Other papulosquamous disorders

Other Papulosquamous Disorders

Psoriasis
Pityriasis
rosea
Lichen
Planus
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 erythematosus
3. Subacute cutaneous lupus erythematosus
4. Tinea corporis
5. Nummular eczema
6. Secondary syphilis
7. Drug eruptions
8. Erythema dyschromicum perstans
9. Keratosis lichenoides chronica
10. Lichen sclerosus
11. Lichenoid dermatitis
12. Lichenoid reaction of graft-versus-host disease
13. Extramammary Paget’s disease

Today’s Topics
• Pityriasis Rosea
• Pityriasis Rubra Pilaris (PRP)
• Parapsoriasis
• Pityriasis Lichenoides (Acute and chronic)

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
I. PRODROME
• May or may not be present only 5% of patients.
• Fever
• Malaise
• Headache
• Mild constitutional symptoms
• Respiratory infection

Clinical Picture
II. “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.

Clinical Picture
III. 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.

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.

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
erythemato
us 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
manifestati
on 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.

Differential Diagnoses
1. Guttate psoriasis
2. Small plaque parapsoriasis
3. PLEVA
4. PLC (especially if persistent)
5. PRP
6. P. alba
7. Tinea corporis
8. Tinea Versicolor
9. Seborrhoeic dermatitis
10. Discoid dermatitis
11. Viral Exanthems
12. Drug eruptions
14. Erythema multiforme

Histological features
1. Patchy epidermal spongiosis
& lymphocytes
2. Lymphocytes surrounding
dermal vessels
3. Extravasated red blood cells
4. 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 (PRP)
• Definition
• Epidemiology
• Etiology
• Classification
• 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 pathogenesis
3. An abnormal immunologic response to particular antigens.
4. Physical trigger.

Clinical Picture
CRITERIA 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 include
1. Distal yellow-brown discoloration
2. Subungual hyperkeratosis
3. Longitudinal ridging
4. Nail plate thickening
5. Splinter hemorrhages
6. Nail dystrophy and shedding may occur.
• 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.

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

Type IV/Circumscribed 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
Variable
Some cases
clear in the
late teens
V
(atypical
juvenile)
5 First few years of life Generalized
•Ichthyosiform dermatitis
•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).

PSORIASIFORM 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
Topical
1.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.

Parapsoriasis
• Definition
• Classification
• Epidemiology
• Etiology
• 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 parapsoriasis
B. 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
• 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.

Clinical Picture
LARGE 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).

1. Pityriasis rosea
2. Drug eruption, esp. PR-like
4. PLC
5. MF
6. 2ry syphilis
7. Nummular dermatitis
1. MF
2. Drug eruption, esp. MF-like
3. Psoriasis
4. Causes of Poikiloderma
a. Poikilodermatous autoimmune
connective tissue disease (e .g .
dermatomyositis)
b. Poikilodermatous genodermatoses
c. 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.

• 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.
• 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. Emollients
3. Topical corticosteroids (mid- to high-potency)
4. Topical coal tar products
5. Phototherapy: Sunlight, Broadband
or Narrowband ultraviolet B
6. Antihistamines (If there is pruritus)
SECONDLINE
(Mainly for large-plaque parapsoriasis cases
considered to be early MF & must be treated)
1. Topical bexarotene
2. Topical imiquimod
3. PUVA
4. Topical mechlorethamine (nitrogen
mustard)
5. Topical carmustine (BCNU)
6. Subcutaneous interferon-α

Prognosis
• Is a stable benign disorder that rarely if ever progresses. It lasts several
months to years and can spontaneously resolve.
• 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
• Definition
• Types
• Epidemiology
• Etiology
• 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 PLC
Lesions • 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.

PLEVA
1. Lymphomatoid papulosis
2. Cutaneous small vessel vasculitis
3. Lichenoid drug eruption
4. Arthropod reactions
5. Varicella, enteroviral exanthems
6. Folliculitis
7. Erythema multiforme
8. Dermatitis herpetiformis
PLC
1. Small plaque parapsoriasis
3. Lichen planus
4. Pityriasis rosea
6. Lymphomatoid papulosis
7. 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 LINE
1. Topical corticosteroids
2. Topical coal tar preparations
3. 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 LINE
1. Topical tacrolimus
2. Methotrexate (10-25 mg PO weekly)
3. Phototherapy (ultraviolet AI, PUVA)
4. Cyclosporine
5. Biological agents: etanercept
6. 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

Other papulosquamous disorders

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