other papulosquamous disorders

Other Papulosquamous Disorders

PsoriasisPityriasis

rosea

Papulosquamous disorders

LichenPlanus

Lichen nitidus

Seborrheic dermatitis

Pityriasis rubra pilaris

Exfoliative dermatitis

Lichen striatus

Parapsoriasis, small / large

plaque

Pityriasis lichenoides

Other important papulosquamous diseases

1. Mycosis fungoides (cutaneous T-cell lymphoma)2. Discoid lupus erythematosus3. Subacute cutaneous lupus erythematosus4. Tinea corporis5. Nummular eczema6. Secondary syphilis7. Drug eruptions8. Erythema dyschromicum perstans9. Keratosis lichenoides chronica10. Lichen sclerosus11. Lichenoid dermatitis12. Lichenoid reaction of graft-versus-host disease13. Extramammary Paget’s disease

Today’s Topics

• Pityriasis Rosea• Pityriasis Rubra Pilaris (PRP) • Parapsoriasis • Pityriasis Lichenoides (Acute and chronic)

Pityriasis Rosea

Pityriasis Rosea (PR)• Definition• Epidemiology• Etiology• Clinical Picture• Differential Diagnoses• Histological features• Treatment

Definition

• IT is common, self limited acute papulosquamous disorder often with distinctive and constant course lasting from 4-10 weeks.

• Pityriasis: Any of several skin diseases marked by the formation and desquamation of fine scales.

• Rosea: rose colored or pink.• Benign Self limiting but associated with increased miscarriage in first

15 wks. of pregnancy.

Epidemiology

Worldwide distribution.AGE: Predominantly occur in adolescents and young adults 10-35 years but

can occur rarely in infants and old persons.SEX: F>M.

SEASON: more in spring & autumn.Most cases of pityriasis rosea (PR) are sporadic.

Etiology

Exact cause unknown but may be due to;i. INFECTIONS: HHV-6, HHV-7 infections but

viral DNA couldn’t be detected from the lesion. Not contagious & gives life long

immunity after outbreak.ii. DRUGS: Arsenic, Adalimumab, Barbiturates,

Bismuth, Captopril, Clonidine, D-penicillamine, Etanercept, Gold, Isotretinoin,

Ketotifen, Lithium, Metronidazole, Nortriptyline, Omeprazole, Terbinafine.

Clinical Picture

Clinical Picture

I. PRODROMEMay or may not be present only 5% of patients.

FeverMalaise

HeadacheMild constitutional symptoms

Respiratory infection

Clinical PictureII. “HERALD PATCH” OR MOTHER PATCH

Seen in 50-80% of cases.Preceding exanthem.

Raised plaque or patch.Large (2 – 10 cm).

Usually single but may be multiple.Usually oval.

Pink or salmon pink.Collarette scales points inwards just inside the well-demarcated border.

Central clearing occasionally & slightly raised advancing margin (mimic tinea).Usually on the trunk.

Herald Patch

Clinical PictureIII. SECONDARY ERUPTION (EXANTHEM)

Appear within 1-2 weeks.May be asymptomatic or pruritic 25-75%.

Mainly of 2 types:1. Small rounded papules with or w/o fine scaling that ↑

in number & spread peripherally.2. Similar lesions to herald patch but smaller (1-2 cm):• Appear as discrete bilateral symmetrical oval salmon

pink in color on the trunk and proximal aspects of the extremities with peripheral collarette scales.

• Run along lines of cleavage (Langer’s lines) /parallel to the ribs giving fir tree or Christmas tree pattern.

Secondary eruption

Langer’s Lines

Christmas tree pattern

Clinical Picture

IV. FADING OF THE LESIONS:Spontaneous remission occurs within 4 to 8 weeks.

Occasionally lasts for 5 months or more.Postinflammatory pigment changes can be observed. Both

hypopigmentation and hyperpigmentation can follow the rash.Recurrences are uncommon.

Mnemonic

Atypical Variants of Pityriasis Rosea

20% of patients.Atypical morphology, distribution, or both.


1. Inverse PR:

Involving the axilla,

groin, hands, feet & may the face. It is

more common in

younger children &

in those with darkly pigmented

skin.

2. Drug-induced

PR: herald patch may be absent.

3. Oral lesions of

various types,

including erythematous plaques,

punctate hemorrh-age, and ulcers.

4. Pityriasis

rosea irritata: usually

results from irritation

and sweating, often as a consequ-ence of

inadequate treatment.

5. Abortive:

herald patch may be the sole manifestation of the

disease and is not

followed by the typical

rash.

6. Vesicular

PR.

7. Erythema multiform

e–like.


8. Papular

PR: scaling papules in the normal

distribution; more

common in young

children, pregnant

women, & black people

(central Hyperpigmen

tation & follicular papules).

9. Pustular PR.

10. Purpuric

PR.

11. Photosensi

tive PR.

12. Urticarial

PR.

13. Unilateral PR: in which

the lesions do not cross the midline.

14.limb-

girdle PR: atypical large patches that tend to be fewer in

number and coalescent.

Atypical Variants of PR

In dark individuals

Differential Diagnoses

1. Guttate psoriasis2. Small plaque parapsoriasis

3. PLEVA 4. PLC (especially if persistent)

5. PRP6. P. alba

7. Tinea corporis 8. Tinea Versicolor

9. Seborrhoeic dermatitis10. Discoid dermatitis11. Viral Exanthems12. Drug eruptions

13. Secondary syphilis14. Erythema multiforme

Histological features


1. Patchy epidermal spongiosis & lymphocytes

2. Lymphocytes surrounding dermal vessels

3. Extravasated red blood cells4. Patchy hyperkeratosis &

parakeratosis


1. Focal (Patchy) or diffuse parakeratosis.2. Absence of granular layer.

3. Mild acanthosis.4. Focal spongiosis.

5. Occasional dyskeratotic cells with an eosinophilic homogeneous appearance. 6. Exocytosis.

7. Perivascular dermal infiltration with lymphocytes.8. Edema of dermis.

9. Often some extravasated red blood cells.10. Increase in eosinophils in drug induced PR.

Treatment

1. Assurance: Since it is self limited no treatment is required.2. Antipruritic lotions.

3. Antihistamines: Symptomatic relief from itching. 4. Topical steroids: Low-mid strength.

5. Systemic steroids: Short course.6. Erythromycin: 1 gm q.i.d for 2 weeks.

7. Phototherapy: NB-UVB light may hasten the disappearance or natural sunlight if treatment is begun in the first week of eruption.

8. Acyclovir: Standard dose not effective.High dose 800 mg five times/d for 1 week may give rapid clearance of the

lesions.

Pityriasis Rubra Pilaris

Pityriasis Rubra Pilaris (PRP)• Definition• Epidemiology• Etiology• Clinical Picture• Classification• Differential Diagnoses• Histological features• Treatment

Definition

A rare chronic papulosquamous skin disease characterized by the appearance of keratotic follicular papules, salmon-colored

erythematous plaques interspersed with distinct islands of uninvolved skin, and palmoplantar keratoderma.

Epidemiology

Rare.M=F.

Nearly all cases are acquired sporadic, with occasional reports of a familial inherited form.

Familial cases show autosomal dominant inheritance with incomplete penetrance and variable expression. Familial PRP usually presents at birth or appears during the first years of

life and runs a chronic course.

Etiology

• The actual cause of PRP is unknown. • May be due to:

1. Dysfunction in keratinization or vitamin A metabolism.2. An autoimmune pathogenesis3. An abnormal immunologic response to particular antigens.4. Physical trigger.

Clinical PictureCRITERIA FOR SPOT DIAGNOSIS:

1. Coalescence scaling orange–red )salmon color) plaques with sharp borders covered with fine powdery scales & islands of sparing (nappes claires) in-between.

2. Follicular papules with an erythematous base especially on the dorsal aspect of the proximal fingers (nutmeg grater appearance).

3. An orange–red waxy keratoderma of the palms and soles in most patients.

Clinical Picture

The plaques may progress to an erythroderma with varying degrees of exfoliation.Erythema with a fine diffuse scale is often seen on the scalp.

Nail changes include1. Distal yellow-brown discoloration2. Subungual hyperkeratosis3. Longitudinal ridging

The mucous membranes are rarely involved, but they may show features similar to oral lichen planus.

Pruritus and burning in 20% of cases.Mild ectropion may develop when the face becomes uniformly erythematous.

Both photoaggravated and phototriggered forms of PRP can also occur.

4. Nail plate thickening5. Splinter hemorrhages 6. Nail dystrophy and shedding may occur.

Classification

According to Griffiths classification there are 6 types of PRP.According to age group and typicality of clinical presentation.

Type I/Classic adult PRP

Type II/Atypical adult PRP

Type III/Classic juvenile PRP

Type IV/Circumscribed juvenile PRP

Type V/Atypical juvenile PRP

Clinical type % of patients Age at onset Distribution Skin findings Course

I (classical adult) 50 Peak in the 6th

decade

Generalized, beginning on the head & neck then spreading caudally

•Red-orange plaques, confluent with islands of sparing•Perifollicular papules with keratotic plugs•Diffuse, waxy PPK

Often resolves within 3 years

II (atypical adult) 5 Adults of various ages Generalized

•Areas of eczematous dermatitis•Ichthyosiform scale on the lower extremities•PPK with lamellated scale•Alopecia

Chronic intractable

III (classic juvenile) 10

Peaks in the first few years of life and the late teens

Generalized •Similar to type I (see above)•<50% have palmoplantar involvement

Often resolves within 3 years

IV (circumscribed

juvenile)25 Prepubertal Focal, favoring the

elbows and knees

•Well-circumscribed, scaly, erythematous plaques•Perifollicular papules with keratotic plugs

Variable Some cases clear in the late teens

V (atypical juvenile)

5 First few years of life Generalized•Ichthyosiform dermatitis •Perifollicular papules with keratotic plugs•Scleroderma-like appearance of hands & feet•Accounts for most familial cases of PRP

Chronic intractable

VI(HIV-associated) NA Variable Generalized

•Similar to type I (see above)•Associated with acne conglobata, hidradinitis suppurativa, and lichen spinulosus

Refractory May respond to HAART


1. Psoriasis.2. Erythroderma.

3. Seborrheic dermatitis (Early cases).4. Dermatomyositis.

5. Cutaneous T-cell lymphoma.6. Erythrokeratodermia variabilis

7. Kawasaki disease (Children with acute-onset PRP).

Histological featuresPSORIASIFORM DERMATITIS:

1. Biopsy from non-follicular lesion consists of distinctive orthokeratosis and parakeratosis alternating in both vertical and horizontal directions (checkerboard pattern).

2. The hair follicles are dilated and filled with a keratinous plug, while the “shoulder effect” of stratum corneum surrounding the follicular opening frequently shows parakeratosis.

3. Focal or confluent hypergranulosis.4. Thick suprapapillary plate.

5. Occasionally, mild spongiosis. 6. Scattered intraepidermal lymphocytes.

7. Acantholysis and focal acantholytic dyskeratosis, may be present.8. Broad slightly elongated epidermal rete ridges, narrow dermal papillae.

9. Perivascular lymphohistiocytic infiltrate in the superficial dermis.10. Small numbers of plasma cells and eosinophils may be present.

Treatment

Empiric as the actual cause of PRP is unknown & in many patients there is spontaneous resolution.

The goals of treatment are to reduce morbidity and to prevent complications. Consider combination treatment.

FIRST LINE SECOND LINE

Topical1.Emollients reduce fissuring and

dryness.2.Keratolytics (salicylic acid, urea).3.Vitamin D3 (calcipotriol).

1.Glucocorticoids (medium to high potency).

2.Topical retinoids (tazarotene).3.Calcineurin inhibitors.

Photo(chemo)therapy(May respond well or may flare)

1.Narrowband UVB.2.Extracorporeal photopheresis.

1.Topical or Systemic PUVA.2.Ultraviolet A1.3.Broadband UVB.

Systemic

1.Oral retinoids: Currently they are the first line of therapy. Isotretinoin (1 to 1.5 mg/kg/day for 3–6 months), although acitretin (0.5 to 0.75 mg/kg per day) may be more effective in clearing lesions.

2.Methotrexate (10 to 25 mg weekly, IM or orally, once a week) has shown variable rates of success may be combination with a systemic retinoid.

3.Triple antiretroviral therapy (for HIV-associated variant).

1.Oral vitamin A: sometimes in combination with vitamins B and D.

2.Azathioprine (100 to 150 mg/day) but its effect is also inconsistent.

3.Cyclosporine (5 mg/kg/day) Several cases of adult-type PRP showed significant clearance in 2-4 wks.

4.Fumaric acid esters.5.Biological agents: TNF-αinhibitors

and Ustekinumab.

Treatment

Erythroderma with islands of sparing on the chest and abdomen and yellow palmar keratoderma with fissuring

Erythema and scaling subsided following 3 infusions of infliximab.

Parapsor ias i s

Parapsoriasis• Definition• Classification• Epidemiology• Etiology• Clinical Picture• Differential Diagnoses• Histological features• Treatment• Prognosis

Definition

Group of idiopathic chronic cutaneous diseases that can be characterized by scaly patches or slightly elevated papules and/or plaques that have

a resemblance to psoriasis.

Classification

A. Small plaque parapsoriasisB. Large plaque parapsoriasis

Epidemiology

Presentation most frequently is in middle age; peak incidence is in the fifth decade of life.

Small plaque parapsoriasis the male-to-female ratio is 3:1.

Etiology

• Exact cause is unknown.• T-cell–predominantly CD4+ infiltrates in the skin.• Small plaque parapsoriasis shows multiple dominant clones.• Large plaque parapsoriasis is a may be due to long-term antigen

stimulation & it is associated with a dominant T-cell clone, one that may represent up to 50% of the T-cell infiltrate.

• Human herpesvirus type 8 may be detected in skin lesions of large plaque parapsoriasis and the significance is unclear.

Clinical Picture

Onset of parapsoriasis is indolent.Asymptomatic or mildly pruritic.

Composed of patches rather than plaques.Typically persistent and can slowly progress to become more extensive.

Favor more sun-protected sites.

Clinical Picture

SMALL PLAQUE PARAPSORIASIS Can last months to several years; the disease often

resolves spontaneously.Lesions are well-circumscribed, round–oval slightly scaly, light salmon-colored patches that measure <5 cm in diameter and are scattered over the trunk and

extremities.

Clinical Picture

SMALL PLAQUE PARAPSORIASIS Digitate pattern “Digitate Dermatosis” is a

distinctive form of small plaque disease that consists of palisading elongated fingerlike patches that

follow the dermatome and are most prominently displayed symmetrically on the lateral flank and

abdomen.They may measure 10 cm or more along their long

axis.

Digitate dermatosis

Clinical PictureLARGE PLAQUE PARAPSORIASIS

It is chronic and progresses over many years, sometimes decades. It may progress to CTCL.

Manifests as faint erythematous patches with arcuate geographic borders. Each lesion often is >5 cm in diameter. Lesions are scattered on the proximal extremities and the

trunk and often show a bathing-suit distribution.Surfaces of the lesions have a faint red-to-salmon color; show

flaky thin scales; and have an atrophic, cigarette-paper or tissue-paper, wrinkling quality or may show poikiloderma or

retiform (all retiform cases progress to overt MF).

Large plaque parapsoriasis

Retiform parapsoriasis


SMALL PLAQUE PARAPSORIASIS 1. Pityriasis rosea

2. Drug eruption, esp. PR-like3. Guttate psoriasis

4. PLC5. MF

6. 2ry syphilis7. Nummular dermatitis

LARGE PLAQUE PARAPSORIASIS 1. MF

2. Drug eruption, esp. MF-like3. Psoriasis

4. Causes of Poikiloderma a. Poikilodermatous autoimmune

connective tissue disease (e .g . dermatomyositis)

b. Poikilodermatous genodermatosesc. Chronic radiodermatitis

Laboratory studies

• Complete blood cell count with differential should be performed, and a high lymphocyte count or the presence of Sézary cells suggests mycosis fungoides.


SMALL PLAQUE PARAPSORIASIS Exhibit a mild, nonspecific spongiotic dermatitis, focal hyperkeratosis, parakeratosis mild superficial

perivascular lymphocytic infiltrate, scale crust, and occasional exocytosis. Lymphocytes are small and do not show atypical features. do not show histologic atypia to suggest malignant

transformation.

LARGE PLAQUE PARAPSORIASIS May have similar histologic findings or an interface lymphocytic infiltrate with a variable degree of lichenoid

features. Blood vessels are dilated, and melanophages can be present.

The epidermis shows flattening of the rete ridges when epidermal atrophy is prominent on clinical examination.

Acanthosis of the epidermis and irregular hyperkeratosis of the cornified layer are present. In contrast to small plaque parapsoriasis, spongiosis is absent.

Treatment

FIRST LINE

1. Assurance (small plaque parapsoriasis)

2. Emollients3. Topical corticosteroids (mid- to high-

potency)4. Topical coal tar products5. Phototherapy: Sunlight, Broadband or

Narrowband ultraviolet B6. Antihistamines (If there is pruritus)

SECOND LINE(Mainly for large-plaque parapsoriasis cases considered to be early MF & must be treated)

1. Topical bexarotene2. Topical imiquimod3. PUVA 4. Topical mechlorethamine (nitrogen

mustard)5. Topical carmustine (BCNU)6. Subcutaneous interferon-α

Prognosis

SMALL PLAQUE PARAPSORIASIS • Is a stable benign disorder that rarely if ever progresses. It lasts several

months to years and can spontaneously resolve.

LARGE PLAQUE PARAPSORIASIS • Is chronic & more ominous in that approximately 10-35% of patients

progress to CTCL. • It does not enter remission without treatment & requires closer follow-up. • Induration or epidermal atrophy should prompt a repeat skin biopsy to

consider a diagnosis of MF in evolution. The 5-year survival rate, however, still remains high and is greater than 90%.

Pityriasis Lichenoides

Pityriasis Lichenoides• Definition• Types• Epidemiology• Etiology• Clinical Picture• Differential Diagnoses• Histological features• Treatment

Definition

They are papular clonal T-cell disorders characterized by recurrent crops of spontaneously regressing erythematous papules.

Types

I. Pityriasis lichenoides et varioliformis acuta (PLEVA/Mucha–Habermann disease)

II. Pityriasis lichenoides chronica (PLC)

Epidemiology

Both are more prevalent in the pediatric population, but it affects patients in all age groups, races and geographic regions.

There is a male predominance.

Etiology

The exact etiology is unknown.May be response to foreign antigens such as infectious agents or drugs. The infiltrate is predominantly T cells (CD8+) that are often monoclonal

thus they are lymphoproliferative disorders.

Clinical Picture

• The acute and chronic forms of pityriasis lichenoides exist on a disease spectrum with variable presentations so many patients have intermediate or mixed manifestations, either serially or concurrently.

• Can resolve spontaneously after weeks to months or it may pursue a chronic relapsing course.

Clinical Picture

PLEVA PLCLesions • Vesicular, ulcerative, crusted or pustular. • Scaly erythematous to red–brown papules.

Course • Rapid. • More indolent.

Resolution • Within weeks may varioliform scars if dermal damage is extensive.

• Over weeks to months hypopigmented macules (more obvious in darkly pigmented individuals and may be the presenting C/O).

Extracut. manifestation

• Malaise, fever, lymphadenopathy, arthritis and/or bacteremia.

• “Febrile ulceronecrotic Mucha–Habermann disease (FUMHD)” severe variants with mucosal, gastrointestinal and pulmonary involvement.

• Absent.


PLEVA1. Lymphomatoid papulosis

2. Cutaneous small vessel vasculitis3. Lichenoid drug eruption

4. Arthropod reactions5. Varicella, enteroviral exanthems

6. Folliculitis7. Erythema multiforme

8. Dermatitis herpetiformis

PLC1. Small plaque parapsoriasis

2. Guttate psoriasis3. Lichen planus

4. Pityriasis rosea5. Secondary syphilis

6. Lymphomatoid papulosis7. Papular dermatitis

8. Lichenoid drug eruption


• Pityriasis lichenoides exhibits a superficial perivascular interface dermatitis in all cases.


PLEVA PLC

S. corneum• Hyperkeratosis• Foci of parakeratosis• Few neutrophils

• Laminated Hyperkeratosis• Foci of thin flat parakeratosis • w/o neutrophils

Granular cell layer • Deficient beneath mound of parakeratosis • Well formed

Prickle cell layer • From edema to extensive epidermal necrosis in well-developed lesions. • No or focal necrotic keratinocytes

Basal cell layer & dermo-epidermal

junction.• Marked vacuolar changes• Denser interface lymphocytic infiltrate.

• Very focal mild basal cell vacuolization which may not be evident in well-developed or resolving lesions.

• Milder interface lymphocytic infiltrate.

Dermis• Shows denser superficial & deep perivascular

wedge-shaped lymphocytic infiltrates.• Numerous erythrocyte extravasation.

• Only a superficial perivascular lymphocytic infiltrate.

• Mild erythrocyte extravasation.

Treatment

FIRST LINE1. Topical corticosteroids

2. Topical coal tar preparations3. Antibiotics (erythromycin 500 mg

PO 2-4× daily; azithromycin; tetracycline 500 mg PO 2-4× daily,

minocycline 100 mg PO twice daily)4. Phototherapy (Sunlight, ultraviolet

A, broadband or narrowband UVB)

SECOND LINE1. Topical tacrolimus2. Methotrexate (10-25 mg PO weekly)3. Phototherapy (ultraviolet AI, PUVA)4. Cyclosporine5. Biological agents: etanercept6. IVIg

Treatment

If a drug association is suspected, stop the offending drug.SPECIAL TREATMENTS:

1. Prednisone: (60/40/20 mg PO taper, 5 days each) If there is fever, arthritis or other systemic findings.

2. Antihistamines: if pruritus is present.3. Other Systemic antibiotics: If there is secondary infection.

REFERENCES• Bolognia 3rd ed• http://dermnetnz.org• Google images• Husein Oozeerally (Presentation)• Jonathan Faulkner (Presentation)• emdicine.com• dermis.net

THANK YOU

other papulosquamous disorders

Health & Medicine

cases of pityriasis

herald patch

atypical variants of

histological features1

atypical morphology

clinical pictureiii

clinical pictureiv

mother patch