seizures, mental retardation, glaucoma, and

hemiplegia

• Klippel-Trenaunay-Weber syndrome PWS over an extremity: soft tissue or bony

hypertrophy

• TreatmentPulsed dye laser, surgical correction of tissue

hypertrophy

Hemangioma

• Superficial (Strawberry Hemangioma): The most common type Dark red or scarlet, sharply circumscribed

plaque or nodule, partially blanch, rubbery texture, without thrill or bruit

• Deep Hemangioma: Skin color or blue-purple and poorly

circumscribed, may have telangiectasia, size and color may change with position or activity; without thrill or bruit

Hemangioma

• May present at birth, most common in first weeks of life

• Often preceded by blanched macule, or discolored lesion

• Head and neck are most common, but can occur anywhere

• Sacral and lumbosacral hemangiomas can be associated: – spinal dysraphism– genitourinary, neurologic, skeletal abnormalities

• ~ 25% have multiple hemangiomas

Hemangioma

• 3 stages for all hemangiomas: Proliferative: maximum size at 1yStabilizationSpontaneous involution:

• 30% by 3y; 50% by 5y; 70% by 7y; and 90% by 9y 2

• Lip and nose lesions less likely to involute completely

• After involution often some cutaneous findings remain: Hypopigmentation, telangiectasia, scars, redundant

tissue

Hemangioma – Treat if…

• Functional impairmentvision impairment, airway obstructions, or

perirectal involvement (may require colostomy) • Hemangiomas cause permanent disfigurement

nose, lip, glabella, ear, large facial hemangiomas, spontaneous involution often results in better cosmetic result than surgical or other intervention

• Ulceration, bleeding

• Urgent treatment:systemic and intra-lesional steroids or IFN alpha

Epidermal Nevus

• Increases in mature epidermal cells in an area of skin

• Papules with a warty surface. May continue to expand and become more verrucous with age

• Majority of lesions are present at birth and up to 95% of the lesions are present by 7 years of age

• Appear anywhere on the body most commonly found on extremities can follow Blaschko's Lines

Aplasia Cutis Congenita

• Failure to form certain layers of skinCan involve bone or meninges

• Autosomal dominant transmission in some cases

• Present at birth

• Ulcer: 1-3cm in diameter, round, well-demarcated, hairless

• 80% lesions located on scalp, most in midline

Aplasia Cutis Congenita

• Larger lesions are deeper with higher risk

hemorrhage or meningitis

• Usually heal spontaneously over months, leaving circular atrophic scar

• May be an isolated lesion OR in associated with:Cleft palate or lip, syndactyly, absence of digits,

eye anomalies, and congenital heart disease

Acne Neonatorum

• Multiple, inflammatory, erythematous papules & pustules

• Mainly on cheeks; also on rest of face, scalp, chest, back

• Papules at 2-4 wks pustules after few weeks resolve spontaneously in 1-3 months

• In 50% of newborns

• A part of the “miniature puberty of the newborn “

Infantile Seborrheic Dermatitis

• Patches of erythema with white-yellow, greasy scale

• Face, scalp (cradle cap), and skin folds

• Occurs at 2-6wks of life clears over weeks/months and generally does not recur

• Increased risk for S. aureus and Candida superinfection, especially in diaper region

• Sebaceous glands are active at birth due to maternal androgens

Atopic Dermatitis

• Main feature: ITCH • Infentile AD:

– 2mo-2yo; 1/3 progress to Child AD; face & scalp, can involve trunk, and later EXTENSOR surfaces

• Child AD:– 2yo to adolescence; 1/3 progress to

Adolescent AD; FLEXURAL surfaces

• Adolescent AD: – May continue into adulthood; hand

dermatitis most common

Atopic Dermatitis

• Erythematous, pruritic papules with dry skin & excoriation

• Atopy triad:– Asthma, allergic rhinitis, AD

• Triggers:– dry or cold climate, irritants such as soaps

or wool, stress or anxiety, secondary bacterial infection, occlusive clothing (sweat and heat intolerance), food allergy

Cutaneous Infections

Diaper Dermatitis

• Perianal in location and is related to the irritant substances found in stool

• A bright red perianal acute dermatitis

• Superinfection with Candida albicans is frequent if dermatitis present for greater than 72 hSatellite lesions border diaper area

Herpes Simplex Infection

• 0.2-0.5/1000 live births • Intrauterine/congenital: 4% of HSV infected

newborns if survives, have late skin and CNS sequelae

• Perinatal: 80-90% of HSV infected newborns:3 possible forms: Localized (skin, eye, or mouth)Disseminated: mortality is 50% even with antiviral

therapy CNS infection (sepsis or encephalitis):40% do not have

skin lesions • Transmission risk:

Mom's primary infection: 50% Recurrent infection: 5%

Herpes Simplex Infection

• Post natal 10% of HSV infected newbornsbreast lesions, family members, and healthcare

workers

• Skin: grouped vesicles on erythematous base• Mucosa:shallow erosion with inflammation

• 80% of neonatal infections are HSV-2; 20% are HSV-1

• Rapid diagnosis of HSV infection is essential Rapid tests: Tzank smear

• IV acyclovir, regardless of the clinical presentation

Tinea Capitis

• Fungal infection of the scalpTrichophyton tonsurans &Microsporum canis most

common

• Mostly affects children rarely adults • Focal or diffuse erythema, scale, broken hairs,

hair lossKerion: dermatophyte induced inflammatory

lesion with edema and pus with risk of scarring and permanent alopecia

• Culture to determine sensitivity of causative organism; KOH slide with hyphae and spores

• Oral antifungal required

Impetigo Contagiosa

• Mostly early childhood, during the summer• Staphylococcal 50-70%, Sterptococcal* or

combined infection• Exposed parts of the body • Erythematous macules thin-walled

vesicles pustules golden yellow crusts

• Autoinoculation; spread peripherally and clears centrally forming large gyrate patterns

Impetigo Contagiosa (II)

• Can complicate other conditions (pediculosis capitis, scabies, herpes simplex, etc…)

*Strep group A ß-hemolytic impetigo may be followed (2-5%) by PSGN, more common (10-15%) with nephrogenic strains (44, 55, 57, 60 & M-type 2) especially in children under age 6. Early treatment Dose Not reduce the risk

• Treatment: Systemic antibiotics & topical therapy

Bullous Impetigo

• Characteristic in newborn infants (4-9 day); highly contagious; threat in nurseries

• Bullae on face & hands constitutional symptoms bacteremia, pneumonia, meningitis

• In warm climates can occur in adults; large fragile bullae in axillae, groins, hands. D.D: pemphigus

• In children onset after insect bite• Bullous Impetigo may be an early

manifestation of HIV infection

Diseases Produced by Toxins

Staphylococcal Scalded Skin Syndrome (SSSS)

• Most common in neonates & young children; rare in adults predisposed by renal /immune compromise

• Staph aureus (group 2 phage type 71) at a distant focus epidermolytic exotoxin fever, skin tenderness & erythema of the neck, groins, axillae sparing the palms soles & mucous membranes Nikolsky’s sign + with generalized exfoliation in large sheets below the granular layer unlike TEN (dermoepidermal junction; drug-induced)

Staphylococcal Scalded Skin Syndrome (II)

• Pathogenesis: the “target” of the epidermolytic exotoxin is desmoglein 1 (a member of the cadherin supergene family;160KD a transmembrane glycoprotein of desmosomes) identical to the target in pemphigus foliaceus

• Skin cultures- negative; • Diagnosis: frozen section • Treatment: Systemic antibiotics & supportive

care• Prognosis: good

Erythema Toxicum Neonatorum

•Impressive title - harmless skin condition•Erythematous macule with a central tiny

papule, seen anywhere - except the palms and soles.

•The lesions are packed with eosinophils, and there may be accompanying eosinophilia in the

blood count .•The cause is unknown, and no treatment is

required as the rash disappears after 1-2 weeks .

Miliaria

•Prickly heat, sweat rash

•Many red macules with central papules, vesicles or pustules are

present .

•These may be on the trunk, diaper area, head or neck .

Subcutaneous Fat Necrosis

•Self limited, benign condition•Sharply demarcated reddish to

violaceous plaques or nodules•Etiology uncertain•Onset first few days- weeks of life•Cheeks, back, buttocks, arms, and

thighs

Infantile Atopic Dermatitis

•Cause is unknown•Red, itchy papules and plaques

that ooze and crust•Sites of Predilection

–Face in the young–Extensor surfaces of the arms and

legs 8-10 mo.–Antecubital and popliteal fossa , neck,

face in older

Viral Exanthems

Smallpox- Variola

•Fatality 40% •First invades upper respiratory tract•From lymph nodes it spreads via

hematogenous spread•Chills, fever, headache, delirium, SZ•Face to upper arms and trunk, and

finally to lower legs

Chickenpox-Varicella

•Herpes virus varicellae•Incubation period 10-21 days•Fever, malaise, cough,

irritability, pruritus•Papulesvesicles crusting•Spreads centripetally

Measles

•Rubeola- paramyxovirus•Occurs in epidemics•Incubation 8-12 days•Fever, lethargy, Cough, coryza,

conjunctivitis with clear discharge and photophobia

•Koplik spots•Rash begins on the face and spreads to

trunk and extremities

Rubella

•German Measles•Epidemic nature•Winter-spring•Prodrome•Face neck trunk•Lymphadenopathy•Serologic testing

Hand-Foot-Mouth Disease

•Enteroviruses–coxsackieviruses A and B–echoviruses

•Vesicular lesions, may be petechial

•Associated with aseptic meningitis, myocarditis

Erythema Infectiosum

•Fifth disease•Mildly contagious, parvovirus B-19•Pre-school and young school-age children•Prodrome: mild malaise•Rash: “slapped cheek”, circumoral pallor,

peripheral mild macular distribution •Complication

Exanthem Subitum

•Roseola Infantum•Children 6-19 months•Abrupt onset of high fever•Febrile seizures•Rash develops after fever

dissipates•Mainly on trunk

Infectious Mononucleosis

•Acute, self limited illness•Epstein-Barr virus•Oral transmission – incubation 30-50 days•Fever, fatigue, pharyngitis, LA,

splenomegaly, atypical lymphocytosis•Exanthem is seen in 10-15%•Erythematous, maculopapular,

morbilliform, scarlatiniform, urticarial, hemorrhagic, or even nodular

Bacterial Exanthems

Impetigo

•Superficial infection of the dermis•Two types:

–Impetigo contagiosa–Bullous impetigo

•Etiology–Group A ß hemolytic streptococcus–Coagulase positive S. aureus

•Treatment : Keflex, erythromycin, Bactroban

Scarlet Fever

•Toxin producing strain of group A -hemolytic streptococcus

•Strep pharyngitis with systemic complaints•Rash from neck to trunk to extremities•Sandpaper feel, erythema, warmth•White and red strawberry tongue•Petechiae in linear form•Complications•Treatment

Staphylococcal Scalded-Skin Syndrome

•Generally in less than 5 years of age•Induced by exotoxin produced by

staphylococci•Fever, papular erythematous rash starting

around mouth- not involving oral mucosa•Positive Nikolsky’s sign•Diagnosis: Tzanck test, bacterial culture•Treatment•Complications

Meningococcemia

•Usually sudden onset of fever, chills, myalgia, and arthralgia

•Rash is macular, nonpruritic, erythematous lesions

•Petechial rash develops in 75% of cases•Neisseria meningitides•Fever, rash, hypotension, shock, DIC•Treatment: PCN G

Differential Diagnosis

•Gonococcemia

•HSP•Typhoid fever•Rickettsial disease•Erythema multiforme•Purpura fulminans

Rocky Mountain Spotted Fever

•Most common rickettsial infection in US

•Abrupt fever, headache, and myalgia

•Rash from extremities towards trunk•Maculespetechiae•Treatment

–Tetracycline–Doxycycline–Chloramphenicol

Cellulitis

•Most common organisms:–S. aureus–S. pyogenes–H. influenza type B (HIB)

•Most common sites?

•CBC, x-ray ?

Periorbital- Orbital Cellulitis

•S. aureus, S. pneumoniae, and HIB

•CBC, blood culture, CT•LP?

•IV antibiotics•Admit

Fungal Infections

Henoch-Schnlein Purpura

•No clear etiologic agent, often post viral

•2-10 years of age•Palpable purpura over the

buttocks and LE•Transient migratory arthritis•Renal and GI involvement

Kawasaki Syndrome

•Unknown etiology•Peak incidence 18-24 months•Clinical findings:

–Fever for at least five days–Conjunctivitis–Polymorphous rash–Oral cavity changes–Cervical adenopathy