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Transcript
Skin conditions in Down’s syndrome Celia Moss Consultant Dermatologist Birmingham Children’s Hospital Explanation This is a text-only version of the lecture given at the DSMIG meeting in London on 28th November 2008. Lecture summary • Rare skin conditions seen in Down’s syndrome • Common skin conditions that are more common in Down’s syndrome Rare conditions seen in Down’s syndrome • Neonatal blistering eruption with leukaemoid reaction • Elastosis perforans serpiginosa • Multiple syringomas • Calcinosis cutis Neonatal blistering eruption • Present at or soon after birth • Mostly on the face but can be anywhere • May be associated with transient blood abnormality like leukaemia • Usually gets better by itself • Haematological follow-up required because risk of myelodysplasia / leukaemia Differential diagnosis • “Milk spots” (sebaceous hyperplasia) • Miliaria • Infection – – – – Staph Candida HSV Pityrosporon • Toxic erythema of the newborn • Transient pustular melanosis • Eosinophilic pustulosis Elastosis perforans serpiginosa • • • • Scaly red papules a few mm across Arranged in lines or rings a few cm across Usually no symptoms May resolve spontaneously after a few years • Cause? May be related to a connectivetissue abnormality in Downs syndrome Multiple syringomas • Erythematous papules a few mm diameter, may be confluent • Central forehead, peri-oral, post auricular and forearms • Asymptomatic (non-itchy) • Appear in older children and adults • No easy treatment Calcinosis cutis • Small, milia-like lesions • dorsal surface of hands, and face • Sometimes results from calcification of pre-existing syringomas Common conditions that are more common in Down’s syndrome • • • • Autoimune disorders Dry skin conditions Infections Poor peripheral circulation Common conditions that are more common in Down’s syndrome • Autoimune disorders – Alopecia areata – Vitiligo? • Dry skin conditions • Infections • Poor peripheral circulation Links between AA and Down’s? • AA occurs in up to 8.8% of Down’s • General increased autoimmunity? • Specific link with chromosome 21? – AA occurs in 30% of people with APS-1 (Autoimmune polyendocrinopathycandidiasis-ectodermal dystrophy) which maps to the Down’s critical region on 21 Alopecia areata • Differential diagnosis – Tinea capitis: inflammatory, scaly alopecia • Treatment – – – – – None Topical steroid ?topical tacrolimus UV Contact sensitiser eg diphencyprone • Bad prognostic indicators – Atopy – “ophiasic” pattern (occipital area) – Probably Down’s syndrome Vitiligo: differential diagnosis • Post inflammatory hypopigmentation • Seborrhoeic dermatitis • Pityriasis alba Vitiligo: management • • • • • Nothing Sunscreens Topical steroid Topical tacrolimus camouflage Common conditions that are more common in Down’s syndrome • • • • Autoimune disorders Dry skin conditions Infections Poor peripheral circulation Common conditions that are more common in Down’s syndrome • Autoimune disorders • Dry skin conditions – – – – – – Xerosis Seborrhoeic dermatitis Loss of elasticity and accelerated ageing Keratosis pilaris Hyperkeratotic psoriasis Patchy lichenified/discoid eczema • Infections • Poor peripheral circulation Emollients • Avoid bubbles (degreasing) • Encourage bathing (hydration) • Provide enough emollient – up to 1kg per week • Acknowledge cultural practice eg – – – – – Baby oil / lotion Cocoa butter Olive oil Almond oil Mustard oil • Provide choice… “I only use a tiny amount of steroid” “I believe in slapping on the steroid” Benefits of steroids outweigh harms when used correctly Finger-tip units for topical steroids Common conditions that are more common in Down’s syndrome • Autoimune disorders • Dry skin conditions • Infections – Cheilitis – Folliculitis and blepharitis – Norwegian scabies – Fungal infections of the feet and nails • Poor peripheral circulation Cheilitis • Presentations: – angular cheilitis – lip fissuring – fissured tongue • may become chronic • may be due to carriage of staph aureus and candida albicans • management: – Swab and treat infection – Regular grease especially at bedtime Staph aureus folliculitis • Prophylactic antiseptics better than repeated antibiotics • antiseptic can be used in the bath and as emollient – eg chlorhexidine, benzalkonium (Oilatum Plus®, Dermol 500®) Pityrosporon (yeast) • Presentations – – – – Dandruff Blepharitis Seborrhoeic dermatitis Folliculitis, especially shoulders • Treat with anti-dandruff or anti yeast preparations – eg clotrimazole, selenium sulphide Treatment of dermatophytes and yeasts • Dermatophytes – topical • • • • imidazoles Terbinafine Amorolfine NOT NYSTATIN – systemic • griseofulvin • terbinafine • itraconazole • Yeasts – topical • imidazoles • Nystatin (candida) • Selenium sulphide (pityriasis versicolor) – systemic • fluconazole • NOT GRISEOFULVIN Treatment of dermatophytes and yeasts • Topicals – Cream for skin – Lacquer for nails – powder for crevices (prevention) • Systemic – Griseofulvin now tablets only – terbinafine - tablets only – imidazoles - tablets or liquid Dividing griseofulvin 125mg tablets 10-20mg/kg Tablet crusher Tablet cutter Common conditions that are more common in Down’s syndrome • • • • Autoimune disorders Dry skin conditions Infections Poor peripheral circulation Common conditions that are more common in Down’s syndrome • • • • Autoimune disorders Dry skin conditions Infections Poor peripheral circulation – Cutis marmorata – Acrocyanosis • Common in newborns • Persist longer in Down’s syndrome • May reflect cardiac defect
