CURRENT ESSENTIALS PEDIATRICS - part 6 ppsx

Chapter 13 Dermatology 255 Seborrheic Dermatitis ■ Essentials of Diagnosis • Erythematous, greasy, scaly dermatitis in the sebum-producing areas of face, scalp, intertriginous folds, and perineum • Affects mainly newborns (scalp and intertriginous areas) and ado- lescents (face, intertriginous areas, perineum) • Pityrosporum ovale (yeast organism) often found in affected areas. Possibly pathogenic • Leiner disease—severe, widespread, infantile seborrheic dermatitis associated with functional defect in C5 complement. Wasting, diarrhea, failure to thrive, superimposed infections • Visual diagnosis is key. Skin biopsy findings are nonspecific ■ Differential Diagnosis • May resemble atopic dermatitis of the scalp in infants • Psoriasis occurs in seborrheic areas in older children • Pityriasis rosea—herald patch and distribution should distinguish • Tinea (dermatophytoses) infections ■ Treatment • Low-potency topical corticosteroids usually suffice • Leiner disease may require therapy with fresh frozen plasma (FFP) • Disease severity is usually worse in adults than children ■ Pearl Seborrhea (cradle cap) is not treated the same as dandruff in adults. Vigorous scrubbing of the scalp, adult dandruff shampoos, and picking the scales may damage the infant’s delicate skin and increase the risk of infection. A little warm olive oil to loosen scales followed by gentle shampoo is a time-honored, harmless therapy. 13 256 Current Essentials: Pediatrics Pityriasis Rosea ■ Essentials of Diagnosis • Papulosquamous eruption of school-aged children with small, red oval plaques with fine scales often aligned parallel to skin tension lines • In 20–80% , the generalized rash is preceded for up to 30 days by a single larger scaly plaque with central clearing (herald patch) often on the trunk (in whites) or extremities (in blacks) • Human herpes virus 7 may be a pathogen • Lasts about 6 weeks with mild-moderate pruritus • History and visual diagnosis are key. Biopsy may be required in confusing cases ■ Differential Diagnosis • Herald patch looks like ringworm • Secondary syphilis should be ruled out in high-risk situations, especially if palms and soles are affected. Obtain VDRL • Psoriasis • Lichen planus • Dermatomyositis, systemic lupus erythematous (SLE) ■ Treatment • Exposure to sunlight may hasten resolution • Oral antihistamines for pruritus • Spontaneous resolution is expected ■ Pearl If pityriasis rosea lasts >12 weeks, refer to dermatologist for more in- depth evaluation of alternative diagnoses. 13 Chapter 13 Dermatology 257 Psoriasis ■ Essentials of diagnosis • Erythematous papules covered by thick white scales • Guttate psoriasis (3–8 mm, drop-like papules with pearly white covering) is the common form of childhood often occurring 3–4 weeks after streptococcal pharyngitis • Chronic psoriasis—larger plaques over elbows, knees, scalp, and sites of trauma. Pinpoint pits in nails and yellow discoloration from onycholysis • Other forms—pustular, acral, follicular are rarer in children • Family history often positive. Underlying cause unclear. May be T-cell–mediated release of cytokines which stimulates ker- atinocyte proliferation • Visual diagnosis is usually accurate. Biopsy shows hyperkeratosis, parakeratosis, acanthosis, epidermal hyperplasia, and peri- capillary inflammatory infiltrate ■ Differential Diagnosis • Other papulosquamous eruptions—pityriasis rosea, tinea corporis, lichen planus, dermatomyositis, lupus, secondary syphilis • Lesions in infants may look like diaper dermatitis • Drug dermatitis especially β-blocker (practolol) ■ Treatment • Topical steroids initial treatment of choice. High-potency preparations required to penetrate skin. Guttate psoriasis usually responsive • Topical calcipotriene (vitamin D derivative) with steroids used in severe cases but hypercalcemia may complicate therapy • Topical skin and scalp preparations contain retinoids, anthralin, coal tar, tar gels for exfoliation • Topical anticytokine therapy has been used in some patients— etanercept, infliximab ■ Pearl Scraping the scales of a psoriatic lesion with the edge of a glass slide leaves minute bleeding points. This may help differentiate from seborrhea. 13 258 Current Essentials: Pediatrics Erythema Multiforme ■ Essentials of Diagnosis • Hypersensitivity reaction involving dermal vascular endothelium following medication, infection, or other illnesses • Sudden-onset, wide distribution (legs, arms, palms, hands, feet, face, and mucous membranes often with truncal sparing). Initial red macules, papules, or blisters gradually enlarge developing concentric rings of pallor and erythema (target lesions) • Skin usually heals spontaneously over 2–8 weeks. Common pre- cipitants are sulfonamides, penicillins, barbiturates, phenytoin, Herpes simplex, Mycoplasma, and many other infections ■ Differential Diagnosis • Stevens-Johnson syndrome—very extensive erythema multiforme rash with mucous membrane and conjunctival involvement, fever, malaise, and arthralgia • Toxic epidermal necrolysis—erythema multiforme with large bullae and epidermal sloughing • Fixed drug eruption—anticonvulsants, nonsteroidal anti- inflammatory drugs (NSAIDs), sulfonamides cause a variety of skin responses that may resemble erythema multiforme • DRESS syndrome—fixed drug eruption with fever, eosinophilia, and systemic symptoms usually after anticonvulsants ■ Treatment • Conservative measures—cool, moist compresses, antihistamines, antipyretics, mild analgesics • Topical anesthetics to reduce dysphagia and dysuria • Prophylactic acyclovir prevents recurrence of rash during herpes reactivation • In severe Stevens-Johnson, dehydration, shock, electrolyte imbal- ance, superinfection, and hypoproteinemia from skin slough may require intensive care unit admission • Intravenous immunoglobulin (IVIG) has been used to prevent progression of rash in Stevens-Johnson syndrome ■ Pearl Corticosteroids are not effective in treating or preventing erythema multiforme. 13 Chapter 13 Dermatology 259 Erythema Nodosum ■ Essentials of Diagnosis • Reactive inflammation of subcutaneous fatty tissue causing single or multiple tender red/violaceous lumps usually over the shins. Resolving nodules look bruised • Occurs in isolation but often after medications (oral contraceptive pills, estrogens, sulfa), infections (strep throat, Yersinia, tubercu- losis, mycoplasma, cat scratch, fungal infections, Epstein-Barr virus, inflammatory bowel disease, autoimmune hepatitis • Resolution usually occurs in 6 weeks but chronic/recurrent disease occurs • Visual diagnosis usually sufficient. Biopsy showing septal inflammation in fatty layers is diagnostic ■ Differential Diagnosis • Nodular vasculitis • Fat necrosis—with corticosteroid therapy and severe pancreatitis • Panniculitis—with SLE, cold injury • Henoch-Schönlein purpura • Scleroderma or dermatomyositis may cause firm dermal patches that resemble erythema nodosum • Factitious lesion or child abuse ■ Treatment • Most lesions resolve spontaneously or with treatment of underlying condition • Anti-inflammatory medications and corticosteroids (oral or local injection) if lesions are very painful or extensive • Corticosteroids may be contraindicated with underlying infection ■ Pearl A rare form of erythema nodosa in childhood affects only the palms or soles. 13 13 Epidermolysis Bullosa (EB) ■ Essentials of Diagnosis • Inherited bullous disorder with intraepidermal blister formation in response to mechanical trauma. 4 major histologic types—EB simplex (92%), junctional EB (1%), dystrophic EB (5%), and hemidesmosomal (<1%) with many variants • Mutations in genes for keratins 5 and 14 (simplex), laminin (junctional), type VII collagen (dystrophica) produce fragility at vari- ous sites in the epidermal basal layer with separation and blistering • EB simplex—usually mild presentation in infancy with hemor- rhagic blisters on legs especially with walking • Junctional and dystrophic forms present at birth with variable, often widespread blistering and slough. Complications include infection, fluid and electrolyte abnormalities, malnutrition, dysphagia, esophageal strictures (mostly dystrophic EB), limb contractures, syndactyly (recessive dystrophic EB), oral contractures, and facial scarring • Biopsy needed to clearly define the epidermal structures affected in each subtype ■ Differential Diagnosis • Some mild patients with EB simplex only have palmar and plantar dyshydrosis • Epidermolytic hyperkeratosis may look like EB at birth ■ Treatment • Most EB simplex patients are not severely affected. Protect skin from injury, especially hands, feet, knees, and elbows. Reduce skin friction points with 5% glutaraldehyde every 3 days. Skin cool- ing can prevent blisters • Treatment of junctional and dystrophic types is supportive— protect skin from injury, daily wound care with nonstick dressings, treat infections, physical therapy, nutritional support, emotional support • Monitor for complications—highly malignant squamous cell car- cinoma is a late complication of recessive dystrophic EB • Gene therapy directed at the collagen VII, laminin, and keratin genes has been attempted in experimental models ■ Pearl Autosomal recessive junctional EB (Herlitz or EB letalis) is the most severe EB subtype with blistering and slough of epithelia of skin, gas- trointestinal (GI), respiratory, eye, and urinary tract causing inanition, infection, and early death. 260 Current Essentials: Pediatrics Chapter 13 Dermatology 261 Ichthyosis ■ Essentials of Diagnosis • Inherited disorder with excessive scaliness of skin • Ichthyosis vulgaris—most common form with variable severity. Scaly dry skin on trunk, abdomen, legs, and buttocks; deep palmar and plantar creases. Autosomal dominant defect in filaggrin • X-linked ichthyosis—thick scales sparing palms and soles, corneal opacities in patient and carrier mother. X-lined defect in choles- terol sulfatase • Epidermolytic hyperkeratosis—rapid epidermal turnover. Skin red, moist, and blistered at birth with subsequent warty scaling especially in flexural areas, palms and soles. Autosomal dominant defect in keratins 1 and 10 • Lamellar ichthyosis—autosomal recessive defect in transglutam- inase 1 with erythroderma, ectropion, rapid epidermal turnover, large skin scales, thick palms and soles. Sepsis, dehydration, dyshydrosis, heat intolerance, corneal damage are complications • Visual diagnosis confirmed by skin biopsy, genetic testing, and enzyme analysis ■ Differential Diagnosis • Atopic dermatitis—occurs in isolation but often complicates ichthyosis vulgaris • Psoriasis • KID syndrome—keratitis, ichthyosis, deafness, and sometimes notched teeth • Sjögren-Larsson syndrome—ichthyosiform dermatitis, spastic diplegia, retinitis pigmentosa, and mental retardation • Human immunodeficiency virus (HIV) patients may develop acquired ichthyosis ■ Treatment • Medications to control scaling (lactic acid with ammonium hydroxide, salicylic acid, topical retinoic acid lubricants), avoid- ance of injury • Topical products must be used with caution because of skin absorption • Treat associated atopic dermatitis • Protect eyes in lamellar ichthyosis—artificial tears, protective covering during sleep, surgery for ectropion may be required • Antibiotics in infants with epidermolytic hyperkeratosis • KID syndrome may respond to topical cyclosporine ■ Pearl Newborns with lamellar ichthyosis may be completely encased in a col- lodion membrane, which sheds at 10–14 days of age revealing the erythematous skin condition. 13 262 Current Essentials: Pediatrics Milia ■ Essentials of Diagnosis • 1–2 mm benign, keratin-filled epidermal cysts on the face of 40% of normal newborns • Cysts arise in immature or obstructed sebaceous glands around and on the nose (neonates) and around the eye (children and adults). Small vesicles may be present • Intraoral counterpart seen in 60–85% of neonates (Epstein pearls, Bohn nodules) • Secondary milia occurs in areas of skin trauma—dermabrasion, burns, EB, lichen planus, porphyria cutanea tarda, strong topical steroids • Visual diagnosis is sufficient in neonates. Secondary milia requires diagnostic evaluation. Biopsy rarely needed ■ Differential Diagnosis • Neonatal acne • Sebaceous gland hyperplasia of neonates due to maternal androgens • Miliaria—obstruction of eccrine sweat ducts in neonates cause tiny grouped vesicles or red papules in intertriginous areas and con- tiguous skin • Pustular melanosis—neutrophil containing pustules leave pig- mented macules upon spontaneous rupture ■ Treatment • No treatment required in neonates. Cysts usually rupture and dis- appear in the first month • Identify and treat causes of secondary milia ■ Pearl Vigorous washing of these “whiteheads” in neonates is not helpful and may worsen the condition. 13 Chapter 13 Dermatology 263 Erythema Toxicum ■ Essentials of Diagnosis • Benign rash in 50% of term newborns onset 24–48 hours • 2–3 cm blotchy red macules on chest; also back, face, and extremities • May be up to 100 lesions • Macular erythema may fade or may progress to urticarial wheals or pustules filled with eosinophils. Blood eosinophilia up to 20% ■ Differential Diagnosis • Intrauterine infection (rubella, cytomegalovirus [CMV], toxo)— rash usually present at birth with purpuric component • Postnatally acquired herpes simplex usually associated with severe illness and vesicular lesions. Neonatal varicella • Pustular melanosis—may appear on palms and soles • Insect bites • Bacterial pyoderma ■ Treatment • Spontaneous resolution usually in 5–7 days but may last for 6 weeks ■ Pearl The cause of this condition is unknown. The presence of eosinophilic pus suggests an allergic reaction to something. Oddly, the longer the length of labor in vaginal deliveries, the higher the incidence and duration of erythema toxicum. Go figure. 13 264 Current Essentials: Pediatrics Pigment Cell Skin Lesions ■ Essentials of Diagnosis • Mongolian spot—blue-black macule over lumbosacral area (occasionally over shoulders and back) in infants of Native American, African American, and Asian descent. Migratory defect in neural crest melanocytes. Benign and fades with time • Café au lait macule—light brown oval macule found anywhere on body in 10% of white and 22% of black children. >6 macules >1.5 cm may indicate NF-1. Patients with McCune-Albright syndrome have large single irregular macules • Spitz nevus—red brown solitary benign nodule consists of bizarre pigment producing cells with many mitoses • Acquired melanocytic nevus—common mole. Increases with age. Clones of melanocytes at dermo-epidermal junction. Benign • Melanoma—rare before puberty. Malignant collections of melanocytes. Local excision and pathologic examination of sus- picious lesions especially with ulceration and bleeding is essential • Congenital melanocytic nevus—larger and darker than acquired nevi and may contain hairs. A nevus covering >5% of body sur- face is called a giant nevus. 1–5% may become malignant ■ Differential Diagnosis • A Mongolian spot may be mistaken for bruising and raise the suspicion of child abuse • Epidermal nevi may be brown, resembling common moles ■ Treatment • No therapy required for Mongolian spots, café au lait macules, Spitz nevus, or common moles • Congenital melanotic nevi should be monitored for change in size or coloration, which may indicate malignant transformation • Melanoma in children with congenital melanotic nevus may develop in areas other than skin • Surgical removal of large acquired or congenital moles for cos- metic reasons may be considered but scarring and keloid may result ■ Pearl The presence of hair in a mole does not carry any greater risk of malig- nancy. Hairs are found in common moles as well as congenital melanocytic nevi. 13 [...]... interferon- -2 a, pulsed dye laser • Life-threatening cystic hygromas treated with injection of picibanil or surgery • Port wine stains treated with dye laser • ■ Pearl Kasabach-Merritt syndrome (platelet trapping with consumptive coagulopathy) does not occur in solitary cutaneous hemangiomas It occurs only with internal hemangiomas, hemangioendotheliomas, and tufted angiomas 13 266 Current Essentials: Pediatrics. .. slim, growth factors (possibly transforming growth factor-a [TGF-a]) released from malignant cancers may provoke AN In a nonobese child with sudden-onset AN, consider this rare possibility Chapter 13 Dermatology 267 Impetigo ■ Essentials of Diagnosis Initial pruritic papule that vesiculates and then ruptures leaving denuded area covered by honey-colored crust Often perioral or in nasolabial folds •... vasoconstrictors, nonsteroidal anti-inflammatory drugs (NSAIDs), and corticosteroids occasionally indicated • Chlamydia—in neonates, systemic and topical erythromycin • • ■ Pearl Vernal conjunctivitis is a seasonal allergic conjunctivitis Dramatic cobblestone changes on the superior palpebral conjunctiva help confirm the diagnosis 272 Current Essentials: Pediatrics Hyphema ■ Essentials of Diagnosis Blunt... corticosteroid • ■ Pearl Although the optic discs in papillitis and papilledema may look the same, papillitis is usually unilateral and papilledema is almost always bilateral 2 76 Current Essentials: Pediatrics Orbital and Periorbital Cellulitis ■ Essentials of Diagnosis • • • • • ■ Differential Diagnosis • • 14 ■ Severe conjunctivitis—viral, bacterial, or allergic Trauma or foreign body Treatment • • • ■ Periorbital... dense cataracts or severe ptosis 278 Current Essentials: Pediatrics Hordeolum (Stye) ■ Essentials of Diagnosis Staphylococcal infection of the sebaceous glands of Zeis or Moll in the upper or lower eyelid • Infection of the meibomian glands on the internal aspect of tarsa are also called styes • Warmth, tenderness, erythema, and local swelling at edge of lid • Recurrent infections common • ■ Differential... ingestion If drinking water contains . gentle shampoo is a time-honored, harmless therapy. 13 2 56 Current Essentials: Pediatrics Pityriasis Rosea ■ Essentials of Diagnosis • Papulosquamous eruption of school-aged children with small,. skin, gas- trointestinal (GI), respiratory, eye, and urinary tract causing inanition, infection, and early death. 260 Current Essentials: Pediatrics Chapter 13 Dermatology 261 Ichthyosis ■ Essentials. completely encased in a col- lodion membrane, which sheds at 10–14 days of age revealing the erythematous skin condition. 13 262 Current Essentials: Pediatrics Milia ■ Essentials of Diagnosis •