Case Descriptions in Pediatric Dermatology Robin L. Hornung, MD, MPH Staff Physician, The Everett Clinic Clinical Associate Professor, Dermatology University of Washington School of Medicine

Case Descriptions in Pediatric Dermatology • I have no relevant conflicts of interest to declare • Topics to discuss: – Common “lumps and bumps” – Common rashes – Couple fun cases

Case Description • 5 yr old healthy boy with gradual appearance, over months, of several brown coalescing papules located in a linear distribution in right groin. First noted at ~1 yr of age • Papules are occasionally itchy

Linear Epidermal Nevus • Benign congenital lesion characterized by hyperplasia of epidermal structures • Can be apparent at birth or arise sometime during early childhood • Often favor the extremities, follow “Blaschko’s lines” as a result of migration of skin cells during embryogenesis • Multiple lesions may be present, sometimes in association with “epidermal nevus syndrome”

Linear Epidermal Nevus • Epidermal nevi are challenging to treat • Topical therapies: retinoids, 5-fluorouracil, and steroids • Most superficial destructive therapies are followed by recurrence: cryotherapy, dermabrasion, electrodesiccation, and laser • Deeper surgical excision • Lesions may continue to extend during childhood, Rx should be delayed

Case Description • 12 yr old girl born with a couple small slightly raised bumps on left jaw • Past year lesion has gotten bigger, with a warty pink bump now growing in it • Occasionally slightly itchy

Nevus Sebaceous (of Jadassohn) • Common congenital lesion that occurs mainly on face and scalp • Usually solitary, hairless, most often present at birth, occasionally seen during early childhood • Can also be found in epidermal nevus syndrome • Often yellowish in color due to sebaceous activity, less notable after infancy • At puberty become thicker, more verrucous, more greasy in appearance: hormonal stimulation

Nevus Sebaceous (of Jadassohn)

Nevus Sebaceous (of Jadassohn) • Multiple secondary neoplasms can occur: – Syringocystadenoma papilliferum (most common) – Apocrine cystadenoma, spiradenoma, trichoblastoma (may be mistaken for BCC) • Basal cell carcinoma previously estimated to occur in 7-50% of lesions • *Incidence may actually be very low (~1%) • Other malignancies: keratoacanthoma, SCC, apocrine carcinoma, malignant eccrine poroma *Cribier B et al. J Am Acad Dermatol 2000;42:263.

Nevus Sebaceous (of Jadassohn) • • • •

Treatment is surgical, and the timing is controversial Consider size and location Consider cosmetic (psycho-social) significance Risk-benefit ratio of general anesthesia (early in life) vs local anesthesia (later in life) • Consider any symptoms or concerns over malignant transformation

Case Description • 7 yr old boy with a firm nodule growing on mid cheek over several months, gradually becoming more pink, firm and occasionally tender • No other lesions elsewhere

Pilomatricoma • Benign tumor derived from the hair matrix • Usually present as a solitary lesion of the face/upper body during first 2 decades • Often very hard (from calcification) giving a positive “teeter-totter” sign where downward pressure one side leads other end to spring up • “Tent sign” where multiple facets (resembling a tent) noted when overlying skin stretched • Can be from 5 mm to > 5 cm

Pilomatricoma

Pilomatricoma • Usually not hereditary but familial forms been recognized, with some rarely associated with myotonic dystrophy • Multiple lesions been also seen in Gardner syndrome • Treatment is by surgical excision

Case Description • 16 yr old female with gradually growing nodule on back, occasionally tender and more swollen • Denies discharge • Small dark area in center

Epidermal Inclusion Cyst • Epidermoid cyst, epidermal cyst -- “sebaceous cyst” is an antiquated name/misnomer • Discrete slow growing nodule that may appear any time after puberty, most commonly on face, scalp, neck, trunk • A true cyst with an epithelial lining • Size varies from a few mm’s to several cm’s • Central punctum often seen, where foul-smelling keratinous debris can be extruded

Epidermal Inclusion Cyst

Epidermal Inclusion Cyst • Lesions can become inflamed, usually when cyst wall ruptures, with foreign body reaction • True secondary infection is quite rare • Can be associated with Gardner syndrome: multiple cysts, pilomatricomas, osteomas, fibromas, and intestinal polyposis • Malignant degeneration rarely reported • Rx is via surgery, getting whole cyst lining

Pilar Cyst • Pilar cysts = trichilemmal cysts • A variant of EICs, ones that are located on the scalp • Often can be multiple

Case Description • 12 year old healthy boy with gradual appearance, over months, of several tiny white papules around eyes • Lesions are not itchy or symtpomatic

Milia • Tiny epidermal cysts which tend to be multiple, 1-2 mm in size, white in color • Occur sporadically in healthy newborns, also occur in areas of abrasion trauma to skin, or in bullous disorders or wounds • Multiple lesions can occur on scrotum, leading occasionally to calcinosis cutis • In children often resolve on own in weeks - months. Needle extraction works well.

Case Description • 8 year old healthy boy with somewhat firm subcutaneous nodule under lateral eyebrow, first noted around 1-2 yrs of age • Lesion does not hurt, but been slowly growing over the years

Dermoid Cyst • A congenital dermoid sinus or dermoid cyst is a developmental epithelium-lined tract (or cyst) that extends inward from surface of the skin • Since midline fusion of dermal and neuroectodermal tissue occurs at caudal and cephalic ends of neural tube, see majority in occipital and lumbosacral regions • For midline lesions need to consider intra-cranial/CNS extension

Case Description

Dermoid Cyst • Dermoid cysts most commonly occur on orbital ridge. No association with deep extension • About 3% of dermoid cysts are located in the nasal midline (glabella, nasal dorsum, columella). Need to consider CNS extension, MRI or CT imagining must be done • Midline lesion DDX: cephaloceles, gliomas, hemangiomas, other malformations • Lesions on orbital ridge do not require imaging before surgery

Case Description • 18 year old healthy young man with a slowly growing soft mass on upper back • No associated pain

Lipoma • • • •

One of the most common benign tumors Can be seen at any age but more common after puberty Common on neck, shoulders, back and abdomen Variable size, single or multiple, soft, rubbery or puttylike consistency, usually nonpainful • Encapsulated tumors made up of adipose cells • Surgical excision usually curative • If painful consider angiolipoma

Case Description • 18 year old healthy young woman with a fairly rapidly growing firm nodule on posterior ear several months after ear piercing • No associated pain

Keloid • Benign dermal tumors, an exaggerated connective tissue response to skin injury • Rare in infancy, incidence increases throughout childhood, max between puberty and 30 yrs • Tendency also runs in families • More common in darkly pigmented individuals • Early lesions pink, smooth, rubbery, can be tender or itchy; common on earlobes, upper chest, back, and wounds under tension • Older lesions become more pigmented, firmer

Keloid • Unlike hypertrophic scars, keloids frequently persist at site of injury, often recur after excision, and always overgrow the original boundaries of the wound • Source of much psychosocial anxiety • Best therapy: multiple steroid injections (triamcinolone acetonide 20-40 mg/cc) • Excision, followed by steroid injections • Pulsed dye laser, silicone occlusive sheets, radiotherapy, IL bleomycin or fluorouracil

Case Description • 10 year old healthy girl with a persistent mass on upper arm • No associated pain • Shortly after birth had a bright red nodule develop rapidly over this area, redness has since gone away

Involuted Hemangioma

Involuted Hemangioma • Just a reminder that a significant proportion of hemangiomas leave residual skin defects after they involute!! • More common in the superficial (“strawberry”) lesions where the skin becomes so stretched and atrophic, and fibro-fatty deposits occur • Prevention of significant growth is best! Early laser, or even systemic propranolol • If significant residua, need to consider surgical excision

Early Hemangioma

Early Hemangioma

Case Description • 12 month old male with a rapidly growing red papule on right nose • Bleeds very easily and profusely, hard to stop • No tenderness, no prior trauma

Case Description

Pyogenic Granuloma • Common acquired vascular lesion of childhood • Also called lobular hemangiomas • Pathogenesis unknown, usually occur in areas of trauma: hands, fingers, arms, face • Look like small hemangiomas, bleed easily, occur in > 1 yr olds • Treatment: shave excision + electrodesiccation, pulsed dye laser in early lesions

Pyogenic Granuloma • 10 children (mean age 2.5 yrs) with facial PGs were treated QD, BID, or 3x/week until resolution • Off-label usage! • Majority of lesions resolved, 3 completely without residua, 1 progressed to surgical excision • May relate to its anti-viral, anti-tumor and immunosuppressive capabilities • Timolol has also been used in a similar trial of 10: 4 with complete response, 3 with partial, 3 with none Tritton SM, et al. Ped Dermatol 2009 26(3):269 Gupta D, et al. Int J Dermatol 2016 55(5):592

Case Description • 10 yo boy with a bright red spot on tip of nose x years, it is not going away, may be growing slightly over time • Pt is getting teased at school • Otherwise healthy

Spider Angioma • Nevus araneus: central body is an arteriole with surrounding telangiectasias • Appear most commonly on exposed areas of skin like face, arms, hands, fingers • Typically idiopathic, occurs in >15% of normal children and young adults • Some regress spontaneously • Best treatment: pulsed dye laser

Case Description

Case Description • 12 month old healthy boy with gradual appearance, over months, of several brown papules mostly over trunk, some on face • Spots are not itchy or scaly

Case Description • However, papules occasionally get swollen, red, and almost vesiculate with friction/rubbing of skin

Urticaria Pigmentosa • Considered one of the mastocytosis disorders • Characterized by accumulation of mast cells – Mastocytomas – Urticaria pigmentosa – Diffuse cutaneous mastocytosis – Telangiectasia macularis eruptiva perstans (rare) • All can have bullous variants • 90% exhibit Darier’s sign: wheal/flare with rubbing

Urticaria Pigmentosa

Urticaria Pigmentosa • Etiology not clearly understood • Growth and regulation of mast cells dependent on stem cell factor, a ligand for the protein product of the c-kit gene • C-kit mutations in adults with mastocytosis, not in kids • Familial cases have been reported

Urticaria Pigmentosa • Rarely need to biopsy for diagnosis • Mast cell mediators include: histamine, prostaglandin D2, heparin, tryptase, chymase, leukotrienes, and others • Symptoms include: itching, flushing, blistering. Rarely systemic sxs: N/V, abdominal pain, diarrhea, hypotension, pulmonary sxs • Extent of dz doesn’t correlate with systemic involvement

Urticaria Pigmentosa • Systemic dz rare in children: GI and skeletal • Most children improve by adolescence, but complete resolution not as common • Treatment: Avoid triggers!! – Physical stimuli, drugs (ASA, NSAIDs, opiates), foods • Can use antihistamines, both sedating and non-sedating, H2 antagonists, cromolyn sodium, PUVA, oral/topical steroids, EpiPen • MastoKids.org

Xerosis and Other Dry Skin Conditions

Ichthyosis Vulgaris

Keratosis Pilaris

Pityriasis Alba

Moisturizers • Goal for moisturization is to enhance the barrier function of the skin by retarding TEWL and creating environment for restoration of the stratum corneum barrier – Very important in dry skin conditions like atopic dermatitis/eczema

• Another goal may be for photoprotection • Another goal may be to exfoliate dry scaly skin • Another goal may be to repair photodamage

Moisturizers • Optimal water content of the stratum corneum is above 10% • Moisturizers raise cutaneous water content through: – Occlusion – Humectancy

Moisturizers • Occlusive moisturizers prevent evaporative water loss by placing oily substance on skin so water can’t penetrate • Many different classes: hydrocarbon oils and waxes, silicone, vegetable oils, animal oils, fatty acids, fatty alcohols, polyhydric alcohols, wax esters, vegetable waxes, phospholipids, sterols • Most effective is petrolatum as reduces TEWL by 99%

Moisturizers • Humectants are substances that attract moisture, can only hydrate the skin from the environment when ambient humidity is >70% • Includes glycerin, honey, sodium lactate, urea, propylene glycol, sorbitol, gelatin, hyaluronic acid • Humectants also improve smoothness • Most moisturizers contain both occlusive and humectant ingredients

Moisturizers • Newer “physiologic moisturizers” can replace lost lipids, reduce transepidermal water loss, and calm inflammation associated with conditions like eczema • Focus on lipid replacement therapy, particularly ceramides, to restore normal balance of the barrier

Moisturizers • Ceramide-dominant moisturizers permeate the stratum corneum and are processed in lamellar bodies, and secreted back into the stratum corneum to become a part of the matrix • Triceram and Epiceram first ones, then CeraVe Cream became the first OTC ceramide cream

Moisturizers • Best time to apply moisturizers to hydrate the skin is right after bath time!

• Best to avoid soaps as much as possible in dry or dermatitic skin conditions!

Case Description • 15 year old girl with numerous itchy erythematous papules over legs (some on arms) • Also with follicular pustules • Started shortly after rash in axillae • History of atopic dermatitis

Case Description

Folliculitis • Refers to an infection of hair follicles • Clinical appearance varies according to location and depth of follicular involvement • Usually begins with yellow-white follicular pustules, often erythematous • Often seen on buttocks and extremities • Often occur in crops and heal with post-inflammatory hyperpigmentation

Folliculitis

Folliculitis

Folliculitis • Staphylococcus aureus most common offender, but Strep and gram negatives can be seen • In immunocompromised children can see commensals such as Pityrosporum yeast or Demodex mites • Rx usually with topical antibiotics (clindamycin lotion a good one for very dry skin), systemic if extensive, antibacterial soaps • Stop auto-inoculation (such as shaving of legs) • Consider culture if unusual or resistant

Case Description • 20 yr old healthy young man developed intensely itchy red bumps on his wrists, hands, ankles and feet x months • Itch often kept him awake at night

Case Description

Case Description

Case Description

Scabies Infestation • Caused by Sarcoptes scabiei, the human mite • Obligate parasites of all developmental stages burrow in the epidermis, laying eggs, depositing feces • Adult female lifespan 15-30 days, lays 1-4 eggs/day • Eggs hatch in 3-4 days, mature into adults 10-14 days • Transmitted by direct contact, sometimes fomites • Survival off the human host is only ~3 days • Average incubation before symptoms is ~3 weeks

Scabies Infestation • Initial symptom typically is pruritus, often worsening at nighttime • Papules, nodules, burrows, vesiculopustules • Interdigital spaces, wrists, ankles, axillae, waist, groin, in infants also on the head, palms and soles • Scabies nodules are red-brown nodules representing a vigorous hypersensitivity response, may last x months • Crusted scabies occurs in the immune compromised, highly contagious!!!

Scabies Infestation • Crusted scabies occurs in the immune compromised, such as HIV, or physically incapacitated • Lesions may mimic eczema, psoriasis or warts, sometimes get nail dystrophy • Can be minimally pruritic • Highly contagious!!! Often the source of large epidemics, can carry thousands of mites • Often there is a delay in diagnosis

• Diagnosis is made via skin scrapings under mineral oil immersion • Sometimes need to scrape parents if child uncooperative • See mites, eggs, feces

Scabies Infestation • Treatment includes permethrin 5% cream applied neck down for 8-14 hours, followed by rinsing • A 2nd treatment 1 week later is often recommended • In infants it should also be applied to scalp and face • Apply well under finger and toenails too • Warning: Sx’s may not clear for several weeks! • Ivermectin (off-label) extremely effective Rx: 150200 mcg/kg/dose, can repeat in 2 weeks • Treatment of all close contacts too!

Scabies Infestation • Environmental decontamination: clothing, bed linens, and towels should be machine washed in hot water and dried using a high heat setting • Items that can’t be washed (stuffed animals) may be dry cleaned or stored in bags for 1 week, as the mite will die when separated from the host • Topical steroids and anti-histamines can be used for symptom relief • Warn that nodules can take weeks or months to heal

Case Description • 17 yr old female on a trip to Europe started waking up with groups of itchy red welts • She was otherwise very well • Her trip included stays in youth hostiles

Bedbug Infestation • Caused by Cimex lectularius • Red-brown wingless blood-sucking nocturnal insects that are 3 - 5 mm in size • Female deposits eggs on rough surfaces, cracks, crevices • Avoid light by hiding during the day, then respond to warmth and carbon dioxide at night (sleeping human) • Occur on any exposed areas of skin like face, neck, arms, hands • Can survive without blood for 6 - 12 months!

Bedbug Infestation

Bedbug Infestation • Treatment is directed at elimination of bugs with insecticides as well as potential hiding sites • Can look for blood stains on your sheets or pillowcases, dark rusty spots of excrement, egg shells, or shed skins on sheets and mattresses, bed clothes, and walls • An offensive, musty odor from the bugs' scent glands • Call an exterminator! • For symptom relief can use topical steroids and antihistamines

Case Description • 5 yr old boy with recurrent itchy bumps on legs, bot also areas on trunk, arms • They have one cat and one dog, had fleas in the past but they are treated

Flea Infestation • Caused by Pulex irritans (human flea), Ctenocephalides canis and felis (dog and cat flea) • Leave urticarial wheals or papules, often with a central hemorrhagic punctum, can see tense vesicles or bullae • Classic “breakfast, lunch, and dinner” sign for crawling or hopping insects • Often only one individual in household will be primarily affected, the “tasty one”!

Flea Infestation

Flea Infestation • Treatment is directed at elimination of bugs with treatment of suspected animal carriers • Do not forget cleaning and spraying of carpets, floors, crevices, and other potentially infested areas • For every flea seen on pet, there are many more in the environment • Flea collars not completely effective, sprays and powders must be repeated every 2 weeks in summer • Potent topical steroids will help resolve individual lesions and symptoms

Case Description • 15 yr old boy developed a very itchy rash on arm, appearing as multiple papules (some vesicles) coalescing into linear streaks • Otherwise healthy • Went hiking in mountains several days earlier

Contact Dermatitis • Can be due to irritant contact dermatitis • Can be a true allergic contact dermatitis • Rhus family of plants most common (eg. Poison ivy) • Nickel very common in kids • Can see id reaction = autosensitization

Allergic Contact Dermatitis

Allergic Contact Dermatitis • Rx for allergic contact derm is to identify and remove the contact allergen, eg: nickel, plants, preservatives, etc. • Rx • Topical steroids are usually mainstay of therapy • PO steroids severe cases • Consider patch testing

Irritant Contact Dermatitis

Irritant Contact Dermatitis • Rx for irritant contact derm is to stop irritants (eg, excessive hand washing, diaper irritants) • Increase moisturization for barrier repair • Topical steroids can be used when significant inflammation

Case Description • 8 yr old girl with recurrent dermatitis of the lips, often irritated • Eats mangoes on occasion

Lip Licker’s Dermatitis • Irritant contact dermatitis • DDX: allergic contact dermatitis, atopic dermatitis • RX: – frequent moisturizing with thick emollients (Aquaphor), – low-strength topical steroids BID-TID – stop licking!!

Unknown

Unknown

Unknown

Have A Nice Day!!