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Pediatric Board Review - Dermatology

Terms in this set (88)

Aplasia cutis
congenital absence of skin that usually
occurs only in a small localized area, most commonly on the scalp (Image 7-1). However, - 20% have underlying skull abnormalities. If it occurs in multiple places on the top of the scalp, look for trisomy 13. Larger or midline defects can have underlying bony defects or vascular
abnormalities. If it occurs as a midline defect, look for spinal dysraphia
Milia
These are l-2-mm, firm, white papules that appear at the surface of pilosebaceous units, most commonly on the face. These tiny, epidermal inclusion cysts generally
resolve spontaneously over several months and require no treatment
Sebaceous Hyperplasia
Seen in approximately 50% of term newborns, this is the most important condition in the differential diagnosis of milia. Typically, several pinpoint, white-to-yellowish, fine papules are present on or around the nose and upper lip. Sebaceous hyperplasia is caused by increased androgen stimulation in utero and generally resolves spontaneously during the first few weeks of life
Neonatal Acne
Neonatal acne (also known as neonatal cephalic pustulosis) is generally apparent within the first 2-4 weeks of life. Look for the distribution of many small, 1-2-mm,
dusky pink papules and pustules on the face and scalp. Comedones are lacking in neonatal acne. Neonatal acne usually
resolves spontaneously within the first 1-2 months of life.
Infantile Acne
In contrast, infantile acne normally becomes evident at 2-4 months of age and is caused by androgenic stimulation of the sebaceous glands. In addition to papules
and pustules, there are typically open and closed comedones distributed over the face. Because infantile acne can be more persistent, and occasionally results in
scarring, treatment with topical benzoyl peroxide or antibiotics can be beneficial
Epstein Pearls
These are small, benign, whitish-yellow papules on either side ofthe raphe on the hard palate of the newborn. These are essentially intraoral milia and resolve without treatment.
Bohn Nodules
These are small, benign retention cysts in the mouths of infants. Found on the alveolar ridges, these lesions generally
resolve spontaneously during the first several months of life.
Miliaria rubra
Prickly heat occurs when the sweat glands are blocked and the sweat escapes into the epidermis, producing red papulovesicles
Miliaria crystallina
also occurs with blocked sweat
glands. The sweat escapes just beneath the surface, producing noninflammatory vesicles that look like "clear droplets."
Erythema Toxicum
This is a common, self-limited, urticarial condition occurring in the first few days of life; rarely is it present at birth. It consists of small pustules filled with eosinophils. The pustules are surrounded by a red wheal. It comes and goes, and it appears at different sites. You see erythema toxicum primarily in term infants, and it typically resolves spontaneously within I week after birth. For reasons we don't understand, erythema
toxicum is almost never seen in significantly premature infants.
Transient Neonatal Pustular Melanosis (TNPM)
This is always present at birth with pustules that transform into scaly, hyperpigmented macules of uniform size
problematic hemangiomas
• Periorbital lesions: ~ 80% risk ofocular complications, including astigmatism, amblyopia, refractive errors, and, occasionally, blindness.
• Beard lesions (mandible, chin, submental): Watch for signs of subglottic hemangioma, including stridor, cough, or swallowing or respiratory difficulties. This usually occurs during the first 6 months of life and is more likely to be associated with bilateral lesions.
• Ear: Risk of obstruction in the external auditory canal, which can cause a conductive hearing loss and, if persistent, can impact/delay the development of normal speech.
• Nose and lip: These hemangiomas have a greater
tendency to ulcerate. Furthermore, these anatomic
locations carry an increased risk of significant cosmetic deformity.
• Midline lumbosacral region: Hemangiomas in this
region carry an increased risk of spinal dysraphism,
particularly when associated with other markers of
dysraphism, such as hypertrichosis, sacral dimple
or skin tag, or deviated intergluteal cleft. Evaluate
infants with these findings for underlying spinal cord abnormalities. MRI scan is the best test to rule out spinal dysraphism, but, in some centers, lumbosacral ultrasound is done first. GU anomalies also have been reported with large, lumbosacral hemangiomas.
• Multiple cutaneous hemangiomas (> 5): Can occur with visceral hemangiomas, especially of the liver
and GI tract. Most infants with> 5 have a benign
self-limited course, but a subset have severe,
disseminated, visceral involvement. These infants
are at risk for high-output congestive heart failure,
hepatic complications (including jaundice and coagulopathy), GI hemorrhage, and thyroid abnormalities.
PHACE syndrome
occurs with large, segmental facial hemangiomas:

• Posterior fossa abnormalities (Dandy-Walker syndrome)
• Hemangioma (generally large, cervicofacial lesions involving cranial nerve VI distribution)
• Arterial anomalies (typically intracerebral
arterial anomalies)
• Cardiac defects, especially coarctation ofthe aorta
• Eye abnormalities (microphthalmia)
Kasabach-Merritt syndrome
aggressive, vascular tumor associated with thrombocytopenia, consumptive coagulopathy and high-output congestive heart failure
Nevus simplex
Nevus simplex (a capillary malformation) is also known as salmon patch, angel kiss (on the glabella), or stork bite (on posterior hairline). You will see these pink-to-red blanching macules on half of all newborns. Most facial lesions fade
with time, while nuchal patches persist. These are
dilated, superficial vessels probably resulting from
vasomotor immaturity
Nevus Flammeus
Port-wine stains-sometimes called nevus flammeus are capillary malformations and occur in - 0.3% of newborns. They usually appear as pink, red, or violaceous
patches that persist throughout life and grow proportionally with the child. The lesions can lighten or darken slightly over time, and some darken and thicken in adulthood
SturgeWeber syndrome
Port-wine stains involving the ophthalmic branch of the trigeminal nerve (V1). Also consists of an ipsilateral cerebral vascular malformation that can cause neurologic complications, including:
• Seizures
• Intellectual disabilities
• Contralateral hemiplegia
• Characteristic ophthalmologic findings, particularly choroidal vascular anomalies and glaucoma
Klippel-Trenaunay syndrome.
A child with this syndrome presents with a vascular malformation (mixed capillary-venous-Iymphatic) of an extremity, with associated soft tissue and/or limb overgrowth, and with development of venous varicosities
Parkes Weber syndrome
similar to Klippel-Trenaunay syndrome but associated with more marked limb overgrowth-in both length and girth. The
skin is characterized by capillary stains, but there are also multiple arteriovenous fistulae that you can see on ultrasound with color Doppler. These patients usually
have a more problematic clinical course and can develop high-output cardiac failure and marked limb overgrowth
Nevus Sebaceous
Nevus sebaceous is considered a type of epidermal nevus-a localized lesion seen most commonly on the scalp and occasionally on the face. It consists of a
yellow- to salmon-colored, hairless plaque that often has a waxy texture. The risk of basal cell carcinoma within a nevus sebaceous was previously estimated at 10-15%! In recent years, some have questioned this statistic and believe that what were previously interpreted as basal cell carcinomas were actually benign hair follicle tumors. Nevertheless, most experts advocate prepubertal removal of a nevus sebaceous.
Nevus Spilus
Nevus spilus (speckled lentiginous nevus)
is relatively common and can be congenital
or acquired. It presents as a well-demarcated, tan or light-brown, non-hairy patch, usually on the trunk, face, or extremities. Characteristically, these
patches tend to develop multiple, small, dark macules and papules throughout the lesion and can resemble a chocolate chip cookie. These lesions have a minimal future risk of neoplastic change into melanoma.
Because they are often large, you would normally not excise the lesion prophylactically unless a worrisome
clinical change warrants it
Congenital Melanocytic Nevi
Congenital melanocytic nevi represent collections of melanocytes (pigment cells) in the skin that are typically present at birth or within the first few months of life
(Image 7-15). The nevi are generally classified based on the adult size of the lesion:
• Small: < 1.5 cm (found in 1-2% of newborns)
• Medium: 1.5-20 cm (present in - 0.6% of newborns)
• Large: > 20 cm (rare; present in - 0.02% of newborns

The future risk of melanoma within small or medium congenital melanocytic nevi remains unclear, but the risk appears to be small, and malignant change is rare before puberty. However, there is a 6-8% lifetime risk of developing melanoma within a large, congenital melanocytic nevus. The risk appears to be substantial during the first 5 years of life. The small- to medium-sized lesions usually present as well-demarcated, raised, uniformly pigmented lesions.
Colors can range from tan to brown to black. They also can develop coarse dark hairs
Mongolian Spots
Mongolian spots generally are present at birth and seen in > 90% of African-American and Native American babies; occurrence is < 10% in Caucasians
Nevus of Ota
Nevus of Ota is most commonly seen in AfricanAmerican or Asian infants. They present as unilateral, irregularly speckled areas of bluish-gray discoloration on the face--specifically on the periorbital area, temple, forehead, cheek, nose, an/or sclera. These are different from Mongolian spots in that they don't clear with time. Rarely, cutaneous and ocular melanoma can occur within these lesions, so periodic ophthalmologic and skin exams are recommended, as well as biopsy of darker or atypical areas that can suggest malignant change
Cafe-au-Iait Spots
Having I or 2 lesions is normal, but large (> 3 cm) or multiple lesions can indicate a neurocutaneous syndrome.
Crowe sign
small, grouped, freckle like, cafe-au-lait spots measuring 1-4 mm in the axilla or groin. These are indicative of neurofibromatosis Type I!
Other associations with cafe-au-lait spots
Neurofibromatosis Type 2, McCune-Albright syndrome, Watson syndrome, and occasionally tuberous sclerosis
Ichthyosis Vulgaris
Ichthyosis vulgaris is the most common form of the ichthyoses and occurs in 1/250. It is autosomal dominant (AD) and is due to loss of function mutations in the gene encoding filaggrin. Ichthyosis typically improves in hot, humid climates and during
the summer months. It is commonly associated with atopic dermatitis.
Treatment is irritant avoidance and use of emollients and keratolytic products
X-Linked Recessive Ichthyosis
X-linked recessive ichthyosis occurs in 1/2,000-6,000 boys and usually becomes apparent at birth or during the first few months of life. The scales are more pronounced than in the AD form and tend to be bigger and darker. The trunk is involved, but the palms and soles are unaffected. It typically affects the neck and ears but spares the antecubital and popliteal fossa. It is due to the absence of the microsomal enzyme steroid sulfatase.
Lamellar Ichthyosis
Lamellar ichthyosis is characterized by generalized large, dark plate-like scaling and minimal-to-no appreciable erythema.
Congenital ichthyosiform erythroderma
presents with smaller, fine scales and generalized pink erythema.
Oculocutaneous Albinism
Albinism occurs in most patients due to a genetic mutation in either the gene that codes for tyrosinase-important for melanin synthesis---or in the gene that codes for P protein, which leads to an abnormal transport of melanin to keratinocytes. These are primarily AR disorders with decreased pigmentation of the skin, hair, and eyes. Photophobia, nystagmus, and poor visual acuity are common.
Hermansky-Pudlak Syndrome
The Hennansky-Pudlak syndrome is a rare AR disorder with albinism, mild bleeding diathesis, and tissue storage of ceroid material. Epistaxis and prolonged bleeding
are common due to platelet storage pool defects. The ceroid material deposits in the lungs, GI tract, and renal tubule cells.
Chediak-Higashi Syndrome
This syndrome is due to a LYSTgene defect and presents with a silvery sheen to the skin and hair due to the accumulation of giant melanosomes, along with the inability
to transport melanin granules to epidennal cells. EBV triggers the "accelerated phase" with atypical lymphocytes, pancytopenia, and organ infiltration. Long-term survival requires bone marrow transplantation.
Type 1 Neurofibromatosis
Type I neurofibromatosis (von Recklinghausen disease) is an autosomal dominant (AD) disorder with multiple
cafe-au-Iait spots and neurofibromas.
Tuberous Sclerosis
Tuberous sclerosis is another AD neurocutaneous disorder and is associated with facial angiofibromas (adenoma sebaceum), ash-leaf spots, shagreen patch, and periungual fibromas.
Gorlin Syndrome
Gorlin syndrome (basal cell nevus syndrome) is an AD disorder due to mutations of the "patched" gene--a tumor suppressor gene that controls cell growth and patterning. These children develop basal cell carcinoma in childhood and have dysmorphic facies, palmoplantar pits, and skeletal defects.
Peutz-Jeghers Syndrome
Peutz-Jeghers syndrome is another AD defect; this one is due to mutations in the serine threonine kinase STKJJ gene. It presents with gastrointestinal polyposes and hyperpigmented macules of the mucosa, perioral areas, digits, palms, and soles
Incontinentia Pigmenti
Incontinentia pigmenti is an X-linked dominant disorder that is lethal in males. In females, the skin manifestations generally clear by adulthood-so a careful history
of a mother with recurrent miscarriages is important.
There are 4 stages of skin manifestations:
I) Patterned blistering that follows the lines of Blaschko (the routes of embryonic cell migration; it is not always obvious that this is a B1aschko-like pattern during the blistering stage). The blistering has a predilection for the extremities and is typically apparent within the first few weeks of life. The blisters often have erythematous background skin.
2) Verrucous papules occur after the first 2-6 weeks of life and persist for months.
3) Hyperpigmented linear swirl patches occur along the
lines ofBlaschko at 3-6 months and then persist for
years.
4) Hypopigmented patches replace the hyperpigmentation in affected adult women.

Cicatricial alopecia occurs in - 1/3 of patients. Delayed eruption of teeth occurs in - 2/3 of patients. The teeth are usually abnonnal in appearance and can be peg- or coneshaped; often, the child is missing teeth
Ataxia Telangiectasia
Ataxia telangiectasia is an AR disorder due to a mutation in the ATM gene, which prevents DNA synthesis from proceeding after radiation damage. Ataxia develops
early. Telangiectasias develop after the ataxia on the bulbar conjunctiva, between ages 2 and 6 years. The telangiectasias subsequently develop on the eyelids,
cheeks, ears, and flexor forearms. Patients have progressive neurologic deterioration and recurrent sinopulmonary infections.
EB simplex
AD disorder and occurs when the blister is located through the basal keratinocytes (lowermost layer of epidermal cells). EB simplex is due to defects in keratin genes 5 and 14. The blisters typically heal without scarring. EB simplex subtypes are usually
the least severe forms of this disorder and can be
localized to the extremities and sites of frequent trauma or friction.
Junctional EB
is AR and results in a defect in the hemidesmosomes or anchoring filaments, with the
cleavage plane through the epidermal-dermal junction. This form is due to many genetic etiologies. Blistering is severe and can occur spontaneously or after trauma. In the most severe Herlitz subtype (due to mutations in the Laminin 5 gene), death by sepsis is common at < 6 months of age
Dystrophic EB
occurs when the cleavage plane lies
below the basement membrane zone in the upper dermis. It is due to defects in the genes that encode Type VII collagen. An AD form results in blistering in localized areas of the knees, elbows, and dorsum of the hands. An AR form results in generalized blistering with extensive scarring, along with the potential for development
of squamous cell carcinoma by adolescence.
Cutis Laxa
Cutis laxa is a disorder of decreased or absent elastic tissue. It can be AR, AD, X-linked recessive, or acquired. It can present with pendulous folds of redundant skin
at birth (recessive form), or it can develop later in life (dominant). Those with severe disease have pulmonary emphysema, bladder or GI diverticula, and inguinal
or umbilical hernias. The acquired form occurs after penicillin or isoniazid administration and is commonly progressive.
Pseudoxanthoma Elasticum
Pseudoxanthoma elasticum has both AR and AD forms. Yellow papules develop on the axilla, groin, and neck-and then become wrinkled. The areas have been
described as "plucked chicken skin." Gl bleeding, claudication, and angina pectoris occur with this disease.
Hypohidrotic Ectodermal Dysplasia
Hypohidrotic ectodermal dysplasia is the most common form of ectodermal dysplasia and is X-linked recessive. Females mayor may not be affected depending on which X chromosome is activated. Facies are characteristic with frontal bossing; flat malar ridges; depressed nasal root; thin upper lip; large, "pouting" lower lip; small
chin; and prominent ears. Pegged teeth are common. Periorbital wrinkling and increased pigmentation are common. Sweating is almost absent, which leads to
heat stress or fevers. Secretions from the nose, eyes, and mouth are lacking as well.
Hidrotic Ectodermal Dysplasia (Clouston Syndrome)
Hidrotic ectodermal dysplasia is an AD disorder with hair and nail hypoplasia. Additionally, there is palmarplantar
keratoderma. Sweating is normal.
Monilethrix
Monilethrix is a rare hair structure abnormality. The hairs have a beaded appearance microscopically. Hair grows 2-3 cm and then breaks off. It is due to mutations in hair keratins.
Progeria (Hutchinson-Gilford Syndrome)
Progeria is a rare AR disorder with premature aging occurring as early as 1 year of age. Growth failure is severe, and the child appears "aged." Atherosclerosis
is common by adolescence. The skin is thin, and you can easily see the veins. Total alopecia and nail dystrophy occur. Characteristically, the child has a squeaky
voice, bird-like face, and a hunched-over shuffling gait. Death occurs due to cardiovascular disease during the teenage years.
Halo nevus
occurs when a ring of depigmentation
develops around a nevus. Halo nevi require no special intervention unless atypical findings occur
Dysplastic nevi
atypical moles) appear on sun-exposed
areas. They have irregular borders with nonuniform color. They are larger than ordinary nevi, > 5 mm in diameter. A single dysplastic nevus has a low risk of malignancy, but an adolescent with multiple dysplastic nevi is at increased risk for malignant melanoma, especially if there is a relevant family history.
Spitz nevi
(spindle and epithelioid cell nevi) are
dome-shaped, red-brown to brown-black, and often occur on the face. Spitz nevi are rarely malignant.
Vitiligo
is acquired macular depigmentation It usually occurs in healthy people, but rarely, it can also be part of the AR polyglandular deficiency, in which case any of the following can occur: DM, Graves disease, Addison's/adrenal insufficiency, hyper/hypothyroidism, hypoparathyroidism, pernicious anemia, and vitiligo. If you see a patient with vitiligo and endocrine abnormalities, think of this AR deficiency!
Lichen striatus
Lichen striatus is common in childhood and presents with a linear group of small, pink to skin-colored papules that slowly flatten leaving a hypopigmented streak that resolves within several months to a few years. It is asymptomatic, self-limited, and occurs most commonly on an ann or leg
Lichen nitidus
Lichen nitidus is a benign, self-limited condition with multiple, 1-2-mm, flat-topped, skin-colored papules that
can be asymptomatic or slightly pruritic. The papules can occur anywhere on the body including the face and genitalia. Some debate whether lichen nitidus is a clinical variant of lichen planus.
Acanthosis nigricans
is hyperpigmented skin with a thickened,
velvety appearance, most noticeable in the skin folds. It rarely is familial. You tend to see it in obese patients, and it is associated with hyperinsulinernia. On exams, look for hyperpigmented skin that involves the axilla or neck of an obese child or adolescent. These patients have an increased risk of developing diabetes; thus screen for hyperglycemia, hyperlipidemia, and
hypertension. In adults, it can be associated with GI cancer
Diffuse hyperpigmentation can occur in
biliary sclerosis, sclerodenna, Addison disease, hemochromatosis (a grayish/bronze coloration), and with the use of busulfan. Other causes include primary biliary cirrhosis, porphyria cutanea tarda, malabsorption/Whipple syndrome, pellagra (niacin deficiency), B)2 deficiency, and folate deficiency.
Hyperpigmentation in sun-exposed areas is common with use of amiodarone and phenothiazines and seen with porphyria cutanea tarda. Hyperpigmentation is diffuse but darker in sun-exposed areas in pellagra, biliary sclerosis,
and sclerodenna. Methotrexate can cause a reactivation of sunbum
Pityriasis alba
is common in school-age children and
presents with areas of hypopigmentation with a fine scale. It most commonly affects the cheeks and extensor extremities. It likely represents a form of postinflammatory hypopigmentation at sites of dry skin or mild eczema and is more apparent in children with darker skin. It often becomes more readily visible in the
summer because the affected skin does not tan normally.
Keratosis pilaris
is a manifestation of the dry skin
associated with atopic dermatitis. It is characterized by many < I-mm, dry, rough, skin-colored to pink, follicular papules distributed symmetrically over the cheeks,
backs of the upper arms, and the anterior thighs. In severe cases, this condition can be more widespread. Treatment consists of avoiding irritants (dyes and perfumes)
and regular use of emollients and keratolytic agents (lactic acid, urea, topical retinoids). This condition runs in families and generally improves some with age.
Juvenile plantar dermatosis
presents with redness, cracking, and fissuring of the weight-bearing part of
the soles. It is aggravated by occlusive footwear (boots, sneakers) and can be confused with tinea pedis. Treat by applying petrolatum for lubrication and an absorbent powder. Sometimes, a moderate topical steroid is required.
Dyshidrotic eczema
(pompholyx) presents with eczema of the palms, soles, and sides of the digits. Look
for small, firm, "tapioca-like" vesicles on the lateral edges of the fingers. It is very itchy, and if fissuring occurs, it can be quite painful. Emollients and potent topical steroids are helpful
If the seborrheic dermatitis is extremely severe, consider
the possibility of
Langerhans cell histiocytosis (formerly
known as histiocytosis X), especially if atrophy, ulceration, or petechiae are present. A skin biopsy and special staining are generally diagnostic
Intertrigo
is an irritant dermatitis caused by maceration and friction and usually is found in the skin folds of obese patients. It can be secondarily infected by Candid albicans. Occasionally, an inverse subtype of psoriasis can be confused with intertrigo.
Treatment for allergic contact dermatitis consists of
cool compresses (Burow solution = aluminum acetate, 1:20), topical glucocorticoids, and emollients. If severe, such as poison ivy dermatitis
on the face, give systemic glucocorticoids for 14 days (shorter courses can lead to rebound flaring).
allergic contact dermatitis to nickel
the presence of an infraumbilical eczematous plaque
acne vulgaris
A majority of adolescents can be treated with topical medications on types:
1) Benzoyl peroxide products-which have both bactericidal and comedolytic effects
2) Antibiotics
3) Retinoids-which are particularly effective for comedonal acne

Topical therapy most often involves tretinoin (Retin-A®), benzoyl peroxide (2.5-10%), and topical erythromycin or clindamycin. Oral therapy is usually with tetracycline, doxycycline, minocycline, or erythromycin. TMP/SMX is occasionally used but carries a higher risk of serious
allergic reactions, including Stevens-Johnson syndrome and toxic epidermal necrolysis. Several oral contraceptives
are also approved for the management of acne.
Acne rosacea
Acne rosacea is seen most commonly in middle-aged patients, but it can occasionally be seen in adolescents. It presents with acne-like lesions, erythema, and telangiectasias on the central face. Even before the lesions appear, patients can have a flushing reaction to various
stimuli (alcohol, stress, exercise, heat, and sun exposure). Once the rosacea manifests, the flush can become permanent.
Rhinophyma (big nose) occurs primarily in adult patients. Treat with oral tetracyclines, topical metronidazole,
or sulfacetamide preparations
Rhinophyma
Alopecia Areata
Alopecia areata can present in 2 ways:
1) Patchy alopecia areata; regrowth potential is good.
2) Alopecia totalis (loss of all scalp hair) or universalis loss of all scalp
Trichotillomania
Trichotillomania is the compulsive pulling, twisting, or breaking of one's own hair. "Moth-eaten" appearanceis common. There are often well-defined patches of hair loss, with hairs of varying lengths, but rarely complete alopecia. The eyelashes and eyebrows can also be affected. Differentiate trichotillomania from alopecia areata, tinea capitis, and secondary syphilis. Behavioral modification and antidepressants, such as fluoxetine, can be helpful
Traction Alopecia
Traction alopecia is common in girls who wear tight ponytails or braids. Hair thinning is usually seen at the scalp margin and the temporal areas in particular. There can be associated inflammation with the development of papules or pustules along the hairline. Over time, chronic traction can cause scarring.
Telogen Effluvium
Telogen effluvium is hair loss that occurs 6 weeks to 4 months after a severe stress to the body. It can be seen following childbirth, acute fever, surgery, crash dieting, stress, thyroid disorders, with certain medications, or after stopping oral contraceptives.
Trichorrhexis Nodosa
Trichorrhexis nodosa is the most common cause of hair breakage in African-American patients. It follows chemical treatments. The hairs break off easily and may not recover for 2--4 years.
Hidradenitis suppurativa
Hidradenitis suppurativa is a chronic, inflammatory, scarring process involving apocrine glands of the axilla and inguinal region.Treatment modalities include topical
and systemic antibiotics, such as erythromycin or the tetracycline family, as well as topical benzoyl peroxides and antibacterial soaps. Severe cases can be amenable to surgical excision of the apocrine glands.
Hyperpigmented (blue-black) gingiva:
seen in Addison disease
Koplik spots:
small, white vesicles on an erythematous
base, which are found on the palate in patients with measles. These normally precede the skin lesions by several days
Hairy leukoplakia
most commonly occurs in patients
with AIDS. It appears as areas of ribbed whiteness along the sides of the tongue. This is due to EpsteinBarr virus in the superficial layers ofthe tongue's squamous epithelium
Peutz-Jeghers
syndrome (multiple intestinal
hamartomatous polyps): Rule out in patients with melanotic pigmentation (freckles) on the lips and buccal mucosa
Macroglossia
big tongue): associated with primary
amyloidosis, acromegaly, trisomy 21, congenital hypothyroidism, Beckwith-Wiedemann syndrome, metabolic storage disease, congenital vascular tumors, and congenital malformations of the tongue.
Geographic tongue"
the appearance ofmigratory, denuded red patches. It is benign and usually asymptomatic. It can be associated with psoriasis
Bald tongue":
atrophy that is associated with pellagra, iron deficiency anemia, pernicious anemia,
and xerostomia (salivary gland problems, as seen in Sjogren syndrome, lymphoma, mumps, sarcoidosis; occasionally idiopathic)
Guttate psoriasis
is common in childhood and presents
with sudden onset of many small, scaly papules and plaques on the face, trunk, and extremities. It can be induced by streptococcal pharyngitis or perianal streptococcal disease.
Erythrodermic psoriasis
an exfoliative reaction in which the entire surface of the skin becomes red, warm,
and scaly; patients are unable to control body temperature (hypo-/hyperthermia is common). Dehydration, hypoalbuminemia, and anemia of chronic disease are common sequelae
CREST syndrome
present with calcinosis cutis (small tender nodules on the fingers), Raynaud phenomenon, esophageal dysmotility, sclerodactyly (sclerosis of the fingers), and telangiectasias. CREST syndrome is rare in children.
Erythema nodosum .
consists of red, tender, warm nodules that usually appear on the shins. It is one ofthe most common types of panniculitis (inflammation of the fat). Although sarcoidosis is one of the causes of erythema nodosum, other more common causes in pediatrics include infection (TB, streptococcal, deep fungal), drugs (especially oral contraceptives,
sulfas, and penicillins), and
inflammatory bowel disease
Pyoderma gangrenosum
an inflammatory ulcer that usually occurs on the legs. It is often associated with
inflammatory bowel disease. It can also occur with rheumatoid arthritis, leukemia, monoclonal (IgA) gammopathy, and chronic, active hepatitis.
Zinc deficiency
causes a red, eczematous, sometimes erosive or vesicular rash that typically
involves the perioral, periocular, or paranasal skin, extensor surfaces of the hands/feet, and perineum/scrotum
acrodermatitis enteropathica.
This is an AR disorder with impaired absorption of zinc from the intestine.
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