Fitzpatrick Lecture

KATRINA D. OLITOQUIT, MD
 Part 4
Psoriasiform Disorders

OUTLINE Part 5
Lichenoid/Granulomatous Disorders
PSORIASIFORM
DISORDERS
 SKIN DISORDERS

CLINICAL FEATURES ETIOLOGY AND DIFFERENTIAL MANAGEMENT

AND PE FINDINGS PATHOGENESIS DIAGNOSIS
PSORIASIS
• Common, immunologically mediated, inflammatory disease
characterized by skin inflammation, epidermal hyperplasia


• Equally common in males and females


• May begin at any age; most likely between of 15-30 years

• Human leukocyte antigen (HLA) class I antigens (HLA-Cw6) -

earlier age of onset and with a positive family history
PLAQUE TYPE PSORIASIS
Classic lesion : well-demarcated, raised, red plaque with a white scaly surface



• presents with "Auspitz sign"

• lesions are symmetric
• (+) Koebner phenomenon (isomorphic response)

Psoriasis vulgaris - most common; symmetrical, localized to the extensor aspects of the
extremities along with scalp, lower lumbosacral, buttocks, and genital involvement

Psoriasis Gyrata - may extend laterally and become circinate because of the confluence of several plaques

Annular Psoriasis - partial central clearing, resulting in ringlike lesions; good prognosis

Rupioid Psoriasis - lesions in the shape of a cone or limpet

Ostraceous psoriasis - ringlike, hyperkeratotic concave lesion, resembling an oyster shell

Elephantine psoriasis - uncommon; thickly scaling, large plaques, usually on the lower extremities

**Woronoff ring** - hypopigmented ring surrounding individual psoriatic lesions; associated with treatment,
most commonly UV radiation or topical corticosteroids
GUTTATE PSORIASIS
small papules over the upper trunk and proximal extremities

SMALL PLAQUE PSORIASIS

resembles guttate psoriasis

onset in older patients; chronic; larger lesions (typically 1–2 cm) that are thicker
and scalier

common adult-onset presentation of psoriasis in Korea and

other Asian countries

INVERSE PSORIASIS
localized in major skin folds

scaling is usually minimal or absent

glossy sharply demarcated erythema localized to areas of

skin to skin contact
 ERYTHRODERMIC PSORIASIS
affects all body sites; prominent feature is erythema
associated with hypothermia, lower extremity edema, high output cardiac failure,
and impaired hepatic and renal function
• worsening of chronic plaque type psoriasis
• sudden, generalized erythroderma or generalized koebner reaction from
non-tolerated external treatment

PUSTULAR PSORIASIS
• Generalized pustular psoriasis (Von Zumbusch type)
(+) fever and sterile pustules; associated with systemic signs and life threatening complications
• Exanthematic pustular psoriasis
(+) viral infection; widespread pustules with generalized plaque psoriasis;
no symptoms; tends not to recur
• Annular pustular psoriasis - pustules on ring-like erythema
• Impetigo herpetiformis - pregnancy (3rd trimester)
• Pustulosis palmaris et plantaris - localized on palms and soles; rare; females (median age
of 47 years old); assoc with psoriatic arthritis; (+) smoking
• Acrodermatitis continua of Hallopeau (dermatitis repens)
localized sterile pustular eruption of the fingers and toes; extremely rare
SEBOPSORIASIS
• erythematous plaques with greasy scales localized to
seborrheic areas

NAPKIN PSORIASIS
• begins between the ages of 3 and 6 months as a confluent red area
on the diaper area

LINEAR PSORIASIS
• psoriatic lesion presents as linear lesion most commonly on the limbs
 NAIL CHANGES
Nail pitting - most common
Onychodystrophy - stronger assoc with PsA
Oil spots and salmon patches - nearly specific

***splinter hemorrhages, subungual hyperkeratosis, onycholysis, anonychia***

IMMUNOPATHOGENESIS

EPIDERMAL T CELLS
• The cytokine profile of psoriatic lesions is rich in interferon (IFN)-y
• Indicative of T helper 1 (Th1) polarization of CD4+ cells, and T cytotoxic 1 (Tc1) polarization of
CD8+ cells
• CD4+ T cells, stimulated by interleukin (IL)-23 and characterized by production of IL-17 or IL-22
• HLAcw6 presents antigens to CD8+ T cells, which are MHC Class I restricted and comprise about
80% of the T cells in the epidermis of psoriatic lesions
• IL-23, IL-17 signaling
• Interferon signaling
• NF-kB signaling
• Keratinocyte responses
RISK FACTORS

• Obesity
• Smoking
• Infections - streptococcal throat infection and guttate psoriasis;
Hepatitis C infection
• Drugs - antimalarials, beta blockers, lithium, NSAIDS, IFN-a
and y, Imiquimod, ACEI, Gemfibrozil
 COURSE
and
PROGNOSIS • Younger age onset and positive family history - more widespread and recurrent disease
• Relapses occur weekly or monthly; others have more stable disease
• Frequent relapses develop more severe disease with rapidly enlarging lesions
• Osteoarthritis - extremely common; joint symptoms before 4th decade or history of warm,
swollen joints (PsA)
• Guttate psoriasis - self-limited; last 12-16 weeks without treatment; 1/3 develop chronic
plaque psoriasis
• Chronic plaque psoriasis - lifelong disease; unpredicting intervals
• Erythrodermic and Generalized Pustular Psoriasis - poorer prognosis; severe and
persistent
PITYRIASIS RUBRA • a rare, inflammatory skin disease of juvenile
PILARIS or adult onset with distinctive clinical
features and a self-limiting or chronic
evolution
• sporadic; unknown etiology
• 6.5% of cases of PRP are familial
• CARD14 mutations
 ETIOLOGY
AND
PATHOGENESIS • Pathogenic mechanisms associated with infection, such as upper
respiratory tract infection and HIV infection, were proposed. 

• In type V PRP, gain-of-function mutations in the CARD14 gene
linked to autosomal dominant inheritance have been identified.

• In lesional PRP, upregulated expression levels were found for
most pro-inflammatory innate cytokines, including TNF, IL-6, IL-12,
IL-23, and IL-1B.
• An increase of TH1 cytokines and, in particular, TH17 cytokines
IL-17A, IL1-7F, and IL-22 was seen.
 • Also known as "parapsoriasis en plaques"
PARAPSORIASIS • Affects mainly adults (middle-aged and older people;
peak incidence of 5th decade)
• Large-plaque parapsoriasis (LPP) and small-plaque
parapsoriasis (SPP) are recognized.
• Acute and chronic forms of pityriasis lichenoides
known today as Pityriasis lichenoides et varioliformis
acuta [PLEVA] and Pityriasis lichenoides chronica
[PLC]
• SPP - male predominance
LARGE PLAQUE PSORIASIS
• oval or irregularly shaped patches or very thin plaques that are asymptomatic or mildly pruritic
• found mainly on the “bathing trunk” and flexural areas
• light red brown or salmon pink, and their surface is covered with small and scanty scales
• Lesions may appear finely wrinkled—”cigarette paper” wrinkling
• Poikiloderma or poikiloderma atrophicans vasculare - triad of atrophy, mottled pigmentation, and
telangiectasia

RETIFORM PARAPSORIASIS - extensive eruption of scaly macules and papules

in a net-like or zebra-stripe pattern that eventually becomes poikilodermatous

SMALL PLAQUE PSORIASIS

• "chronic superficial dermatitis"
• round or oval discrete patches or very thin plaques, mainly on the trunk
• they are asymptomatic and covered with fine, moderately adherent scales




DIGITATE DERMATOSIS - yellowish or fawn-colored lesions; follows lines of cleavage of
the skin and gives the appearance of a hug that left fingerprints on the trunk
 ETIOLOGY
AND
PATHOGENESIS

• Skin-associated lymphoid tissue (SALT)

• All types are cutaneous T-cell lymphoproliferative disorders:
LPP, SPP, and Pityriasis Lichenoides have shown to be monoclonal
disorders
COMPLICATIONS

• LPP can be associated with other forms of parapsoriasis and overt cutaneous
lymphomas


• Data from a recent Danish cohort study suggests that parapsoriasis and mycosis
fungoides are associated with an increased risk for cardiovascular disease as well
as increased risk for subsequent hematologic and nonhematologic malignancies
and increased mortality


 COURSE
and
PROGNOSIS
• Both LPP and SPP may persist for years to decades
• 10% to 30% of cases of LPP progress to overt MF
• LPP represents the clinically benign end of the MF disease spectrum, 

with transformation to large cell lymphoma at the opposite extreme
• Retiform variant is said to progress to overt MF in most cases
• SPP is a clinically benign disorder
The goal of treatment is to suppress the disorder
to prevent possible progression to overt MF.



The patient should be examined carefully every 3

months initially and every 6 months to 1 year
subsequently for evidence of progression.



Repeated multiple biopsies of suspicious lesions

should be performed.


PITYRIASIS LICHENOIDES
• children and young adults but can affect all ages with seasonal variation in onset favoring fall and winter
• male predominance
• PLC is 3 to 6 times more common than PLEVA
• tend to concentrate on the trunk and proximal extremities, but any region of the skin, even mucous
membranes

PLC - recurrent crops of erythematous scaly papules that spontaneously regress over
several weeks to months; CD8+ or CD4+ cells predominate

PLEVA - recurrent crops of erythematous papules that develop

crusts, vesicles, pustules, or erosions before spontaneously
regressing within a matter of weeks

***Pityriasis lichenoides with ulceronecrosis and hyperthermia

(PLUH) or Febrile ulceronecrotic Mucha–Habermann
disease (FUMHD)*** - more severe ulcerative variant; presents as purpuric papulo-nodules with
central ulcers
 ETIOLOGY
AND
PATHOGENESIS
• The etiology of pityriasis lichenoides is unknown.
• Infection
• Medications
• Reduction in CD1A+ antigen-presenting dendritic (Langerhans) cells within the
central epidermis of pityriasis lichenoides lesions.
• Keratinocytes and endothelial cells are HLA-DR+, which sugguests activation by
T-cell cytokines
COMPLICATIONS

• Secondary infection is the most common complication

• PLEVA may be associated with low-grade fever, malaise, headache,
and arthralgia.
• PLUH or FUMHD can develop high fever, malaise, myalgia, arthralgia,
gastrointestinal, and central nervous system symptoms which can
occasionally be fatal.
 COURSE
and
PROGNOSIS
• The disorder may resolve spontaneously within a 

few months or, less commonly, persist for years. 



• PLEVA usually has a shorter duration than PLC

The mainstay of traditional therapy has been a combination of
topical corticosteroids and phototherapy

Systemic antibiotics, such as tetracyclines,

erythromycin, and azithromycin, are used primarily for their
anti-inflammatory rather than antibiotic effects.



Cases with minimal disease activity may not require any

treatment.


PITYRIASIS ROSEA
• Common self-limited papulosquamous eruption typically lasting 5 to 8 weeks
• Most common in teenagers and young adults (between the ages of 10-35 years)
• Begins with a solitary lesion on the trunk "herald patch" which remains isolated on
average for 2 weeks in adults and 4 days in children
• Followed by the onset of a secondary eruption of morphologically similar but smaller
lesions on the trunk and proximal extremities.
• Prodromal symptoms can occur
• Pruritus is severe in 25%, mild to moderate in 50%, and absent in 25% of patients
• Lesions are aligned with their long axis parallel to lines of cleavage giving a
“Christmas tree” distribution on the upper chest and back
 RELAPSING FORM
• single episode of relapse within 1 year of the initial episode
• the secondary episodes generally lack a herald patch, are shorter lived, and consist of fewer and more
localized lesions than the initial eruption.


PERSISTENT FORM
• eruption last for greater than 12 weeks without interruption
• it commonly has a herald patch, and oral manifestations


CLINICAL FINDINGS:
Herald Patch
• presents as a well-demarcated, thin, oval to round plaque that is usually pink, rose colored, erythematous,
or, less commonly, hyperpigmented.
• It frequently has a slightly depressed center and fine collarette of scale within the periphery of the lesion
• Sites: trunk (50%) followed by the extremities, and neck




Secondary Eruption
• multiple, round-to-oval, papules, and plaques; are often light pink with a fine collarette of scale and
resemble the herald patch in miniature.
• Sites: found bilaterally and symmetrically on the trunk and proximal extremities


 ETIOLOGY
AND
PATHOGENESIS
• HHV7> HHV6
• One study demonstrated that the inflammatory infiltrate of
PR was predominantly T cells with an increased CD4 to
CD8 ratio as well as an increased proportion of
Langerhans cells
3 ESSENTIAL FEATURES:

• discrete circular or oval lesions

• scaling on most lesions
• peripheral collarette of scale with central clearance
on at least 2 lesions


OPTIONAL CRITERIA:
• truncal and proximal limb distribution with less than 10% of lesions
distal to the mid-upper-arm and mid-thighs
• distribution of most lesions along the ribs
• herald patch appearing at least 2 days before the eruption
 COURSE
and
PROGNOSIS
• Typically self-resolves after an average of 45 days (ranges from 2
weeks to 5 months)
• Eruptions lasting longer than 3 months may fit into the proposed
category of persistent PR
• Upon resolution: post-inflammatory hyper/hypopigmentation
• No other significant long-term clinical outcomes from PR.
• Recurrence can occur (uncommon)
TREATMENT

Self-limited and therefore no treatment is necessary in many cases


• Topical Steroids
• Antihistamines
• Macrolide
• Acyclovir
 LICHENOID &
GRANULOMATOUS
DISORDERS
LICHEN PLANUS
● Lesions are well-marginated, flat-topped, red-violet polygonal papules; papules are
grouped and often coalesce into plaques
● Symmetrical and grouped lesions affecting the flexural aspects of the arms and legs.
● Wickham striae (highly characteristic), fine, white and adherent reticulate scale,
are noted in well-developed lesions.
● Exhibits Koebner phenomenon - acute setting
● ⅔ of cases present during 30-60 years - peak onset at 55-74 years; age of onset
earlier in women
● Childhood LP (1-5%)
4 P's
Purple
Polygonal
Pruritic
Papules
 CLINICAL VARIANTS

ANNULAR LINEAR, HYPERTROPHIC ATROPHIC VESICULOBULLOUS

BLASCHKOID, & • rare; secondary to an
• highly pruritic, refractory
• oligolesional dse
• annular lesions ZOSTERIFORM to treatment, and exuberant
• 10% • well-marginated,
associated with relapse inflammatory response
• arcuate grouping thickened, elevated, blue-white
• and an exaggerated
of individual • linear pattern purple-red, papules or
Max-Joseph space
papules coalescing secondary to trauma hyperkeratotic plaques plaques with
• sites: lower
to form with a • 0.2% may follow lines and nodules central atrophy
extremities
central clearing of Blaschko. • sites: anterior shins and • sites: proximal
• tend to occur in acute
• Related to IPJs lower extremity
• sites: penis and flares of lichen planus
• verrucal lesions and trunk
scrotum postzygotic, somatic
• follicular accentuation,
mutations • ddx: lichen
elevation, and chalk-like
• Varicella zoster - sclerosus et ***Oral vesiculobullous LP***
scale
chronic venous atrophicus and • often symptomatic and leads
zosteriform LP •
to erosion & ulceration
insufficiency - common MF

EROSIVE &
ULCERATIVE
• significant pain and
scarring
• have other ectodermal
involvement - aids in the
dx.
• scarring alopecia and
loss of the toenails
• assoc with SCC - has
chronic lesions ***Lichen planus follicularis tumidus***
• sites: feet and oral
cavity
CLINICAL VARIANTS
FOLLICULAR LICHEN ACTINIC NAIL


PLANUS
• alone or in assoc with other
• subtropical countries
cutaneous or mucosal PIGMENTOSUS • young individuals of
• classic nail lichen
forms of LP
 planus, 20-nail
Middle Eastern descent; dystrophy and
• site: scalp
MC in the spring and idiopathic atrophy of
• 3 variants : lichen • hyperpigmented,
• summer months.
planopilaris, frontal dark-brown macules the nails.
• site: face
fibrosing alopecia, and • site: sun-exposed and • MC findings are
• annular,
Gram-Little- Piccardi-
flexural folds diffuse nail
well-marginated,
Lassueur syndrome involvement with
• more common in hyperpigmented
(GLPLS). 
 thinning, longitudinal
darker skinned brown-violet, flat-topped,

 ridging, and distal
individuals plaques with a slightly
rolled border nail splitting
• rare: pruritic, red-violet • ddx: ashy dermatosis
pseudo-tumoral facial and posterior • minimally symptomatic
auricular plaques with yellow cysts Pterygium is a classic finding in
• classic lesions - non–SE
• ddx: folliculotropic MF, and nail lichen planus
cutaneous LE areas
 LICHEN PLANUS OF THE SCALP
LICHEN PLANOPILARIS
• scarring alopecia
• vertex scalp
• diffuse erythema with perifollicular hyperkeratosis and livid
erythema

FRONTAL FIBROSING ALOPECIA

• frontotemporal recession caused by inflammatory destruction
of the hair follicles; slow progression over years
• 75% of women - concomitant loss of the eyebrows
• Postmenopausal women

GLPLS
• rare subtype
• cicatricial alopecia of the scalp & nonscarring alopecia of the
axilla and groin
• follicular papules on the trunk and extremities

PSEUDOPELADE OF BROCQ
• rare; scarring alopecia and fibrosis
• distinct pathologic features are absent
• end stage of follicular fibrosis caused by a primary inflammatory dermatosis
 • Affects mouth or genitalia

MUCOSAL
• Types of oral LP: reticular (MC - asymptomatic),
plaque-like, atrophic, papular, erosive or ulcerative, and
bullous forms
• MC sites: buccal mucosa, followed by the tongue and

LICHEN PLANUS
gingiva
• Erosive and ulcerative oral LP - tongue; extremely
painful
• Male genitalia are involved in 25% (MC site: glans penis)
• Anal lesions - leukokeratosis, hyperkeratosis, fissuring,
and erosions
• Vulvar and vaginal LP is present in 25% to 60% of
patients with oral LP
• Conjunctival LP - cicatricial conjunctivitis
 INVERSE LICHEN PLANUS
• red-brown, discrete papules and flat-topped plaques
• Sites: axillae, inframammary region and groin
• absence of involvement in sun-exposed areas
• aka lichen planus pigmentosus inversus

PALMOPLANTAR LICHEN PLANUS

• red-purple, scaly plaques with or without hyperkeratosis;
absent Wickham striae
• Sites: internal plantar arch on the feet and the thenar
and hypothenar eminence on the hands
 Drug-Induced Lichen Planus

SPECIAL FORMS Lichen Planus–Lupus Erythematosus

Overlap


Lichen Planus Pemphigoides

Keratosis Lichenoides Chronica

(Nekam Disease)


Lichenoid Graft versus Host
Disease

Lichenoid Keratosis

Lichenoid Dermatitis
 ETIOLOGY
AND
PATHOGENESIS
• Unknown pathogenesis
• Infection, immune, metabolic, and genetic causes
• CD8 T cell - effector cell of lichen planus
• Antigen Recognition -an integral role of LCs, keratinocytes, and CD4 T
helper cells has an integral role in antigen
• Presentation as well as the initiation and propagation of the Th1 response
• Lymphocyte Activation
• Keratinocyte Apoptosis
• Genetic/Epigenetic Regulation
CXCR-3 ligand, CXCL-9 - most specific marker
 COURSE
and
PROGNOSIS • Tends to be a self-resolving disease; however, there is a paucity of research
into the resolution phase of disease.

• Heals with post-inflammatory hyperpigmentation - common in darker
skinned individuals

• Most cutaneous LP resolves within 1-2 years and may be associated with
relapses.
• Recurrence - 20% of cases (more common in generalized cutaneous
disease)
• Malignant transformation (Oral LP) - low; SCC; HPV16; MC site-tongue
• RF: long-standing disease, erosive or atrophic types, tobacco use, and
possibly esophageal involvement.
 Skin directed therapies
• Topical corticosteroids
• Topical Calcineurin Inhibitors
• Intralesional corticosteroids - resistant and hypertrophic LP
• Phototherapy



TREATMENT Systemic therapies

• Systemic corticosteroids

(Cutaneous LP) •
•
Sulfasalazine
Metronidazole - first line non-immunosuppressive systemic agent
• Acitretin - hypertrophic LP
• Antimalarials - actinic LP
• Methotrexate - hypertrophic LP and LPP
• Mycophenolate mofetil - refractory cutaneous LP
• Azathioprine
• Cyclosporine
 The cornerstone of treatment in oral LP is
good oral hygiene with regular professional dental cleanings.


Skin directed therapies
• Topical corticosteroids
• Topical Calcineurin Inhibitors
• Intralesional corticosteroids

TREATMENT • Retinoids

Systemic therapies
(Oral LP) •
•
Oral corticosteroids
Oral retinoids
• Antimalarials - Hydroxycholorquine
• Methotrexate
• Mycophenolate mofetil
• Cyclosporine
• Other therapies
TREATMENT
● Skin directed therapies - high potency steroids
Lichen Planopilaris ● Systemic therapies - Hydroxychloroquine

● Skin directed therapies – high potency

Frontal Fibrosing steroids / intralesional steroids
● Systemic therapies - Oral 5α-reductase
Alopecia
inhibitors, Hydroxychloroquine

Nail Lichen Planus ● Ultrapotent topical and intralesional

corticosteroids
LICHEN NITIDUS

• Composed of multiple 1- to 2-mm, discrete, smooth, round, skin-colored papules; may be umbilicated
with a glistening appearance.
• Generalized lichen nitidus - pruritic, grouped papules, (+) Koebner phenomenon
• Most frequent sites: trunk, genitalia, face, neck, hands, and lower extremities
• Linear, Blaschkoid, generalized, and actinic disease
• Nail abnormalities with palmar disease - longitudinal, beaded ridging, and terminal splitting with or
without irregular pitting
• Actinic Lichen nitidus - due to repetitive exposure to sun
• 1% in lichen planus
 ETIOLOGY
AND
PATHOGENESIS
• Regarded as an idiopathic lichenoid tissue reaction with
distinctive clinical and histologic features


• Theory: exogenous antigens and allergens stimulate epidermal
and dermal antigen-presenting cells (eg, Langerhans cells) to
activate a cell-mediated response, initiate lymphocyte
accumulation, and form discrete inflammatory papules
 COURSE
and
PROGNOSIS
• Typically a focal, asymptomatic, chronic inflammatory
reaction
• Eventually resolves spontaneously within 1 year in two
thirds of patients or, less frequently, over a few years
LICHEN STRIATUS

• Rare, idiopathic, linear dermatosis; <18 years with a mean age of onset between
3 and 5 years of age
• Sudden onset of flat-topped, 1- to 3-mm, pink, tan, or hypopigmented papules in
a linear configuration or Blaschkoid distribution
• Pruritus - 5% to 34% of cases; atopic individuals and adults
• MC sites: extremities and trunk ; Less commonly, the face, and nails
• Nail involvement - uncommon; longitudinal ridging, onycholysis, splitting, fraying,
and loss of the nail plate (reversible with treatment)
 ETIOLOGY
AND
PATHOGENESIS

• Blaschko-linear pattern suggests somatic mosaicism

• External precipitants have been implicated in the loss of
tolerance and triggering an immune response.
• Possible precipitating factors include environmental stressors,
drugs ( specifically interferon), vaccines (bacillus
Calmette–Guérin), viral antigens, hypersensitivity, and skin injury
 COURSE
AND
PROGNOSIS
• Self limited disease (approximately 6 to 12 months)
• Post-inflammatory hypopigmentation - 50% of cases;
self-limited; lesions heal without scarring


o
TREATMENT

• Topical steroids
• Topical calcineurin inhibitors
• Oral antihistamines
GRANULOMA
ANNULARE

• Relatively common disorder; the exact prevalence is unknown; occurs more

often in children and young adults
• Most cases are sporadic
• Typical history is of one or more papules with centrifugal enlargement and
central clearing
• Generalized or disseminated variant - more common in adults
 CLINICAL VARIANTS

LOCALIZED GENERALIZED SUBCUTANEOUS PERFORATING PATCH

• MC form - • 8-15% of cases; • predominantly • rare • Macular

adults in children • superficial small
annular lesions
• wide- spread papules develop
• skin colored, • firm to hard, • erythematous,
papules, coalesce central
erythematous, or usually red- brown, or
to form umbilication or
violaceous asymptomatic
• small annular crusting, and there violaceous
• annular margin plaques or larger nodules may be discharge patches
is firm to discolored patches • MC site: of a creamy fluid
without an
palpation with raised • Sites:
anterior lower annular rim
• Sites: dorsal • arcuate and localized-dorsal
legs (pretibial)
hands and feet, serpiginous margin hands and fingers
lower limbs, • Sites: trunk, neck or generalized
wrists and extremities -trunk and
extremities
 ETIOLOGY
AND
PATHOGENESIS
Predisposing events
• Non specific trauma
• Sun exposure,
• Drugs
Pathogenic mechanisms
• a primary degenerative process of connective tissue initiating granulomatous inflammation
• a lymphocyte-mediated immune reaction resulting in macrophage activation and cytokine-mediated
degradation of connective tissue
• a subtle vasculitis or other microangiopathy leading to tissue injury
Systemic disorders
• Diabetes mellitus and Thyroid disease, Malignancy, and Dyslipidemia
 COURSE
AND
PROGNOSIS
• Most cases resolve spontaneously without sequelae.
• May clear within a few weeks; majority disappear within 2 years.
• Recurrence - may develop months or even years later (frequently at the
same site)
• Generalized granuloma annulare - more protracted course 

• Perforating granuloma annulare - scarring
TREATMENT
SARCOIDOSIS

• Has replaced syphilis as the "great mimicker"

• A multisystem disease; granulomatous inflammation of unknown
etiology commonly occurring in the lung and the skin, but any organ
system can be affected
• Affects third decade of life; slightly higher in women
• Highest prevalence - Denmark and Sweden; African descents in US
PAPULES AND PLAQUES
• MC form - papular form
• firm 1- to 5-mm papules;
often have a translucent
red-brown or yellow-brown
color
• “APPLE JELLY” (on diascopy)-
NOT pathognomonic
SUBCUTANEOUS NODULES
(Darier-Roussy Sarcoid)
• nodules may be tender or painless
• Site: extremities
NAIL FINDINGS
• deformation and
discoloration
LUPUS PERNIO SCAR SARCOID
• symmetric, violaceous, indurated
plaques and nodules
 • within scar tissue, at
• high incidence of systemic involvement traumatized skin sites,
and upper respi disease

• Sites: nose, earlobes, cheeks, and and around embedded
digits foreign material such as
• Angiolupoid lesions - pink and
silica and tattoo ink
violaceous papules and plaques with
prominent telangiectasias on the face
HYPOPIGMENTED ALOPECIA
• hypopigmented macules occur
• both scarring and nonscarring,
primarily in darkly pigmented persons
• may occur on the face and scalp
.
MUCOUS MEMBRANES
• papules and plaques of mucosal
surface and tongue “strawberry
gums”
 • Erythema nodosum - main; assoc
with Lofgren syndrome
• Neutrophilic dermatoses -
Sweet Syndrome, Pyoderma
NONSPECIFIC gangrenosum
• Nonspecific erythematous
CUTANEOUS eruptions resembling viral
exanthem or drug eruption
LESIONS • Prurigo nodules
• Erythroderma
• Lower extremity edema
 • PULMONARY - most common; (fibrotic
disease may lead to pulmonary HTN
and chronic Aspergillus infection)
• OCULAR - Uveitis (MC); Optic neuritis
• CARDIAC - cardiac arrhythmias and LV
NONCUTANEOUS dysfunction
• NEURO - facial nerve (MC); aseptic
FINDINGS meningitis and peripheral neuropathy
• RENAL - granulomatous inflammation or
nephrolithiasis due to hypercalcemia
(increase 1 alpha hydroxylase activity)
• HEPATIC - rarely with signs or
symptoms; liver can be a good biopsy
site to confirm the diagnosis
 ETIOLOGY
AND
PATHOGENESIS • Triggered by exposure to an antigen(s) with
hyperactivity of the cell-mediated immune system
leading to granulomatous inflammation


Risk factors
• Infectious agents such as mycobacteria,
Propionibacterium acnes, and Chlamydia
• Mineral dusts, including silica, iron, and titanium
• Lifelong nonsmokers are more likely to develop the
disease than smokers
• Genetics play a major role in determining
susceptibility to sarcoidosis
 COURSE
AND
PROGNOSIS
• Granulomatous inflammation of sarcoidosis can remit spontaneously or with therapy
• Remissions often occur within the first 6 months after diagnosis, although it may take 2 to 5
years.

• Lofgren syndrome - >80% rate of spontaneous remission, generally within 4 to 6 weeks

• Lupus pernio - chronic disease and is assoc with upper respiratory tract involvement,
pulmonary fibrosis, and bony cysts
• Worse prognosis - African descent, higher stage chest radiograph (greater than stage I), age
older than 40 years, splenic involvement, disease duration >2 years, and forced vital capacity
less than 1.5 L
Thank You for your
kind attention!