Derm atologia pediatrica: le lesioni e le m e n ta ri

Dott. Angelo Milazzo Pediatra di Fam iglia Responsabile della Rete di Derm atologia Pediatrica della Regione Sicilia Società Europea di Derm atologia Pediatrica Docente di Pediatria di Fam iglia all’Università di Catania Primary lesions

• There are no universally agreed upon definitions of term s or, in particular, dim ensions of prim ary lesions. • - circum scribed area of skin, up to 1.0 cm , with a changeM acule from norm al skin color, which is neither raised above nor depressed below the surrounding skin. Many use the term for lesions much greater than 1.0 cm . Term does not include . purpura • - a flat, circum scribed, discoloration of skin or mucous mPatch em brane greater than 1.0 cm in diam eter. • - discrete solid area of skin that is elevated by palpationPapule above the surrounding skin less than 1 cm in diam eter. Variations include accum inate,and keratotic, flat- topped, follicular, um bilicated, pedunculated, necrotitic and o th e rs . • - sim ilar to a papule but greater that 1.0 cm in diamPlaque eter. Often form ed by the confluence or coalescence of papules. Secondary features may include, am ong others, atrophy, lichenification or hyperkeratosis. Vesicles

• - a circum scribed fluid-filled lesion less than 1Vesicle .0 cm in diam eter that is usually elevated above the surrounding skin. May be described as solitary, grouped, um bilicated, dyshidrotic, spongiotic, multi- locular or uni-locular. • - a circum scribed fluid-filled lesion greater than 1Bulla .0 cm in diam eter that is usually elevated above the surrounding skin. May attain diam eters of several cm s and are described as tense, or flacid. • - descrete elevated vesicle or bulla of skin, usuallyPustule small, containing purulent exudate composed of inflam m atory leukocytes (pus), with or without cellular debris. May be superficial, deep-seated, follicular, grouped, etc. and may arise secondarily from a vesicle. Nodules – Tumors

• - descrete, solid, palpable, round or ovalNodule (elipsoidal) lesion of the skin measuring up to 1.0 cm in diam eter (or long axis). Applies to processes involving any or all levels of the skin, and is a general term for any mass, benign or malignant. • - a term used by som e for a "nodule" greaterTumor than 1.0 cm in diam eter. Applies to processes involving any or all levels of the skin, and is a general term for any mass, benign or malignant. U rtic a ria

- an evanescent, round or irregular, • Wheal often flat-topped elevation of skin with a pale red color, arising from edem a in the superficial derm is. May vary from 2-3 mm to 10 or more cm in diam eter, with round or arcuate configurations. Should be distinguished from , a massive edem a involving the entire derm is and subcutaneous tissues. Secondary lesions

• - a hard plaque of dense fibrotic tissue covered by a thin epidermScar is. A mark of injury from any sort of process (physical or pathologic). • - Atrophy usually refers to thinning of the epiderm is leavingAtrophy an easily wrinkled and/or shiny surface. Atrophy may also apply to derm al and/or subcutaneous tissue, with or without changes in the epiderm is. • - loss of skin tissue or substance from the surface downward,Ulcer leaving an uncovered or denuded wound that is slow to heal. • - a superficial denudation of the skin, usually im plying theErosion loss of the epiderm is. - a vertical splitting or separation of the skin. • Fissure • - dried surface fluid, often serous (inspissated serum ) in nature,Crust with or without tissue debris. For purposes of this docum ent this includes the term "scab". Secondary lesions

• - a scratch m ark, often with denudation of the skin toExcoriation form a sm all ulcer. Exposure of the corium by mechanical rem oval of the epiderm is. • - a thin flake of epithelium (mostly com posed of corneoctyes)Scale which is separated from the underlying intact skin proper. • - a thickening of the skin surface an increaseLichenification of skin markings, usually seen with chronicand coalescence of papular lesions, especially atopic eczem a. • - a lushly grow ing, proliferating, process, usually withVegetating elevated or exophytic features. • - technically secondary features, but includedLinear / Figurate here solely out of convenience.not These are that skin lesions may assum e, which aid in theirconfigurations diagnostic identification. Figurate includes geom etrical shapes (e.g. annular, arciform , cyclic, etc.). M acular Lesions

• Brown • Grey Slate Blue • Black • White • Pink - Red • Yellow • Scale Brown M acular Lesions

• · Ephilides • · Neurofibromatosis • · Albright's disease • · Peutz Jeghers syndrome • · Xeroderm pigmentosum • · Acropigmentation • · Seborrheic keratosis • · Neurocutaneous melanosis • · Nieman-Pick disease • · M elasma (Chloasma) • · Kwashiokor • · Photochemical reactions • · Berlock dermatitis • · Ultraviolet light (sun tan) • · Thermal radiation - ab igne • · Becker's nevus • · Hemochromatosis Brown M acular Lesions

• · Trauma • · Post-inflammatory hyperpigmentation • · M alignant melanoma • · Lentigo melanoma • · M astocytosis (urticaria pigmentosa) • · Encephalitis (chronic) • · Cronkhite - Canada syndrome • · Tatoo • · Reticular acropigmentation of Kitamura • · Dermatopathia pigmentosa reticularis • · Fanconi's syndrome • · Riehl's melanosis • · Periorbital melanosis • · Franceschetti - Jadassohn syndrome • · Gaucher's disease • · Dyskeratosis congenita • · Addison's disease

Grey Slate Blue M acular Lesions

• Oculodermal melanocytosis (Nevus of Ota) • Dermal melanocytosis (M ongolian spot) • Blue melanocytic nevus • Incontinentia pigmenti • Franceschetti - Jadassohn syndrome • Hemochromotosis • Chronic nutritional insufficiency • Fixed • Pinta • Erythema dyschromicum perstans • M alignant melanoma • Tatoo • M aculae cerulae • Ochronosis • M etastatic melanoma Black and white M acular

• Black M acular Lesions Lesions • M elanocytic nevus • M alignant melanoma • Ecchymosis (blue-black) • White and Hypomelanotic M acular Lesions • Vitiligo • Albinism • Piebaldism • Vogt-Koyanagi-Harada syndrome • Tuberous sclerosis • Waardenburg's syndrome • Hyperthyroidism • Hypoparathyroidism • Addison's disease • Hypopituitarism • Pernicious anemia • Halo nevus (Leukoderma acquisitum centrifugum - Hyde and Sutton) • Nevus depigmentosus • Leprosy - tuberculoid • Pityriasis alba • Chediak-Higashi syndrome • Post-inflammatory depigmentation • Phenylketonuria (pigment dilution) • Alezzandrini's sundrome • Idiopathic guttate hypomelanosis • Syphilitic leukoderma (L2) • Scleroderma / morphea

Pink – red - yellow M acular Lesions • Exanthems • Drug eruptions • Telangiectasia • Nevus flammeus (Port wine stain - salmon patch) • Jaundice • Xanthomata (plantar - plantar) • Xanthelasma (sometimes paplable) • Carotenemia • Pseudoxanthoma elasticum • Necrobiosis lipoidica diabeticorum • Amyloidosis (primary) • Fordyce condition • Solar elastosis

M acules with Scale

• Tinea versicolor • Seborrheic keratosis • Pityriasis alba

Papular Lesions

• With Scale • Koebner Phenomenon • Facial Papule(s) Papulo-squamous differential

• Pityriasis rosea • Psoriasis • Lichen planus • Pityriasis rubra pilaris • Parapsoriasis • Secondary syphilis • Tinea versicolor • Tinea corporis • Seborrheic dermatitis • Drug reaction - lichenoid (e.g. atabrine, gold, isoniazid)

Koebner Phenomenon

• Lichen planus • Eczema (active) • Psoriasis

• Auto-inoculation: Verruca, M olluscum contagiosum, etc.)

Flesh Colored Papules

• Adenoma sebaceum of Pringle • Basal cell carcinoma • Intraepidermal nevus • Acrochordon • Dermatofibroma • Verruca vulgaris / plana • M olluscum contagiosum • Syringoma • Acne cyst • Hidrocystoma – Apocrine – Eccrine • Epithelioid inclusion cyst ("Sebaceous" cyst) • Epithelioma adenoides cystica / Trichoepithelioma • Neurofibroma • Nevus lipomatous superficialis • Nodular elastoidosis (with cysts and comedones) / (Favre - Racouchot syndrome) • Lichen amyloidosis • Lipoid proteinosis • Trichofolliculoma • M ilia • Colloid milium • M etastatic lesions

Nodular Lesions

• Nodules on Legs • Nodules around Joints Nodules on the Legs • • Subacute nodular migratory • Weber-Christian disease (relapsing febrile nodular panniculitis) • Superficial migratory thrombophlebitis • Acute pancreatitis with nodular subcutaneous fat necrosis • Polyarteritis nodosa • Insect bites • Sarcoidosis • Erythema induratum (Bazin) • Erythema nodosum leprosum • Trichophyton rubrum granuloma (M ajocchi) • Zirconium and Silicon granuloma • Deep fingal infection • Actinomycosis, blastomycosis, sporotrichosis, coccidioidmycosis • M eningococcal or pneumococcical septicemia • Kaposi's sarcoma - other neoplasm • Rheumatoid nodules • Sweet's syndrome - usually tender plaque(s) • Behcet's disease • Parasites (leishmaniasis, echinococcosis, cysticercosis, shistosomiasis, onchocerciasis, myiasis) • Rothman-M akai syndrome Nodules around Joints

• Arthritis – Rheumatoid arthritis – Gouty tophi (Tophaceous gout) • Infectious disease – Papulonecrotic tuberculid - elbows, fingers • M etabolic disease – Xanthoma tuberosum – Calcinosis cutis • Neoplasms – M yxoid cyst • M iscellaneous – Erythema elevatum diutinum – Reticulohistiocytoma

Tumor Lesions - Differential by Color

• Brown • Black - Grey • Blue • Pink - Red - Purple • Skin Colored - Yellow - Fawn – Orange

• Painful Dermal Tumors Brown • M alignant melanoma • Spitz nevus • M elanocytic nevus • Pyogenic granuloma (brown to red) • Seborrheic keratosis • Comedone • Basal cell carcinoma • Acanthoma (brown to pink) • Histiocytoma • Skin tags (Acrochordons) • Clear cell acanthoma • Trichofolliculoma • Actinic keratosis • Squamous cell carcinoma • Paget's disease • Nevus of Ota (brown to blue) • Neuroma (brown, pink or purple) • Leiomyoma (dusky brown-pink-red) • Hidradenoma papilliferum (reddish-brown when ulcerated) • Sarcoidosis

Black-Grey

• M elanocytic nevus • Blue Nevus • M alignant melanoma • Seborrheic keratosis • Basal cell carcinoma • Paget's disease (pink-grey-yellow) • Hematoma • Thrombosed plantar wart • Apocrine hidrocystoma (blue-grey-black) • Bowen's disease • Granuloma telangiectaticum (blue-black) • Eccrine hidrocystoma Blue

• Blue nevus • M alignant melanoma • Neurofibroma • Histiocytoma • Thrombosed plantar wart • Apocrine hydrocystoma (blue-grey-black) • Nevus of Ota (blue-brown) • Granuloma telangiectaticum (blue-black) • Kaposi's sarcoma (dark blue or purple) • Lymphangiosarcoma of Stewart and Treves (dusky blue to red) • Eccrine hidrocystoma Pink - Red - Purple

• Spitz nevus • Pyogenic granuloma • M alignant melanoma • Hemangioma (purple) • Keratoacanthoma (flesh colored to red) • Histiocytoma • Eccrine poroma (acrospiroma) (pink to livid red) • Hidradenoma papilliferum (ma be reddish brown) • Dermal cylindroma (pink to red) Skin Colored - Yellow - Fawn - Orange • Basal cell carcinoma - Examples at the Image Database. • Seborrheic keratosis • Sweat gland nevus (orange - red - brown) • Trichoepithelioma (pink - yellow) • Sebaceous adenoma (yellow) • Sebaceous carcinoma (yellow - orange) • M ilium (white - yellow) • Steatocystoma multiplex (yellow) • Syringoma (yellow - mauve - translucent) • Actinic keratosis (yellow) • Neurolemmoma (pink - grey - yellow) • Eccrine spiradenoma (flesh colored) • Syringoma papilliferum (flesh colored)

• Painful Dermal Tumors • Angiolipoma • Angioleiomyoma • Eccrine spiradenoma • Glomus tumor • Neuroma • Neurolemmoma • Leiomyoma

Vesicular / Bullous Lesions

• Vesicular / Bullous Lesions • Epidermal • Subepidermal • Hands • Grouped • Umbilicated Epidermal Vesicular / Bullous Lesions

• Pemphigus vulgaris • Pemphigus erythematosus (Senear-Usher Syndrome) • Pemphigus foliaceus • Pemphigus vegetans • Benign familial pemphigus (Hailey and Hailey Disease) • Epidermal Vesicular / Bullous Lesions • Incontinentia pigmenti • Toxic epidermal necrolysis • Impetigo / Bullous impetigo • Bullous congenital ichthyosiform • Epidermolysis bullosa simplex • Cockayne's disease (Recurrent bullous eruption of hands and feet) • Cantheridine • M iliaria crystallina • M iliaria rubra • Acrodermatitis enteropathica • Eczematous blisters: – Allergic and contact dermatitis (e.g. garlic) – Fungal: Inflammatory infections and id reactions – Dyshidrosis (Pompholyx) – Ultraviolet light – Others • Bacterial vesicles / papulovesicles – Impetigo / Bullous impetigo – M eningococcemia-stretococcemia, often hemorrhagic • Viral vesicles: – Hand, foot and mouth disease (Coxsackie A16) – Varicella - Zoster – Others • Parasitic vesicles:

– Scabies - Examples Hand lesions

• Vesicular / Bullous Lesions of the Hands Hand, foot and mouth disease (Coxsackie A16) - • Acute eczematous dermatitis • Dermatophytid • Dyshidrotic eczema • Scabies - Examples • Bullous - Example. • Epidermolysis bullosa syndromes - Examples: See Image Database • Congenital syphilis • Porphyria cutanea tarda • Erysipeloid • Physical factors • Viral: Herpes simplex, varicella - zoster, others. - Examples: See Image Database • Toxic epidermal necrolysis • Bullous lichen sclerosis et atrophicus • Fixed drug • Blister beetles

Subepidermal Vesicular / Bullous

• Erythema multiforme Lesions • Bullous Pemphigoid • Cicatricial pemphigoid (Benign mucosal pemphigoid) • Dermatitis herpetiformis • Dermolytic epidermolysis bullosa: – Dominant – Recessive – Acquired - Examples • Junctional epidermolysis bullosa • M endes da Costa Syndrome (M acular dystrophic epidermolysis bullosa) • Porphyria cutanea tarda • Bullous • Bullous lichen planus • Fixed drug eruption (i.e. barbiturates) • Burns • Suction • Friction or shearing forces • Trauma (i.e. secondary to a bone fracture) • Lichen sclerosis et atrophicus • Urticaria pigmentosa • Vesicular polymorphous light eruption • Herpes gestationis • Ionizing radiation Grouped Vesicles and Pustules

• Herpes simplex • Herpes zoster • Impetigo herpetiformis • Dermatitis herpetiformis • Herpes genitalis • Herpes gestationis • Subcorneal pustulosis

Pustular Lesions • Folliculitis • Candidiasis • Impetigo • Impetigo herpetiformis • Acne vulgaris • Chloracne • Steroid induced acne (e.g. dexamethasone) • Pustular drug eruption • Rosacea (Acne rosacea) - M ore examples • Erythema toxicum neonatorum • Transient neonatal pustular melanosis • Incontinentia pigmenti (1st stage) (usually vesiculobullous) • Perioral dermatitis • Pustular psoriasis – Generalized pustular psoriasis of Zumbusch – Acrodermatitis continua • Acropustulosis • Palmoplantar pustulosis • Subcorneal pustular dermatosis (of Sneddon-Wilkinson) • Eosinophilic folliculitis (Ofugi's disease) • Hidradenitis suppurativa • Folliculitis decalvans

Umbilicated Vesicles

• Varicella • Herpes simplex • Herpes zoster Wheal or Urtica Lesions

• Urticaria, NOS • Cholinergic urticaria • , including dermagraphism • Contact urticaria • Systemic lupus erythematosus • Hypocomplementemic vasculitis • Insect bite • Drug eruption • Anaphylaxis • Erythema multiforme (early)

Cyst Lesions

Epidermoid cyst - ( Follicular infundibular cyst) • Pilar cyst (Tricholemmal cyst) • Dermoid cyst • M ucinous cyst • Hydrocystoma (blue hue) – Eccrine – Apocrine (more blue) • M ilia • Thyroglossa • Bronchogenic cysts • Branchial cleft cyst