Pediatric Dermatology Digital Lecture Series : Chapter 25 Dr. D. Sudha Vani M.D., Assistant Professor Department of DVL Gandhi medical college, Hyderabad

CONTENTS Importance Difference between adult and neonatal skin Classification Physiological Acquired Genetic / nevoid Neoplastic Adolescent dermatoses Child abuse Principles of treatment MCQs Photo quiz

Importance of Pediatric Dermatology Approximately 30% of patients in dermatology practice are children. Most of the times, diagnosis is based on clinical examination alone. Early diagnosis and treatment is necessary to reduce disease burden on parents. Good clinical examination and high index of suspicion are mandatory.

Differences in adult and neonatal skin Adult Skin Neonatal Skin Surface Dry Vernix (gelatinous) Full thickness 2.1 mm 1.2 mm Epidermal thickness More than 50µm 40-50 um 20-25 µm in premature DEJ Good cohesion Less cohesion Dermis Normal Less collagen & elastic fibers Melanosomes Fewer Hair Vellus & Terminal Lanugo & Vellus Permeability & Absorption Good resistance Increased

Classification of Pediatric Skin Diseases Physiological Acquired Infective disorders : Bacterial, Viral, Fungal Infestations: Scabies, Pediculosis, Parasitic Inflammatory / Immunological disorders: Eczemas, Psoriasis, Lichen planus, Bullous disorders Genetic / Nevoid disorders : Hemangiomas, Linear epidermal nevus, Epidermolysis bullosa, Tuberous sclerosis, Neurofibromatosis, etc. Neoplastic disorders : Langerhans cell histiocytosis, Mastocytosis

Physiological Neonatal Dermatomes Vernix caseosa Sebaceous hyperplasia Mongolian spot Physiological scaling Sucking blisters Cutis marmorata Physiological jaundice Miliaria Milia Neonatal Acne Cradle cap Erythema toxicum neonatorum Transient neonatal pustulosis

Vernix Caseosa White, waxy, lipophilic substance covering the newborn skin. Composition- protein, lipids and water. Provides hydrophobic layer of protection and has antimicrobial and antioxidant properties. Absent in neonates born more than 42 weeks.

Vernix Caseosa

Sebaceous Hyperplasia Small yellow-white papules on the nose Thought to be as a result of hyperandrogenic state Resolves spontaneously

Mongolian spot Seen at birth - Single/multiple, slate gray/blue macules of size 3 to 10 cms Sites: lumbosacral region, buttocks, shoulders Fade within the first 3-4 years of life Delay in migration of melanocytes from neural crest to dermo -epidermal junction.

Physiological scaling Seen in 75% normal infants occurring in 1st week of life. First around the ankles, later on hands and feet and soon becomes generalized. Maximum intensity by eighth day, subsides by 3-4 wks. Sucking Blister One or two solitary blisters present at birth and seen over fingertips/hands/forearm. Caused by vigorous sucking. Skin becomes fragile due to weak dermo-epidermal attachment. Heals rapidly without treatment within 2 weeks of life. DD : Herpes simplex, Bullous impetigo, Epidermolysis bullosa.

Physiological / Neonatal Jaundice Cutis marmorata Benign cutaneous vascular lesions characterized by reticulated bluish mottling of the skin. Accentuated by cold temperatures. Seen on trunk and extremities. Physiological / Neonatal Jaundice Normal phenomenon seen in 1st wk of life. Initially starts on face around nose. Bronze Baby Syndrome : associated with phototherapy in children.

Miliaria Superficial vesicles due to obstruction of sweat ducts Miliaria crystallina : Obstruction at level of S. Corneum Following fever, hot humid weather, phototherapy Tiny clear vesicles seen over forehead, neck, trunk Miliaria rubra (prickly heat) Obstruction deeper in the epidermis Seen in hot weather Non follicular papules on an erythematous base 1 to 4 mm in diameter on trunk, face, flexures Usually subside in 2-3 days

Miliaria Miliaria pustulosa : Papulo-vesicular lesion that becomes a sterile pustule Miliaria profunda Obstruction at DEJ Leakage of sweat into dermis Advise : Cold water baths, remain in cool areas, avoid occlusive clothing Treatment : Calamine lotion, talcum powder Antibiotics in secondary infection.

Milia Idiopathic small superficial keratin filled cysts affecting all age groups. Resolves spontaneously in newborns. Epstein Pearls : Milia on palate of newborn Neonatal Acne Seen in first 3 months of life. Transplacental transfer of maternal hormones. Non-scarring type, resolves spontaneously

Cradle Cap Seborrhoeic dermatitis of scalp. Thick, greasy, adherent scales on scalp. Also seen over the ears, trunk and intertriginous areas. Usually begins in first 3 months and is self limiting. Application of oil for few hours to soften scales, 1% hydrocortisone cream can be used. .

Erythema Toxicum Neonatorum Benign, idiopathic self limiting eruption Characterized by papules and pustules on blotchy erythema Seen in 1st wk of infancy and resolves spontaneously within 1-2 wks. Present all over the body more so on buttocks and back

Transient Neonatal Pustular Melanosis Idiopathic eruption within 1st day of life. Sterile fragile pustules seen over forehead, chin, neck lower back and shins. Treatment not required.

Diaper Dermatitis (Napkin Rash) Most common Irritant dermatitis in the perineal region of neonates due to occlusion, friction, prolonged skin contact with urine, feces and fabrics. Wetness leads to maceration of skin and secondary infection with candidiasis. Intertriginous areas are invariably spared. DD : Seborrheic dermatitis, candidiasis, Psoriasis, Intertrigo Treatment : Cleansing Hydrocortisone cream twice daily Zinc oxide & Petrolatum are used as barrier creams

Infantile Naevi and Childhood Dermatoses Infective, inflammatory diseases and childhood eczemas have been discussed in respective chapters. Certain common and genetic-naevoid conditions seen in infants and children will be discussed.

Congenital Melanocytic Nevus Present at birth 1% of infants are born with CMN Small - <0.5cm, Moderate - <20cm Large - >20cm (1 in 20,000) Giant CMN - >50cm, also called “Bathing trunk nevus” Verrucous, hairy, nodular having potential to become Melanomas. Treatment : Serial surgical excision

Congenital Melanocytic Nevus

Linear Epidermal Nevus Hamartomas of embryonal ectodermal origin. Seen at birth or early childhood as a linear raised warty lesion. Located over head, neck, trunk & extremities. Treatment: Surgical excision with grafting, Radiotherapy, Cryotherapy, CO2 Laser. Retinoic acid and Calcipotriol.

Nevus of Ota Unilateral patch of bluish discoloration (dermal melanocytosis) in the periocular area along ophthalmic and maxillary divisions of the trigeminal nerve. Sites : periobital area, sclera, conjunctiva, temple, forehead, malar area, nose. When it appears on shoulder - Nevus of Ito.

Nevus Depigmentosus Single, well circumscribed, hypopigmented or depigmented macule or patch seen at birth. Usually stable in size and distribution Sites : trunk and proximal extremities with serrated edges.

Nevus Anemicus Becker’s naevus Usually congenital, single or multiple hypopigmented patches blanchable on diascopy. Due to localized increased vascular response to catecholamines. Becker’s naevus First appearance is in adolescence and more common in males Irregular hyperpigmented smooth macule, develops coarse hair later Sites : shoulder, anterior chest or scapular region. Lasers may be used for cosmetic benefit.

Haemangiomas Most common vascular nevi of childhood. Incidence more in preterm infants with female predilection beginning at one month of age. Lesions undergo a proliferative phase followed by stabilization and eventual spontaneous involution. Complications Ulceration, bleeding, Secondary infection, Mutilation and scarring, Cosmetic disfigurement. Treatment Systemic Corticosteroids, propranolol, surgical excision and lasers.

Strawberry Haemangioma

Vascular malformations Stable dilatations of superficial or deep vasculature. Present at birth or any any age. Can be capillary, arterial, venous, lymphatic or mixed. Clinical types Salmon patch Port-wine stain Sturge-Weber syndrome Klippel-Trenaunay syndrome

Salmon Patch (Stork Bite) Present in 30 to 40% of neonates. Superficial, red or pink flat lesions. Seen over forehead, upper eyelid, glabellar area, nape of neck. Resolution in first year of life.

Port-Wine Stain (Nevus Flammeus) Present at birth Common sites are face, neck and mucous membrane Flat pink-red lesion Sharply unilateral in distribution, may develop nodules with age Persists and becomes darker in adulthood Complications : Glaucoma, Choroidal angiomas Treatment : Flash pulsed dye laser

Portwine Stain

Sturge-Weber Syndrome Port wine stain in distribution of ophthalmic branch of trigeminal nerve . May be associated with seizures, ipsilateral glaucoma, behavioural problems and mental retardation. Characteristic intracranial S-shaped calcifications.

Neurofibromatosis (NF) Autosomal dominant disorder. Benign growths of peripheral nerve sheaths. Riccardi classified NF into eight distinct clinical types in 1982. Affects skin, soft tissue, nervous system, bone and other organs. Classical skin lesions are Café au lait macules and neurofibromas.

Neurofibromatosis - 1 (Von Recklinghausen’s disease) Diagnostic criteria for NF-1 Presence of two or more of the following: Six or more café au lait macules: >0.5 cm in children >1.5cm in adults Two or more neurofibromas of any type or 1 plexiform neurofibroma. Axillary or inguinal freckling. Two or more Lisch nodules (brown coloured small nodules on iris surface). Optic glioma. A distinctive osseous lesion. A first-degree relative with NF-1.

Neurofibromatosis

Neurofibromatosis - 2 Bilateral acoustic neuromas Multiple CNS tumors Few café au lait macules Few neurofibromas No axillary freckling No Lisch nodules Juvenile posterior subcapsular cataracts Treatment : Multi- disciplinary management surgical excision lasers

Tuberous sclerosis (Bourneville’s disease) Syn. EPILOIA (Epilepsy, Low IQ, Adenoma sebaceum) AD inheritance Skin Lesions: Ash leaf macules / hypopigmented macules. Angiofibroma (adenoma sebaceum) begins at 2-5 years of age as small pink papules on mid-face. Shagreen’s patch (yellowish brown plaque on lumbo - sacral area). Koenen’s tumors (periungual fibroma) Mental retardation, Seizures Treatment : angiofibromas with lasers and electrodessication.

Tuberous sclerosis (Bourneville’s disease) Angiofibromas Ash leaf macule

Ichthyosis Inherited disorder of keratinization. Characterized by the accumulation of scales on the skin surface, dry skin. Fish like scales most prominent over the trunk, abdomen, buttocks and legs. May be associated with ectropion, eclabion, nail dystrophy, internal organ involvement in few cases. Types : Ichthyosis vulgaris: most common X-linked ichthyosis Lamellar ichthyosis Collodion baby / Harlequin fetus

Lamellar Ichthyosis

Xeroderma pigmentosum Photosensitivity, oculocutaneous pigmentation and early neoplasia resulting from abnormal DNA repair. Autosomal Recessive. Multiple freckles, atrophic hypo-pigmented macules over photo-exposed skin. Prone to malignancies BCC, SCC, malignant melanoma & systemic malignancies. Treatment : strict photo-protection, eye protection.

Epidermolysis Bullosa Inherited bullous disorders characterized by non-inflammatory blister formation at sites of trauma. Variable severity depending on genetic defect. Types : Simple, Junctional, Dystrophic. Some subtypes, especially the milder EB forms, improve with age. Autosomal recessive types have bad prognosis with severe mucosal, esophageal involvement and atrophic scarring of skin. Treatment : Prevention of trauma, wound care.

Epidermolysis Bullosa

Ectodermal dysplasia Autosomal X linked recessive is most common. Characterised by defects in hair, teeth, nail and sweat glands. Hypohidrotic ectodermal dysplasia: Sparse hair, decreased sweat and absent dentition. Risk of hyperthermia in warm weather

Adolescent Dermatoses Acne Dandruff Striae Pseudo-acanthosis nigricans Contact dermatitis to cosmetics, perfumes, artificial jewellery / accessories (metals) Hyperhidrosis

Dandruff (Pityriasis capitis) Most common condition affecting the scalp. Causative organism: Malassezia species. Seen as mild, moderate or severe scaling of scalp. May or may not be associated with itching. Simple dandruff does not cause hair loss. Striae (Stretch Marks) Seen as pinkish white lines around knees, axillae, outer aspect of thighs, lumbosacral region. Sudden increase in height or weight causes rupture of connective tissue beneath an intact epidermis.

Striae

Pseudoacanthosis nigricans Velvety hyperpigmented skin in flexures Asymptomatic Mostly seen in obese children

Cutaneous manifestations Child abuse Includes physical abuse, neglect, sexual exploitation. Cutaneous manifestations Bruises. Traumatic alopecia. Thermal burns. Sexual abuse : Vaginal tears, anal tears, hematomas. Sexually transmitted infections.

General principles of skin care in neonates children Bathing, soaps and cleansers : Gentle cleansers are advised. Excessive cleansing, scrubbing & incomplete rinsing lead to irritation. Shampoos : Should be isotonic to tears and less irritating to eyes. Shampooing twice a week controls normal flaking. Care of the diaper area : Gentle cleansing & limiting use of disposable diapers. Intertriginous areas should be kept dry and ventilated.

Differences in treatment of Pediatric and Adult Patients Conservative management is best. Surface area is more in children as compared with adults. Percentage of absorption of topical drugs is more. Try to use lowest effective dose of medications. Do not use treatments which may retard growth or mental development. Avoid off-label uses of medications.

MCQ’s Q.1) One of the criteria to diagnose neurofibromatosis-I is cafe-au-lait macule. The size of the macule in children should be > 1cm > 0.5cm > 1.5cm > 0.05cm Q.2) In erythema toxicum neonatorum, Wright stain of a pustule reveals majority of the cells are of Neutrophils Eosinophils Lymphocytes Mast cells Ans : Q. 1 – B, Q. 2 – B

MCQ’s Q.3) In Miliaria crystallina, obstruction of sweat duct is at the level of Stratum corneum Stratum granulosum Stratum spinosum Dermoepidermal junction Q.4) Koenen’s tumor is associated with Neurofibromatosis Icthyosis Tuberous sclerosis Epidermolysis bullosa Ans : Q. 3 – A, Q. 4 – C

MCQ’s Q.5) Neonatal perineal dermatitis, intertriginous areas are invariably spared in Allergic contact dermatitis Diaper dermatitis Seborrheic dermatitis Candidiasis Ans : Q. 5 – B

Q. Identify the condition. Photo Quiz Ans : Strawberry Haemangioma Q. Identify the condition.

Q. What is the most probable diagnosis Photo Quiz Ans : Diaper Rash Q. What is the most probable diagnosis

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