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2019. 9.

Papulosquamous diseases
구진인설질환

SANG WOONG YOUN / SEONG JIN JO

Department of Dermatology
Seoul National University College of Medicine

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Papulosquamous diseases

• Psoriasis vulgaris (건선) 가장 대표적, 특징적인 질환이라 가장 중요하게 다룰 것

• Pityriasis rosea (장미색 비강진) 심심찮게 보는 질환이라 이것도 좀 열심히.

• Pityriasis rubra pilaris (모공성 홍색 비강진)


• Lichen planus (편평태선)
• Pityriasis alba (백색 비강진)

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What is papulosquamous
disease?

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이렇게 생긴걸 전부 papillosquamous Ds.라고 부른다.

main lesion: papule + scale

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What is the difference from the other
erythematous skin lesions?

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Psoriasis
(건선)

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Epidemiology

• prevalence: US (2%~3%), Korea (<0.5%) 흔하고

• prevalence ranges from 0.1% ~ 3%

• variation: race & environment

• age of onset: may begin at any ages 평생동안 고생할 수 있는


질환.

• most likely to appear between 15 ~ 30

• early onset(22.5yrs) vs. late onset (55yrs)

• severe disease in early onset patients

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mode of inheritance

• multifactorial inheritance

• 1/3 of patients: relatives with psoriasis

• 8.1% (from one parent), 41% (both parents)

• HLA-B13, HLA-Bw57, HLA-Cw6, HLA-DR7

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Pathogenesis

• Genetic factors 유전적인 요인도 있고


면역학적인 dysregulation도 중요하게 작용한다.

• Immune dysregulation 이 2가지를 위주로 설명!

• initiating or aggravating factors

• dysfunctions in keratinocyte proliferation &


differentiation

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Genetic factors

• nine chromosomal loci: psoriasis susceptibility


genes 1~9 (PSORS1 ~ PSORS9)

• PSORS1 gene

• HLA-C protein (variant HLA-Cw6)

• associated with guttate psoriasis 물방울 건선과 관련성이 높다.

• not associated with late onset psoriasis/PPP

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genetic background가 있다.

Major psoriatic gene variants

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Immunopathogenesis

• Dysregulated immune mechanisms in psoriasis

• infiltrated immune cells (T cells, dendritic cells)

• clonal T cells in the lesions

• therapeutic effects of immunoregulatory drugs

• may cure psoriasis after BMT

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immunopathogenesis of psoriasis

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Clinical types of psoriasis

• plaque psoriasis (판상건선)

• guttate psoriasis (물방울건선)

• pustular psoriasis (고름물집건선)

• localized vs. generalized

• erythrodermic psoriasis (홍색피부건선)

• exfoliative psoriasis (박탈건선)

• psoriasis of specific areas: scalp, face, nails, genitalia


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Plaque psoriasis

• common psoriasis lesions

• erythematous papules/plaques with silvery white


scales

• Koebner(isomorphic) phenomenon

• Auspitz sign

• itching: not common, but in the aggravation stage

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early psoriasis
; typical scaly papule
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chronic plaque type psoriasis

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Guttate psoriasis

• acute psoriasis

• 0.5 ~1.5cm sized guttate papules

• mainly in children or young adults

• after streptococcal throat infection

• increased ASO titer

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guttate psoriasis

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Pustular psoriasis (1)

• Localized pustular psoriasis (Barber type)

• palmoplantar pustulosis (PPP)

• recurrent pustules on palms/soles

• sterile pustules

• frequently resistant to treatment

• different drug responses than psoriasis

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localized pustular psoriasis

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Pustular psoriasis (2)

• Generalized pustular psoriasis (von Zumbusch)


• systemic symptoms: fever, arthralgia, general malaise
• leukocytosis, hypercalcemia
• life-threatening: sepsis, dehydration
• IL-36 receptor antagonist(IL-36ra) gene mutation

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generalized pustular psoriasis
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Erythrodermic psoriasis

• severe redness with sheet-like exfoliation

• severe itching and pain

• affects the most of body surface

• increased heart rate, body temperature

• fluid & electrolyte imbalance

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erythrodermic psoriasis

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Psoriasis of specific areas

• scalp

• face

• nails

• genitalia

• affects more to patients’ QoL

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scalp psoriasis

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facial psoriasis

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nail psoriasis (nail pitting)

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nail psoriasis (subungual hyperkeratosis)

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genital psoriasis

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Disease modifying factors
• Obesity: severe psoriasis

• Smoking: two fold risk of severe psoriasis

• Infection

• streptococcal throat infection: guttate psoriasis

• HIV

• Drugs

• antimalarials, β-blockers, NSAIDs, IFN-α, IFN-Ɣ,


imiquimod, ACE inhibitors, lithium
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Comorbidities

• Cardiovascular diseases

• Metabolic syndromes: DM, dyslipidemia, obesity

• Fatty liver

• Psoriatic arthritis

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organs or diseases caused by IMIDs

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Diagnosis

• clinical findings

• skin biopsy

• differential diagnosis

• nummular eczema
• mycosis fungoides
• superficial fungal infection
• other papulosquamous diseases

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histopathology of
psoriasis

Munro microabscess
spongiform pustule of Kogoj

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Treatment
• principles of psoriasis treatment

• maximum effectiveness

• minimum side effects

• topical therapy: ointments, phototherapy

• systemic therapy: oral medications, biologics

• monotherapy vs. combination therapy

• rotational therapy

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Topical agents

• vitamin D analogues: calcipotriol, calcitriol, tacalcitol

• retinoid: tazarotene

• steroids: potency according to the location

• tar, anthralin - old medication, inhibit cell proliferation

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Phototherapy

• UVB phototherapy

• broadband UVB (BBUVB) : tar, anthralin


application, emollient phototherapy

• narrowband UVB (NBUVB): 311nm

• UVA phototherapy

• PUVA: psoralen (photosensitizer) + UVA

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Systemic medications
• Retinoids: acitretin, etretinate

• inhibition of keratinocytes proliferation

• promotion of keratinocytes differentiation

• Cyclosporine A

• immunomodulation of activated T cells

• Methotrexate (MTX)

• inhibition of DNA synthesis

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Biologics

• TNF-α inhibitor: etanercept, infliximab, adalimumab,


golimumab

• IL-12/23 inhibitor: ustekinumab

• IL-17A inhibitor: secukinumab, ixekizumab

• IL-23 inhibitor: guselkumab, risakizumab

• T cell blocker

• alefacept, efalizumab (not in use)

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Pityriasis rosea
(장미색 비강진)

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Epidemiology

• age group: 15 ~ 40

• slight female predominance

• no racial difference

• seasonal variation: spring, autumn

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Etiology

• not clear

• associated with HHV-6, HHV-7

• prodromal symptoms
• clustering of disease
• spontaneous remission
• no recurrence
• insect bite, autoimmune disorder, drug rash

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Clinical manifestations
• prodromal symptoms: 5%

• herald patch (primary plaque): 50~90%

• oval or round, collarette of fine scaling


• mostly on the trunk
• Rash

• 7-14 days after herald patch


• distributed over trunk and extremity
• “Christmas tree pattern” distribution

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herald patch (원발반)

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Christmas tree pattern rash

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Diagnosis

• clinical diagnosis is possible

• biopsy is needed for differential diagnosis

• herald patch: tinea versicolar/corporis

• rash: psoriasis, syphilid, viral exanthem, drug


eruption, lichen planus

• lab findings: normal

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Treatment

• spontaneous regression after 6~8 wks

• conservative treatment

• low potency steroid lotion

• antihistamines

• UVB phototherapy: recalcitrant pityriasis rosea

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Pityriasis rubra pilaris
(모공홍색비강진)

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Epidemiology

• familial or acquired chronic skin disease

• bimodal age incidence

• familial type: early childhood

• acquired type: fifth~sixth decades

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Etiology

• poorly understood

• vitamin A deficiency

• deficiency of retinol binding protein

• altered immune response

• genetic factors: AD inheritance

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Clinical manifestation

• scaling erythroderma

• nappes claires: islands of normal skin

• follicular hyperkeratosis

• palmoplantar keratoderma

• nail change: subungual hyperkeratosis, splinter


hemorrhage, yellowish discoloration

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childhood PRP
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follicular papules in PRP

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nappes claires: clear island like normal skin in erythroderma

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orange-yellow tint sandal-like keratoderma

Palmoplantar keratoderma in PRP

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Diagnosis

• clinical findings

• follicular papules with keratin plug

• keratoderma

• biopsy

• differential diagnosis

• psoriasis, ichthyosis

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Treatment

• topicals: keratolytics, steroids

• topical vitamin D: calcipotriol

• retinoids: isotretinoin, acitretin

• methotrexate

• UVB phototherapy: not helpful

• persists throughout life with fluctuation

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Lichen planus
(편평태선)

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Clinical manifestations

• violaceous, flat-topped, polygonal papule

• Wickham striae

• Koebner phenomenon (+)

• distribution: wrist, flexors of extremity, oral cavity,


glans penis

• itching: severe

• oral lesion: painful

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Clinical types of lichen planus
• acute widespread
• chronic localized
• hypertrophic
• atrophic
• vesiculo-bullous
• follicular (lichen planopilaris)
• annular
• actinic
• hepatitis-associated

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Wickham striae

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oral lichen planus - lace like pattern

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Etiology

• idiopathic

• immune reaction to viruses: HBV, HCV

• drugs: drug induced lichen planus

• β blockers, gold, antimalarials

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Diagnosis

• clinical findings

• biopsy

• differential diagnosis

• psoriasis
• syphilid
• lichenoid drug eruption
• oral lesion: leukoplakia, candidiasis

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Treatment

• no specific treatment

• topical steroids

• oral lesion: intralesional injection of steroids

• antihistamine for pruritus

• systemic treatment: cyclosporine / phototherapy

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Pityriasis alba
(백색잔비늘증, 백색비강진)

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• common benign condition of preadolescent
children

• affecting head and neck

• pink patch fading into a hypopigmented spot


with powdery scale

• no subjective symptoms

• self-limited disease with several months

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Pityriasis alba
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Thank you for your attentions.

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