Derm #7: Pediatric Dermatology

Front 1

What is vernix caseosa?

When is epidermal maturation complete in the newborn?

Back 1

Vernix caseosa= layer covering newborn. desquamated cells, lanugo hairs, antimicrobials, sebaceous gland secretions. Sheds in a few days.

Maturation is completed by ~34 weeks

Front 2

Why should caution be exercised in using any topical substances in neonates?

Back 2

1. ↑ surface area: body weight ratio
2.potentially increased permeability and abnormal barrier (esp. in those born with blistering disorders or erythroderma)

Front 3

What is the yellow color due to? What accounts for this appearance of the skin (note- this neonate is 6 hours old)?

Back 3

Meconium

Normal skin looks wrinkled because baby was in warm, wet environment.

Front 4

What is this called? Where is the pigment located?

How can it be differentiated from bruising? What happens to the pigment?

Back 4

Mongolian spot aka. Dermal Melanosis.

Different from bruising- doesn't go through color changes of bruising. Regresses throughout life.

Front 5

What is this called? Does it fade over time?

Back 5

Angel's kiss or "Nevus simplex" capillary stain

Around 50% persist throughout life. Can fade and return when baby becomes emotional/cries. Not bruises (eyelid bruising is rare in babies!)

Front 6

What is this called? How does it form?

Back 6

Stork bite

It's a "Nevus Simplex" capillary stain (from dilated capillaries)

Front 7

What is this common rash? When does it develop?

Back 7

Erythema Toxicum (looks like flea bites or pustules)

Baby is a newborn (develops after birth). Benign.

Front 8

What is this called and in what demographic is it commonly seen? When does it develop?

Back 8

Transient neonatal pustular melanosis

Develops in african american children IN UTERO. Sometimes see colarette of scale (from desquamation around lesion).

Front 9

What is on the shoulder of this baby?

Back 9

Lanugo hairs (common in babies, shed with the vernix caseosa) but can be seen in adults in certain malignant conditions.

Front 10

What is this? What demographic is it typically seen in?

Back 10

Hyperpigmentation

Typically seen in Indian, Asian, pigmented races. It will distribute over first couple of months of life. Babies are lighter at birth than later in life.

Front 11

What is the small lesion on the baby's right cheek?

Back 11

Milia (mini epidermal inclusion cyst filled with keratin). It will desquamate/shed over time.

Front 12

What is this condition (seen on nose)? What can it result from?

Back 12

Sebaceous hyperplasia

Can result from maternal hormone stimulation.

Front 13

What is this condition? How can you make it go away?

Back 13

Physiologic Cutis Marmorata (marbling of skin)

When child is cold, vasoconstriction results in this pattern. Will disappear by warming up child (place into isolette).

Front 14

Name the normal neonatal lesions? In what climate does this condition show?

Back 14

Miliaria crystallina (fragile, superficial)
Miliaria rubra (prickly appearance)

Both transient and occurs when child is in warm environment.

Front 15

What is this? What is the word for failure to close?

Back 15

Nasal encephalocele (pocket of brain tissue through an area of DYSRAPHISM).

Front 16

What is the condition on the top left called? What about the bottom right? If you see a baby with either of these, what do you want to do?

Back 16

Top left= midline hemangioma, Bottom right= "fawn tail" or hypertrichosis over midline. Both suggestive of occult spina bifida.

**work up the child because they can develop bladder spasticity early on.

Front 17

What is this lesion called? Why do we worry about it so much?

Back 17

Dermoid cyst (contains hair, sebum, keratin, glands).

Dangerous because some can contain ostium that has a sinus tract with extension to CNS. Easy access for bacteria --> recurrent meningitis.

Front 18

What condition might this child be recurrently afflicted by? What do you have to do to treat this?

Back 18

Recurrent meningitis (this is a dermal sinus that tracks to the meninges, and if bacteria enters it can cause infection).

Needs to be removed by neurosurgeon.

Front 19

What is this lesion called? If it occurs at the vertex/midline what can it be associated with?

Back 19

Aplasia cutis congenita. (congenital absence of skin)

May not be associated with anomalies but if in midline, could be associated with underlying NEURAL TUBE DEFECT. Make sure to image!

Front 20

What would you be worried about in this child who also has a port wine stain?

Back 20

This combination (hair collar + port wine) suggests an atreitic encephalocele. In general, you want to image these patients for possible underlying malformations.

Hair collar= ring of dark, coarse hair.

Front 21

What is the name of the patterns shown here? Disorders that follow this pattern suggest what?

Back 21

Lines of Blaschko (lines of skin embryogenesis)

Disease following the lines represent genomic/epigenomic MOSAICISM.

ex: linear epidermal nevus

Front 22

What is this called?

Back 22

Nevus sebaceous (organoid hamartoma). Overgrowth of sebaceous glands in the area of a nevus.

Front 23

What is this lesion called? What does it tell you about the origin of the condition?

Back 23

Hypomelanosis of Ito or mosaic hypopigmentation

Occurs in swirling pattern that tells you it's a Genetic Mosaicism.

Front 24

What is this spot?

Back 24

An accessory nipple (occurs in the milk line). Might get mistaken for a nevus.

Front 25

What does an epidermal melanin unit consist of?

Back 25

Melanocyte + up to 30 keratinocytes (melanosomes which contain melanin pigment are discharged into keratinocyte)

Front 26

What is this condition called? Where on the body does it tend to present? When in life is it evident?

What other conditions might this person have?

Back 26

Vitiligo- presents on extensor surfaces mainly

Acquired disorder- presents later in life (one key difference from Piebaldism). Absence of melanocytes.

Likely related to autoimmune mechanism so other autoimmune disorders may run in the family.

Front 27

What is this called? What is the treatment for vitiligo?

Back 27

Poliosis (vitiligo involvement in hair)

Treatment = 1) corticosteroids- effects autoimmune mxn. 2) Repigmentation (via phototherapy)- because some pigment is preserved in these individuals.

Front 28

What might this person be affected with? How can you tell the difference between this and Vitiligo?

Back 28

Post Inflammatory Hypopigmentation (pityriasis alba). Possibly the person has tinea versicolor infection.

Melanocytes are intact but defective transfer of melanosome to keratinocyte. Under Wood's lamp, vitiligo looks chalk white.

Front 29

This individual has what mutation/inheritance pattern? What is the difference between this and Vitiligo?

Back 29

Mutation in cKIT proto-oncogene (Autosomal dominant).

This is congenital mutation (born with it). White forelock. Problem with production of pigment (melanocytes exist but pigment doesn't).

Front 30

How is melanin synthesized? What is the crucial enzyme in the synthesis of melanin?

Back 30

L-phenylalanine is turned into L-dopa which eventually becomes Eumelanin (black pigment) or Pheomelanin (red pigment)

Tyrosinase= crucial enzyme(L-tyr to L-dopa, and L-dopa to dopaquinone)

Front 31

What is this condition called? What mutation does this individual have? What is their melanocyte count?

Back 31

Oculocutaneous Albinism

OCA-1= tyrosinase negative, AR. Normal NUMBER of melanocyte. Note: translucent iris, burn easily. Nystagmus (vision problem).

Front 32

This is the fundus exam of a person with what condition? How can you tell? What would the person look like if you transiluminated their eye?

Back 32

Oculocutaneous Albinism

You can make out choroidal circulation because they have a lack of pigment (it stands out). Red Iris Reflex is seen upon transilumination of eye.

Front 33

What condition does this individual have and how can you tell? What mutation does he have?

Back 33

OCA-2 (they have more yellow/reddish hair).

Mutation in P-protein (which is responsible for transport of melanin). Often hunted and killed in Tanzania because believed to have magical body parts.

Front 34

What is this? Is it congenital?

What is the most significant predisposing factor for this type of lesion?

Back 34

Early hemangioma (looks flat, like a bruise). NOT congenital (though it can present early in life).

Predisposing factor= Low Birth Weight

Front 35

What is the typical course of this lesion? Should it be monitored or is it benign?

Back 35

The lesion itself is benign and will regress over time. However this one is periocular and can compress eye causing amblyopia so should be monitored.

Front 36

What is this? Is it problematic?

Back 36

Hemangioma that is midline. Associated with occult spinal dysraphism (should get imaged).

Front 37

What is this? Is it problematic?

Back 37

Perineal/segmental hemangioma that can cause PELVIS syndrome (associated with painful ulcerations).

Front 38

What is this? What might this child have in addition to this lesion?

Back 38

PHACES syndrome- Posterior fossa abnormality, Hemangioma, Arterial abnormality, Coarctation of aorta, Eye anomalies, Sternal abnormalities

Upper segmental lesion associated with posterior fossa anomalies and arterial anomaly (she was missing R internal carotid).

Front 39

How do you treat hemangiomas?

Back 39

No treatment (typically spontaneously involute).

If there is a high risk hemangioma (airway compromise, affects eye, etc.) you can treat with Propranolol or Systemic steroids (too thick for laser). Or surgery.

Front 40

This lesion is often confused with a hemangioma. What is it?

Back 40

Pyogenic granuloma (often seen in sites of trauma or warts, friable and bleeds easily).

Front 41

What is this called? If it was in the distribution of V1 (first branch of the trigeminal) what would it be called?

Back 41

Port-Wine Stain- i.e. capillary malformation (if in V1 distribution, it would be Sturge Weber Syndrome).

Front 42

What is this syndrome associated with (note- it is in the V1 distribution of the trigeminal nerve)? How does this lesion differ from a hemangioma?

Back 42

Seizures and glaucoma (child has a glass eye). Differs from hemangioma because this is present AT BIRTH!

Front 43

What is the causative agent seen in this pathology? What type of infectious agent is it and how is it transmitted?

Back 43

Molluscusm Contagiosum (DNA poxvirus)
Spreads by fomite, autoinoculation (lives despite chlorine!)

Virus (note inclusion bodies)

Front 44

What is the causative agent? Treatment?

Back 44

Molluscum contagiosum (note umbillicated vesicles)

Treat with Cantharidin (extracted from beetles). Otherwise, you can open lesions but it is painful.

Front 45

What is the boggly plaque seen here called?

What is it associated with and how does it get transmitted?

Back 45

Boggy plaque = kerion (from inflammatory response)
Tinea Capitis is also seen (trasmit via shared hair brush, fomites).

i.e. scalp ringworm

Front 46

What is the most common cause of tinea capitis? Second most common cause? How can you tell the difference between these two?

Back 46

Trichophyton tonsurans = most common cause (seen in African Americans typically). Does not fluoresce.

Microsporum canis= 2nd most common *transmitted by infected cat/dog. Fluoresces blue/green under wood's lamp.

Front 47

How could you diagnose this causative agent in your office? What would you do to treat this?

Back 47

This is microsporum canis, t. capitis (alopecia with broken hair often seen).

KOH prep: to show endothrix (t. tonsurans) or ectothrix (m. canis).

*Treat with GRISEOFULVIN, or Terbinafine (of flucanazole)

Front 48

How are warts transmitted?

Back 48

Skin-to-skin contact, fomites (locker room floor), autotransmission, vertical transmission at birth.

Front 49

Name the wart:

1. common wart
2. flat wart
3. plantar warts (on feed)
4. anogenital or mucosal warts

Back 49

1. common = verrucae vulgaris
2. flat = verrucae plana
3. plantar= verrucae plantaris
4. genital = verruca acuminata

Front 50

What type of wart is this? what is the black dot?

Back 50

Common wart = verrucae vulgaris
Black dot represents thrombosed capillaries

Front 51

What type of warts are seen here?

Back 51

Verruca plana- flat warts

Flat top papules, can koebnerize to form linear arrangements due to scratching

Front 52

How do you distinguish these spots (are they warts or corns)?

Back 52

Plantar warts (typically in weight bearing areas). Can become tender.

Warts, when shaved have small black dot appearance (whereas corns do not). Corns= pinpoint callus on foot.

Front 53

A child that is <2 years old has this presentation. What do you suspect is the causative agent and what is the mode of transmission?

Back 53

<2= likely vertical transmission, Genital HPV (6,11)

2-8= likely from sexual abuse, Genital HPV 6,11

Front 54

What is seen on this histopathology? How do you treat it?

Back 54

HPV (note the halo around nucleus)

No specific antiviral treatment! Wait for it to regress, use keratolytics, cryosurgery, or podophyllin/trichloroacetic acid for condyloma acuminata.

Front 55

What infectious agent causes this disease? How was it transmitted? Is the child contagious?

Back 55

Parvovirus B19-- causes fifth's disease (slap in the face appearance). Transmitted via respiratory route.

The child, one they have macular erythema rash, is not contagious.

Front 56

What is the condition seen here?

Back 56

Irritant diaper dermatitis (note the sparing of the folds!).

Erythema, maceration, and scale from wetness, urinary contact.

Front 57

What condition is seen here? How do you know?

Back 57

Candida diaper dermatitis. You can make it out because of the satellite lesions and colorette of scale.

Child might also have thrush (oral candidiasis)

Front 58

What condition is this? How does it differ from diaper dermatitis?

Back 58

Napkin psoriasis (resembles seborrheic dermatitis). Well demarcated areas of erythema that INVOLVE the crease (unlike diaper dermatitis which spares the creases)

Front 59

What is this condition called? How would the baby present? How would you treat it?

Back 59

Acrodermatitis enteropathica
Baby may be irritable, have diarrhea, periorofacial/acral dermatitis, and alopecia

Typically seen after weaning (lack of Zn from breast milk). GIVE ZINC!

Front 60

This child presents with a diaper dermatitis that is resistant to treatment. The rash is composed of brownish papules and purpura. This lesion is also found under the arms. What might it be?

Back 60

Langerhans cell histiocytosis- refer to Hematology & get biopsy.

Front 61

A child is found who has multiple lesions of this type. What else would seal the diagnosis of NF-1?

Back 61

2+:
6+ cafe au lait
Axillary freckling
Plexiform neurofibroma
Two or more lisch nodules of iris
Optic nerve glioma
family history

Front 62

What is the mutation in individuals with Neurofibromatosis 1?

What is the mode of transmission?

Back 62

Neurofibromin (gene on 17q). Encodes GTPase which acts as a tumor suppressor.

Mode of transmission = Autosomal Dominant

Front 63

This is subtle- but a child who has this condition in addition to multiple cafe-au-lait spots should be worked up for?

Back 63

Tibial bowing (a skeletal dysplasia)- associated with NF-1.

Also note the plexiform neurofibroma that here mimics a necus.

Front 64

What does this little baby have? What is the major risk for the child?

Back 64

Neonatal lupus erythematosus. Raccoon eyes= characteristic lesion.

Heart block (because antibodies can deposit in AV node and affect bundle of His)

Front 65

What is this condition primarily due to (what is the mode of transmission)?

Back 65

Vertical transmission (transplacental transfer of IgG Anti-Ro SSA and Anti-La SSB antibodies).

*note: mom does not need to have active disease

Front 66

This 4.5 year old boy presents with a stubborn perianal rash that is not responding to topical steroid or nystatin. What is it?

Back 66

Group A Beta-hemolytic strep infection
"strep tush"

Tests positive for strep on throat culture

Front 67

What is this (note, the belt buckle)?

Back 67

Nickel contact dermatitis