Mucosal Disease In Children

Question 1

Are children more prone to mucosal lesions and why

Answer 1

Children are associated with higher frequency of some intraoral mucosal lesions as there is immunological immaturity.

Question 2

Give examples of congenital and acquired oral mucosal lesions in childhood

Answer 2

Congenital - hereditary (white sponge neavus) or developmental (Fordyce spots, geographic tongue)

Acquired - traumatic (frictional keratosis, traumatic ulcer, mucocele), drug-related( chemotherapy induced stomatitis), chronic inflammation (non-infective) (recurrent aphthous ulcers, erythema multiforme, orofacial granulomatous, oral LP,mucous membrane pemphigoid, pemphigus, epidermolysis bullosa), hyperplastic (pyogenic granuloma, giant cell epulis, vascular deformation, pigmentary changes) neoplastic, infective( HSV-1, VZV, Coxackie virusz HPV, Candida, Streptococcus -scarlet fever)

Question 3

White sponge naevus

Answer 3

Inherited as autosomal dominant condition
Uncommon
Clinical features: white, soft, irregularly thickened, no defined borders, bilateral, genital tissues may be affected as well, can be misdiagnosed with candidiasis

Histopathology: uniform acanthosis, hyperparakeratosis, intracellular oedema, no dysplasia, no inflammation

Management: reassurance

Question 4

Fordyce spots

Answer 4

Not a lesion but variation of normal anatomical structure
Ectopic sebaceous glands
Bilateral, common on buccal mucosa

Management: reassurance

Question 5

Foliate papillae

Answer 5

Pinkish soft nodules
Bilateral, ventral tongue
Made of lymphatic tissue, can be inflamed/hyperplastic

Management: reassurance

Question 6

Geographic tongue (erythema migrans)

Answer 6

Clinical features: dorsum of the tongue, irregular, smooth, red areas of depapilation with sharply-defined edge
Recurrent, migrates

histology: thinning of epithelium at centre, mild hyperplasia at periphery, epithelium infiltrated by neutrophils

Management: reassurance, if symptomatic - exclude haematological deficiencies

Question 7

Frictional keratosis

Answer 7

Clinical features: pale and translucent or white/dense with rough surface

Histology: epithelial hyperplasia, with thick granular cell layer and kyperorthokeratosis, scattered subepithelial lymphocytes

Question 8

Traumatic ulcer

Answer 8

Clinical features: mechanical, thermal or chemical trauma
Inflammation levels and clinical features vary
Yellowish floor of fibrin slough often present

Histology: non specific ulceration, destruction of epithelium, tissue infiltration by neutrophils

Question 9

Mucocele

Answer 9

Clinical features: most commonly on labial mucosa, overlying mucosa is intact and healthy, topically circular, firm or fluctuant and bluish

Histology: damage to duct of salivary gland, mucin and macrophages surrounded by compressed connective tissue, no epithelial lining

Management: reassurance/excision if symptomatic or interferes with oral functions

Question 10

Recurrent aphthous ulcers

Answer 10

Clinical features: idiopathic, but systemic factors known to modulate the severity of disease, onset in childhood but peak in adolescence
Three types: minor, major, herpetiform

Histology: non specific ulceration, initial lymphocytic infiltration, destruction of epithelium, tissue infiltration by neutrophils, dilated vessel

Extra oral involvement should be excluded ( if in GI- coeliac or Crohn’s; if genital/ocular- Bechet’s)

Question 11

Erythema multiforme

Answer 11

Clinical features: mostly HSV related, drug induced, cutaneous erythema or target lesions, ulceration of outer lip with bleeding, crusting, intraoral irregular finrin-covered erosion and erythema

Histology: necrosis of keratinocyes, epithelial infiltration by inflammatory cells, intraepithelial or subepithelial vesiculation

Question 12

Orofacial granulomatous

Answer 12

Clinical features: swelling of the lip, mucosal cobblestoning/nodularity, mucosal tags, gingival hyperplasia, aphthous oral ulcers, in children - more prone to develop Crohn’s

Histology: granulomas containing multinucleated giant cells, lymphoedema

• Can be related to sarcoidosis and tuberculosis

Question 13

Oral lichen planus

Answer 13

Rare in children

Clinical features: white striae and/or mucosal atrophy, and/or erosions, and/or plaques, desquamative gingivitis in isolation or in combination with above, bilateral lesions

Histology: basal membrane thickening, band-like lymphocytic infiltrate, colloid bodies- apoptotic basal cells

Question 14

Mucus membrane pemphigoid

Answer 14

Rare in children

Clinical features: bullae, sometimes intact, can appear as blood blisters, if reptired- leaves raw ulcer, desquamative gingivitis, conjunctival involvement, cutaneous involvement less prominent than bullous pemphigoid

Histology: separation of full thickness of epithelium from connective tissue, linear deposition of IgG and C3/4 along basal membrane, auto-antibodies bind along the basal membrane in direct immunofluorescence assay, CT infiltrated with inflammatory cells

Question 15

Pemphigus

Answer 15

Rare in children

Clinical features: fragile vesicles/bullae, ruptured vesicles, Nikolsky sign positive, widespread cutaneous involvement

Histology: acantholysis, intraepithelial vesicles, chicken wire deposition of IgG and C3/4 within the epithelium, anti-desmoglein 3 antibodies bind intracellular substance in direct immunofluorescence

Question 16

Epidermolysis bullosa

Answer 16

Rare in children
Subepithelial bullae leading to severe scarring after minimal trauma
Type VII collagen defect
Autosomal recessive

Question 17

Pyogenic granuloma

Answer 17

Secondary to chronic irritation

Clinical features: soft, red polypoid swelling, most commonly gingival but not exclusively

Histology: granulation tissue, dilated blood vessels in oedematous connective tissue, dense infiltration by neutrophils
NO granuloma, NO pyogenic bacteria/pus

Question 18

Giant cell epulis (peripheral giant cell granuloma)

Answer 18

Clinical features: soft, violaceous swelling, central gingival segments

Histology: subepithelial clusters of giant cells in fibro-vascular tissue

• Central giant cell granuloma and underlying hyperparathyroidism needs to be excluded!

Question 19

Vascular malformation

Answer 19

Clinical features: purple nodular lesion, blanches upon pressure, can be focal or systemic

Histology: capillary: small vessels and vasoformative tissue
Cavernous: large blood filled sinusoids

Question 20

Pigmentary changes

Answer 20

Generalized pigmentary changes (post-inflammation, drug-induced) are uncommon in children

Pautz- Jeghers syndrome: heritable, intestinal polyposis, mucocutaneous pigmented lesions appearing during childhood

Addison’s disease: diffused oral pigmentation can be an early sign
Mild acanthosis, melanin in basal layer

Question 21

Viral infectious mucosal changes

Answer 21

HSV-1 : perimar herpetic stomatitis, herpes labial is
VZV: chickenpox, zooster ( recurrence)
Coxackie virus: hand-food-mouth disease, herpengina
HPV: warts, primary squamous cell papilloma (rare)
Epstein Barr virus: infectious mononucleosis
Measles virus: measles

Question 22

Fungal infections mucosal diseases

Answer 22

Candida:
Pseudomembranous candidosis - -angular cheilitis ( associated with OFG)
- erythematous candidiasis ( rare in children)
-mucocutaneous candidosis (rare, hereditary)

Question 23

Histopathology of HSV, VZV- associated lesions

Answer 23

1. Intranuclear oedema at basal cell layer
2. Ballooning degeneration - swelling of keratinocyes and loss of attachment
3. Progressive cell swelling
4. Rupture of infected cells and release of viral particles to non-infected cells

Question 24

Warts

Answer 24

Clinical features: white, pinkish, raised, sessile or pedunculated, often multiple, mainly secondary to self inoculation

Histopathology: papillary processes of hyperplastic epithelium with acanthosis, hyperkeratosis, hyperplastic basal cell layer, connective tissue core, koilocytes often present

Question 25

Pseudomembranous candidosis

Answer 25

Clinical features: acute, thick white pseudomembranes of various sizes, erythematous background, often idespread
Diagnosis is clinical but can do culture and sensitivity to treatment

Histopathology: hyperplastic epithelium, parakeratin, superficial necrotic layer, hyphal invasion down to spinous cell layer, neutrophilic infiltration at spinous layer, light subepithelial lymphatic infiltrate