Dermatology - Pediatric Birthmarks and Exanthems

From Iusmicm

Contents 1 Pediatric Birthmarks and Exanthems 1.1 Objectives 1.2 Pediatric Birthmarks 1.2.1 Tumors versus Malformations 1.2.2 Infantile Hemangiomas 1.2.3 Capillary malformations 1.2.3.1 Port-wine stain 1.2.3.2 Port-wine stains and Sturge-Weber Syndrome 1.2.4 Congenital Melanocytic Nevi 1.3 Childhood Exanthems 1.3.1 Varicella (Herpes = Chickenpox) 1.3.2 Rubeola (Measles, Morbilli) 1.3.3 Rubella (German Measles) 1.3.4 Erythema Infectiosum (fifth disease) 1.3.5 Roseola Infantum 1.3.6 Hand-Foot-and-Mouth Disease 1.3.7 Scarlet Fever

[edit] Pediatric Birthmarks and Exanthems

[edit] Objectives

• Differentiate common pediatric birthmarks
• Understand the natural history and treatment options
• Be familiar with signs and symptoms associated with various pediatric exanthems

[edit] Pediatric Birthmarks

• There are three major types of pediatric birthmarks: hemangiomas, port-wine stains, and congenital melanoytic nevi
• An exanthem is a cutaneous lesion whereas an enanthem is a mucous membrane lesion.

[edit] Tumors versus Malformations

• We classify birthmarks as either tumors or malformations, depending on the amount of abnormality of the structure
• Tumors include (proliferative): infantile hemangiomas, tufted angiomas, and kaposiform hemangioendotheliumas
• Malformations include (abnormal formation): port-wine stains (capillary), venous, lymphatic, and arteriovenous malformations

[edit] Infantile Hemangiomas

• An infantile hemangioma is a benign tumor of vascular endothelium
• About 4-5% of Caucasian infants develop an infantile hemangiomas
  • Most hemangiomas develop in the first year of life
  • Most spontaneously regress by age 5-10
  • http://3.bp.blogspot.com/_uH5esL2gOT0/SFfBjYtEOLI/AAAAAAAAAuI/_YE8XMRxYSo/s400/hemangioma
• Risk factors include: being female, premature birth, low birth weight, and being a twin
• We no longer use the terms "capillary" or "cavernous" when describing hermangiomas (in an attempt to standardize the nomenclature).
• Infantile hermangiomas can be superficial, deep, or mixed.
• Infantile hermangiomas can be localized, segmental, indeterminate, or multifocal.
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• Ulceration is one of the complications when hemangiomas are at a high-friction area.

• Localized:
  • Doesn't indicate underlying structural development issues
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• Segmental:
  • Often associated with improper development of a particular metamome (embryological unit)
  • May indicate underlying structural developmental issues
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• Indeterminate:
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• Multifocal:
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• Treatment for infantile hermangiomas
  • Observation (90% or more)
  • Corticosteroids (for large, disfiguring, ulcerating, etc.)
    • The mainline therapy
    • Likely works by inhibiting factors that promote angiogenesis
  • Beta-blockers: propranolol, timolol
    • An alternative therapy
    • Likely works by inhibiting VEGF / FGF, inducing vasoconstriction, and perhaps even by inducing apoptosis of the endothelial cells
    • Based on case studies as of now
  • Interferon-alpha
    • An alternative therapy
    • Used when corticosteroid are not effective
    • A potent inhibitor of angiogenesis
  • Laser therapy (selected cases)
    • Only after involuted if it leave some tissue behind.
    • Cannot be used on the primary hemangioma
  • Surgical excision (rarely)
  • Vincristine
    • An alternative therapy
    • A mitotic inhibitor from madigascar periwinkle that is used as a chemotherapy
    • Induces apoptosis of tumor cells and endothelial cells
    • Also used in other infantile birthmark tumors like tufted angiomas and kaposiform hemangioendotheliomas

[edit] Capillary malformations

• Capillary malformations cause vascular stains on the superficial epdiermis.
  • These are often colloquially referred to as as a "salmon patch", an "angel's kiss", a "stork bite", or a "nevus simplex"
• May light up upon normal physiological capillary dilation (anger, hard work, etc.)
• Capillary malformations are very common
• Capillary malformations tend to occur on the glabella, the eyelids, the nose, the upper lip, and the nape of the neck.
  • Note that the glabella is the smooth part of the forehead above and between the eyes
• Most capillary formations fade over 1-2 years, except those in the neck location.
• Capillary malformations include many types; we will focus on port-wine stains.
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[edit] Port-wine stain

• A port-wine stain is a superficial vascular malformation
• Less than 0.5% of live births manifest a port-wine stain
• Port-wine stains do not regress
• Can generate capillary malformations that can bleed.
• There is potential for a port-wine stain to thicken and develop benign vascular papules
  • Recall that a papule is a "Circumcised, flat (non-palpable), discolored".
  • A macule is a small papule.
• Treatment for port-wine stain is by vascular lasering
  • Lasers selectively destroy certain cells while leaving surrounding cells intact
  • In this case we aim for the vascular cells but not the rest of the dermal / epidermal cells
• Port-wine stains can be progressive: darkening, developing blebs, developing bone and soft tissue hypertrophy
• Describe PW stains by their trigeminal distribution
  • Because in V1 or peri-optical, we worry about WEb-Sturger syndrome with neuro issues
    • Should be monitored regularly

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[edit] Port-wine stains and Sturge-Weber Syndrome

• Sturge-Weber syndrome is characterized by:
  • Port-wine stains, seizures, mental deficiency, intracranial bleeding, intraocular bleeding
• Sturge-Weber sydndrome is only associated with port-wine stains in the CN5 V1 distribution
• 15% of V1 PWS will end up with Sturg-Weber syndrome
• Bilateral V1 port-wine stains are at higher risk for having Sturge-Weber syndrome

[edit] Congenital Melanocytic Nevi

• Another form of infantile birthmark is the melanocytic nevi
• Melanocytic nevi are bening hamartomas of melanocytic cells
  • Recall that a hamartoma is an abnormal growth of normal cells
• We classify melanocytic nevi by their size: small, medium, and large.
  • Size < 2cm
  • Medium is less than 10-20 cm
  • Large is bathing-trunk size
    • 20% risk for melanoma
    • 50% of melanomas develop in the first 5 years

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• CMN (congenital melanocytic nevus) management includes monitorying and potentially surgical removal
  • Watch for the ABCDEs (asymmetr, irregular borders, heterogenous color, large diameter, and expansion)
  • May remove only small, hard to follow areas of really large nevi
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• CMN may develop a nodule or other characteristics whereas acquired nevi are unlikely to develop secondary characteristics.

[edit] Childhood Exanthems

• An exanthem is an eruption at the skin secondary to a system infection.
• Examples of systemic infections that cause exanthems:
  • varicella (chickenpox),
  • rubeola (measles),
  • rubella (German measles),
  • roseola infantum,
  • erythema infectiosum,
  • hand-foot-mouth disease,
  • scarlet fever

[edit] Varicella (Herpes = Chickenpox)

• Varicella is a systemic infection by herpes
• VZV = varicella zoster vaccine
  • Can result in systemic infection; rare
  • Usually occurs in immune deficient pts
    • One type is zn deficiency
• Incubation of the infection takes 14-16 days
• Characterized by itchy lesions at different stages
  • Described as "dew drops on a rose petal"
• It is better to get chickenpox as a child!
  • Because older pts are more likely to have pulmonary complications via varicella pneumonia.
• Clinical vignette: 3 yo male presents with fever, headache, sore throat. Develops a rash on face several days later. Facial rash is followed by papular (as in a large, circumscribed, non-elevated, discolored patch) rash on face and over trunk and extremities including the palms and soles. Palpetral conjunctivitis and photophobia followed.
• Recall that a Tsanck smear is used to diagnose chickenpox / shingles

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[edit] Rubeola (Measles, Morbilli)

• Morbiliform means measles; anything can be the cause, but it is showing up like measles
• Rubeola (measles) is a systemic infection by a paramyxovirus of the genus Morbillivirus
• Rubeola has a 10-14 day incubation period
  • Recall that varicella's incubation period is 14-16 days
• Rubeola (measles) presents as cough, coryza, conjunctivitis, photophobia, and fever.
  • Coryza is catarrhal inflammation of the nasal mucuc membrane
  • Catarrhal is having to do with a respiratory infection
• Rash begins on day 4-5; morbilliform eruption
• Rubeola (measles) is characterized by koplik spots on the oral mucosa
  • Small, irregular, bright red spots with a central bluish-white speck
  • Usually near the second molars
  • Appear 24-48 hours before the rash
• Complications of Rubeola:
  • Bronchitis
  • Encephalitis (1 / 1000)
  • Ear infection (otitis media)
  • Pneumonia

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• Measles (rubeola) was eradicated in the US in 2000
• There are still 30-40 million deaths worldwide each year
• In the US in 2011, there have been 211 cases so far
  • 25 of them are in California
  • 85% of them are in unvaccinated children
  • Vaccination exemptions are on the rise
  • CDC predicts that an outbreak can occur if 5-10% of the population is not vaccinated
• Measles (rubeola) clinical vignette: a 4 yo boy has a low grade temperature, sore throat, cough, and lympadenopathy followed by a rash on the face that spreads to the trunk and extermities. Eruptiosn disappear 2 days later but a mild desquamation resides
  • Note that both varicella and rubeola have a rash that starts on the head and moves to the trunk / extremities.
  • Varicella has a fever, photophobia, conjunctivitis, and rash on the palms and soles that set it apart from rubeola.
  • Rubeola has lymphadenopathy and eruptions that resolve in 48 hours, which sets it apart from varicella.

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[edit] Rubella (German Measles)

• Rubella is a systemic infection by rubella virus
• A morbilliform illness
• Rubella (German Measles) is considered a milder form of rubeola (measles)
• Rubella (German Measles) has a 14-21 day incubation
  • This is a longer incubation period than varicell (14-16 days) and rubeola (10-14 days)
• Rubella is characterized by encephalitis and thrombocytopenia (which set it apart from rubeola)
• Rubella has a progression twoard the cephalocaudal poles.

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[edit] Erythema Infectiosum (fifth disease)

• Erythema infectiosum is a systemic infection by parvovirus B19
• Can recur in just a couple weeks
• Note that "fifth disease" was a name given in recognition that erythema infectiosum was typically the fifth sickness a child experienced.
• Incubation is 7-14 days
  • Potentially shorter than varicella, rubeola, and rubella
• Erythema infectiosum has three distinct phases: slapped cheeks, fishnet erythema, and recurrence
• Erythema infectiosum is marked by purpuric (itchy) petechiae-like lesions in a gloves and socks distribution.
• Erythema infectiosum clinical vignette: 2 yo active child has 4 days of fever (up to 102F); parents no other symptoms. On 5th day, fever resolves and full body non-pruritic rash is observed.

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[edit] Roseola Infantum

• Roseola infantum is a infection by herpes, strains 6 or 7
• High fever goes away with advent of rash
  • Note that rubeola as in standard measles is a systemic infection by a paramyxovirus of the Morbillivirus genus
  • Recall: Rubeola (paramyxovirus of morbillivirus virdae) -> Standard Measels, Rubella (rubella virus) -> German Measles, Roseola (herpes virus 6 / 7) -> Roseola infantum
• Roseola infantum has a 5-15 day incubation period
  • One of the widest ranges of the childhood exanthem-causing systemic infections
• Roseola infantum is marked by high fever in an otherwise well child
  • Recall that varicella can also present with fever but will have other non-well symptoms like headaches, photophobia, and conjunctivitis.
• Roseola infantum (not simply "roseola") presents with a pale-pink macular rash as the fever fades.
  • Note that, like varicella, roseola's rash can manifest on the hands.
  • Roseola infantum's rash can also develop on the tongue or on the mucosal membrane.
  • Note that roseola infantum has the palest rash.

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[edit] Hand-Foot-and-Mouth Disease

• Hand-Foot-and-Mouth disease is a systemic infection by Coxsackie virus A16 or Enterovirus 71
• HFM has the very short incubation period: 3-6 days.
  • Only scarlet fever has a shorter incubation period.
• HFM occurs in 3-year cycles; manifests in summer and fall
• Lesions of coxsackie / enterovirus hand-foo-and-mouth disease are primarily found on the mouth--less so on the hands and feet.
  • Note that HFM mouth lesions are easily differentiated from koplik spots of measles (rubeola).
• Hand-foot-and-mouth infections last about 7-10 days

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[edit] Scarlet Fever

• Scarlet fever is a systemic distribution of streptococcal toxin
  • Fine little discrete papules that are all over the trunk
• Desquamation can be pretty severe: peeling of skin on hands, trunk
  • Severe form can even make nails look like they will come off
• Scarlet fever has the shortest incubation period of the exanthems (we studied): 2-4 days.
• Scarlet fever presents as fever, pharyngitis, and a strawberry tongue.
• The rash of scarlet fever begins on the neck and spreads to the trunk and then the extremities.
  • Scarlet fever rash is marked by its sandpaper texture.
• There is significant desquamation after resolution of scarlet fever rash.

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