papulosquamous disorders: seb derm, psoriasis, palmoplantar, pust derm, erythroderma chris weyer...
TRANSCRIPT
Papulosquamous Disorders: Papulosquamous Disorders: Seb Derm, Psoriasis, Palmoplantar, Pust Seb Derm, Psoriasis, Palmoplantar, Pust
Derm, ErythrodermaDerm, Erythroderma
Chris Weyer D.O.Chris Weyer D.O.
Dermatology Resident, PGY-1Dermatology Resident, PGY-1
Northeast Regional Medical Northeast Regional Medical CenterCenter
Seborrheic DermatitisSeborrheic Dermatitis
2-5 % of the population2-5 % of the population Chronic, superficial, inflammatory disease Chronic, superficial, inflammatory disease
caused by the yeast, caused by the yeast, Malassezia globosaMalassezia globosa Most commonly affects the scalpMost commonly affects the scalp Also, affects the eyebrows, ears, eyelids, Also, affects the eyebrows, ears, eyelids,
nasolabial creases, lips, sternal area, nasolabial creases, lips, sternal area, axillae, submammary folds, umbilicus, axillae, submammary folds, umbilicus, groin, and gluteal creasegroin, and gluteal crease
Characterized by itchy, pink, yellow or Characterized by itchy, pink, yellow or erythematous patches that may be dry, erythematous patches that may be dry, moist, or greasymoist, or greasy
Infantile Seborrheic Infantile Seborrheic DermatitisDermatitis
Presents around 1 Presents around 1 week after birthweek after birth
AKA “Cradle cap”AKA “Cradle cap” Superimposed Superimposed
Candida Candida may occurmay occur
Adult Seborrheic Adult Seborrheic DermatitisDermatitis
Erythematous, Erythematous, greasy yellow scalegreasy yellow scale
SymmetricalSymmetrical If extensive, think If extensive, think
about about immunosuppressioimmunosuppression: HIV, diabetes, n: HIV, diabetes, and Parkinson’s and Parkinson’s diseasedisease
Histology of Seb DermHistology of Seb Derm Epidermis is acanthoticEpidermis is acanthotic Focal scale crust Focal scale crust
adjacent to follicular adjacent to follicular ostiaostia
Slight spongiosisSlight spongiosis Adult seb derm has Adult seb derm has
superficial perivascular superficial perivascular & perifollicular & perifollicular lymphocytic infiltrate lymphocytic infiltrate composed mainly of composed mainly of lymphocyteslymphocytes
Older lesions show Older lesions show irregular acanthosis & irregular acanthosis & focal parakeratosisfocal parakeratosis
Histo features are Histo features are combination of combination of psoriasis & spongiotic psoriasis & spongiotic dermderm
Treatment-Adolescent & Treatment-Adolescent & AdultAdult
Regular use of medicated shampoos -- over-Regular use of medicated shampoos -- over-the-counter: sulfide, tar, zinc, pyrithionatethe-counter: sulfide, tar, zinc, pyrithionate
Nizoral shampoo & creamNizoral shampoo & cream Loprox shampoo & solutionLoprox shampoo & solution Elidel creamElidel cream Corticosteroids for quick control or flaresCorticosteroids for quick control or flares Key: leave shampoo on scalp for 3 minutes Key: leave shampoo on scalp for 3 minutes
daily; apply daily; apply beforebefore going into shower, as going into shower, as patient will not leave on shampoo for 3 patient will not leave on shampoo for 3 minutes while in showerminutes while in shower
Infantile seborrheic Infantile seborrheic dermatitisdermatitis
Responds well to topical therapyResponds well to topical therapy Hydrocortisone 1% on the face & Hydrocortisone 1% on the face &
skin foldsskin folds This may be combined with an This may be combined with an
antifungal agent for skin foldsantifungal agent for skin folds Remove scale after softening with an Remove scale after softening with an
oil preparation or a weak keratolytic oil preparation or a weak keratolytic agentagent
ReassuranceReassurance
PsoriasisPsoriasis
A common, chronic, recurrent, A common, chronic, recurrent, inflammatory disease of the skin inflammatory disease of the skin characterized by round, characterized by round, circumscribed, erythematous, dry, circumscribed, erythematous, dry, scaling plaques of various sizes, scaling plaques of various sizes, covered by grayish white or covered by grayish white or silvery white, imbricated and silvery white, imbricated and lamellar scaleslamellar scales
Predilection for the scalp, nails, Predilection for the scalp, nails, extensor surfaces, elbows, knees, extensor surfaces, elbows, knees, umbilical, and sacral regionumbilical, and sacral region
Psoriasis Psoriasis nail nail changeschangesonycholysis, nail onycholysis, nail pitting, and oil pitting, and oil spot phenomenonspot phenomenon
Psoriasis clinical Psoriasis clinical featuresfeatures
Koebner’s phenomenonKoebner’s phenomenon – the – the appearance of typical lesions of psoriasis appearance of typical lesions of psoriasis at sites of injuryat sites of injury
Auspitz’s sign – pinpoint bleeding when Auspitz’s sign – pinpoint bleeding when the psoriatic scale is forcibly removed, the psoriatic scale is forcibly removed, this occurs because of severe thinning of this occurs because of severe thinning of the epidermis over the tips of the dermal the epidermis over the tips of the dermal papillapapilla
Woronoff ring – is concentric blanching Woronoff ring – is concentric blanching of the erythematous skin at or near the of the erythematous skin at or near the periphery of the healing psoriatic plaqueperiphery of the healing psoriatic plaque
PathogenesisPathogenesis
Hyperproliferative disorder driven Hyperproliferative disorder driven by inflammatory mediatorsby inflammatory mediators
TH1 cytokines- IL-2,6,8,12; IFN-g, TH1 cytokines- IL-2,6,8,12; IFN-g, TNF-aTNF-a
IL-8: accumulation of neutrophilsIL-8: accumulation of neutrophils IL-12: Primary TH1 signal, promotes IL-12: Primary TH1 signal, promotes
IFN-g productionIFN-g production
InheritenceInheritence
Heredity has a high influence, Heredity has a high influence, incidence increases with incidence increases with generationsgenerations
Evidence that susceptibility to Evidence that susceptibility to psoriasis is linked to class I and II psoriasis is linked to class I and II MHC chromo 6MHC chromo 6
Genetic loci: PSORS1 (6); PSORS2 Genetic loci: PSORS1 (6); PSORS2 (17q)(17q)
HLA asso: early onset -Cw6, -B57, HLA asso: early onset -Cw6, -B57, and -DR7 and -DR7 late onset –Cw2late onset –Cw2
Inheritance contInheritance cont
It is also believed that any individual It is also believed that any individual that has that has
-B13 or –B17 has a fivefold risk of -B13 or –B17 has a fivefold risk of developing psoriasis developing psoriasis
pustular psoriasis: HLA-B27pustular psoriasis: HLA-B27 guttate and erythrodermic psoriasis: -guttate and erythrodermic psoriasis: -
B13,-B17B13,-B17 Palmarplantar pust: -B8, -Bw35, -Cw7, Palmarplantar pust: -B8, -Bw35, -Cw7,
-DR3-DR3
Epidemiology of Epidemiology of psoriasispsoriasis
Seen in 1-2% of US populationSeen in 1-2% of US population Equal frequency in both sexesEqual frequency in both sexes Mean age of onset is 27 yearsMean age of onset is 27 years Sunlight may improve psoriasis by Sunlight may improve psoriasis by
decreasing T-cellsdecreasing T-cells Emotional stress, smoking, and Emotional stress, smoking, and
alcohol aggravates psoriasisalcohol aggravates psoriasis Increased incidence of celiacs, Increased incidence of celiacs,
lymphomalymphoma
Drug-induced psoriasisDrug-induced psoriasis
May be induced by many drugs: beta May be induced by many drugs: beta blockers, lithium, and antimalarials, blockers, lithium, and antimalarials, terbinafine, calcium channel terbinafine, calcium channel blockers, captopril, glyburide, and blockers, captopril, glyburide, and lipid lowering agents, such as lipid lowering agents, such as gemfibrozilgemfibrozil
PathologyPathology Regular epidermal Regular epidermal
hyperplasia with long, test-hyperplasia with long, test-tube-shaped rete ridgestube-shaped rete ridges
Thinning of dermal papillaeThinning of dermal papillae Granular layer is thin or Granular layer is thin or
absentabsent Overlying parakeratosisOverlying parakeratosis Small collections of Small collections of
neutrophils (Munro neutrophils (Munro microabcesses) may be microabcesses) may be present in the stratum present in the stratum corneumcorneum
There is a perivascular There is a perivascular mononuclear cell infiltratemononuclear cell infiltrate
Types of PsoriasisTypes of Psoriasis Seborrheic-Like, “sebopsoriasis”Seborrheic-Like, “sebopsoriasis” InverseInverse NapkinNapkin ArthritisArthritis GuttateGuttate Generialized Pustular (von Zumbusch)Generialized Pustular (von Zumbusch) Acro dermatitis Continua of HallopeauAcro dermatitis Continua of Hallopeau Impetigo HerpetiformisImpetigo Herpetiformis Keratoderma BlennorrhagicaKeratoderma Blennorrhagica ErythrodermicErythrodermic
Inverse PsoriasisInverse Psoriasis
Flexural or Flexural or intertrigenous intertrigenous locationlocation
Shiny erythematous plaques in the axilla that lack scale.
““Napkin” PsoriasisNapkin” Psoriasis
Infants 2-8 moInfants 2-8 mo Erythematous sharply demarcated in Erythematous sharply demarcated in
diaper areadiaper area Lesions typically clear w/ topical txLesions typically clear w/ topical tx Infants may be at risk for psoriasis in Infants may be at risk for psoriasis in
adulthoodadulthood
Five clinical patterns of Five clinical patterns of psoriatic arthritispsoriatic arthritis
Asymmetrical DIP joint involvement with Asymmetrical DIP joint involvement with nail damage, 16%nail damage, 16%
Arthritis mutilans with osteolysis of Arthritis mutilans with osteolysis of phalanges and metacarpals, 5%phalanges and metacarpals, 5%
Symmetrical polyarthritis-like rheumatoid Symmetrical polyarthritis-like rheumatoid arthritis, with claw hands, 15%arthritis, with claw hands, 15%
Oligoarthritis with swelling and Oligoarthritis with swelling and tenosynovitis of one or a few hand joints, tenosynovitis of one or a few hand joints, 70%70%
Ankylosing spondylitis alone or with Ankylosing spondylitis alone or with peripheral arthritis, 5%peripheral arthritis, 5%
Radiographic findings of Radiographic findings of psoriatic arthritispsoriatic arthritis
Erosion of terminal phalangeal tufts; Erosion of terminal phalangeal tufts; tapering of phalanges or metacarpals; tapering of phalanges or metacarpals; “cupping’ of proximal ends of phalanges; “cupping’ of proximal ends of phalanges; bony ankylosis; osteolysis of metatarsals; bony ankylosis; osteolysis of metatarsals; predilection for distal and proximal predilection for distal and proximal interphalangeal joints; paravertebral interphalangeal joints; paravertebral ossification; asymmetrical sacroiliitis; and ossification; asymmetrical sacroiliitis; and “bamboo spine”“bamboo spine”
Nearly half the patients with psoriatic Nearly half the patients with psoriatic arthritis have HLA-B27arthritis have HLA-B27
Tx- biologics, MTX, cyclosporin- dz Tx- biologics, MTX, cyclosporin- dz modifying prevent deformitymodifying prevent deformity
Guttate PsoriasisGuttate Psoriasis Usually, occurs as an abrupt eruption Usually, occurs as an abrupt eruption
following an acute infection, such as following an acute infection, such as streptococcal pharyngitis streptococcal pharyngitis
Occurs mostly in patients under age 30Occurs mostly in patients under age 30 Recurrent episodes are likelyRecurrent episodes are likely This type of psoriasis is usually rapidly This type of psoriasis is usually rapidly
responsive to topical steroids or UVBresponsive to topical steroids or UVB Tx strep infxTx strep infx
Guttate Psoriasis
A: Small papules and plaques of guttate psoriasis; note Koebner phenomenon
B: Numerous papules due to Koebner phenomenon after sunburn
Generalized pustular Generalized pustular psoriasispsoriasis
(von Zumbusch)(von Zumbusch) Typical patients have Typical patients have plaque psoriasis and plaque psoriasis and often psoriatic arthritisoften psoriatic arthritis
The onset is sudden, The onset is sudden, with formation of lakes with formation of lakes of pus periungally, on of pus periungally, on the palms, and at the the palms, and at the edge of psoriatic edge of psoriatic plaquesplaques
Pruritus, pain, fever, Pruritus, pain, fever, and malaiseand malaise
Fetid odor developsFetid odor develops
Generalized pustular Generalized pustular psoriasispsoriasis
(von Zumbusch)(von Zumbusch) Etiology unclearEtiology unclear Iodides, coal tar, steroid withdrawal, Iodides, coal tar, steroid withdrawal,
terbinafine, minocycline, terbinafine, minocycline, hydroxychloroquine, acetazolamide, hydroxychloroquine, acetazolamide, and salicylates may trigger the and salicylates may trigger the attacksattacks
May occur in infantsMay occur in infants Acitretin is drug of choice, with a Acitretin is drug of choice, with a
rapid and predictable responserapid and predictable response Isotretinoin, cyclosporine, Isotretinoin, cyclosporine,
methotrexate, dapsonemethotrexate, dapsone
Acrodermatitis Continua Acrodermatitis Continua of Hallopeauof Hallopeau
Acral erythematious plaques with Acral erythematious plaques with nail involvmentnail involvment
Fingernails float away on lakes of Fingernails float away on lakes of pus: anonychiapus: anonychia
Fingers taper to long keratotic Fingers taper to long keratotic pointspoints
Impetigo HerpetiformisImpetigo Herpetiformis
Generalized pustular psoriasis of Generalized pustular psoriasis of pregnancypregnancy
Retinoids not indicatedRetinoids not indicated Prednisone: controversial, may Prednisone: controversial, may
induce pustular flare, also matures induce pustular flare, also matures fetal lungfetal lung
Early delivery strongly encouragedEarly delivery strongly encouraged
Treatment- TopicalsTreatment- Topicals Corticosteroids- class I- 2wks, pulse, occlusion; Corticosteroids- class I- 2wks, pulse, occlusion;
rapid return when stoppedrapid return when stopped Calcipotriene- keratinocyte differentiation; Calcipotriene- keratinocyte differentiation;
plaque and scalp typeplaque and scalp type Tars- oils and shmp; stinks (literally)Tars- oils and shmp; stinks (literally) Macrolactams (tacrolimus, pimicrolimus)- Macrolactams (tacrolimus, pimicrolimus)-
prevent steroid atrophy/acneprevent steroid atrophy/acne Salicylic acid- keratolyticSalicylic acid- keratolytic Tazorac- modulate differentiation/proliferationTazorac- modulate differentiation/proliferation Anthralin- SCAT, supresses neut superoxide, Anthralin- SCAT, supresses neut superoxide,
inhib mono IL-6,8/TNF-ainhib mono IL-6,8/TNF-a
Treatment- LightTreatment- Light
NB-UVB (311-313nm), MED, 70% NB-UVB (311-313nm), MED, 70% responseresponse
Goeckerman Tech- Goeckerman Tech- 2-5% tar bath QD + UV; clear 18 d w/ long 2-5% tar bath QD + UV; clear 18 d w/ long
term responseterm response Ingram- Ingram-
tar bath QD/carbonisdetergens/ UV/ tar bath QD/carbonisdetergens/ UV/ anthalin/talcum/stocking dressinganthalin/talcum/stocking dressing
PUVA- 2x/wk, clear 20-25 tx; risk PUVA- 2x/wk, clear 20-25 tx; risk cataracts, SCC, MMcataracts, SCC, MM
MethotrexateMethotrexate First effective systemic drug for First effective systemic drug for
psoriasis and is the standard for psoriasis and is the standard for systemic therapysystemic therapy
Blocks synthesis of deoxyribonucleic Blocks synthesis of deoxyribonucleic acid, which decreases cell divisionacid, which decreases cell division
Indications- erythroderma, arthritis, Indications- erythroderma, arthritis, pustular, lg BSApustular, lg BSA
MTXMTX
For patients with no risk factors For patients with no risk factors for liver disease, the first liver for liver disease, the first liver biopsy should be obtained at biopsy should be obtained at approximately 1.5g of cumulative approximately 1.5g of cumulative methotrexate methotrexate
Weekly dosing for oral or IM – Weekly dosing for oral or IM – divided in three doses, 12 hours divided in three doses, 12 hours apartapart
IM injections have a much lower IM injections have a much lower risk of hepatotoxicityrisk of hepatotoxicity
CyclosporineCyclosporine
May down-modulate May down-modulate proinflammatory epidermal proinflammatory epidermal cytokinescytokines
Rapid clearing and returnRapid clearing and return Low risk of renal tox w/ <6mo txLow risk of renal tox w/ <6mo tx Follow BP, CrFollow BP, Cr
DietDiet
Most recent trials have demonstrated Most recent trials have demonstrated the antiinflammatory effects of fish oils the antiinflammatory effects of fish oils rich in n-3 polyunsaturated fatty acids rich in n-3 polyunsaturated fatty acids in rheumatoid arthritis, inflammatory in rheumatoid arthritis, inflammatory bowel disease, psoriasis and asthmabowel disease, psoriasis and asthma
Gluten-free dietGluten-free diet Koo J., Lee E., Lee C.S., Lebwohl M.. Koo J., Lee E., Lee C.S., Lebwohl M..
PsoriasisPsoriasis. J Am Acad Dermatol . J Am Acad Dermatol 2004;50:613-22. 2004;50:613-22.
Oral antimicrobial Oral antimicrobial therapytherapy
Recent evidence suggest that Recent evidence suggest that Staphylococcus aureusStaphylococcus aureus and and streptococci secrete a large family of streptococci secrete a large family of exotoxins that are superantigens, exotoxins that are superantigens, producing massive T-cell activationproducing massive T-cell activation
Oral antibiotic for psoriasis patients Oral antibiotic for psoriasis patients infected with these organisms is infected with these organisms is imperativeimperative
RetinoidsRetinoids Treatment with 13-cis-retinoic acid can Treatment with 13-cis-retinoic acid can
produce good results, especially in produce good results, especially in pustular psoriasispustular psoriasis
It is a potent teratogenIt is a potent teratogen Combinations of retinoic acids with Combinations of retinoic acids with
photochemotherapy for chronic plaque photochemotherapy for chronic plaque psoriasis may also be very affectivepsoriasis may also be very affective
Etretinate- long ½ life, EtOH changes Etretinate- long ½ life, EtOH changes acitretinacitretin
Acitretin – avoid pregnancy for up to 3 Acitretin – avoid pregnancy for up to 3 years following cessation of therapyyears following cessation of therapy
DapsoneDapsone
Use is limited largely to Use is limited largely to palmoplantar eruptionspalmoplantar eruptions
22ndnd-3-3rdrd line therapy line therapy
Combination therapyCombination therapy
Patient on methotrexate may have Patient on methotrexate may have their dose minimized with their dose minimized with concomitant topical steroid useconcomitant topical steroid use
PUVA with acitretin, cyclosprine, PUVA with acitretin, cyclosprine, or methotrexateor methotrexate
Topical Dovonex with Acitretin, Topical Dovonex with Acitretin, cyclosporine, methotrexate, and cyclosporine, methotrexate, and phototherapyphototherapy
Biologic AgentsBiologic Agents
Alefacept (Amevive)Alefacept (Amevive) Efalizumab (Raptiva)Efalizumab (Raptiva) Etanercept (Enbrel)Etanercept (Enbrel) Infliximab (Remicaide)Infliximab (Remicaide) Adalimumab (Humira)Adalimumab (Humira) These are indicated for moderate-to-These are indicated for moderate-to-
severe plaque psoriasissevere plaque psoriasis
Journal of the American Academy of DermatologyVolume 53 • Number 2 • August 2005Copyright © 2005 American Academy of Dermatology, Inc. Biologics in psoriasis: A quick reference guide Valencia D. Thomas, MD F. Clarissa Yang, MD Joseph C. Kvedar, MD
Reiter’s syndromeReiter’s syndrome
Triad: urethritis, conjunctivitis, and Triad: urethritis, conjunctivitis, and arthritisarthritis Can’t see, can’t pee, can’t climb a tree!Can’t see, can’t pee, can’t climb a tree!
May also be other features that involve May also be other features that involve the skin , mucous membranes, GI tract, the skin , mucous membranes, GI tract, and cardiovascular systemand cardiovascular system
American Rheumatism Association - American Rheumatism Association - criterion of peripheral arthritis of more criterion of peripheral arthritis of more than 1 month duration, in association than 1 month duration, in association with urethritis and/or cervicitiswith urethritis and/or cervicitis
Young men of HLA-B27 genotypeYoung men of HLA-B27 genotype
Clinical featuresClinical features May also have fever, May also have fever,
weakness, and weight weakness, and weight lossloss
A nonbacterial urethritis A nonbacterial urethritis may develop with may develop with painful and bloody painful and bloody urination and pyuria urination and pyuria
About 1/3 of patients About 1/3 of patients develop conjunctivitisdevelop conjunctivitis
Keratitis is usually Keratitis is usually superficial and very superficial and very painful. Iritis is painful. Iritis is common.common.
Reiter’s syndromeReiter’s syndrome
An asymmetric An asymmetric arthritis afflicts arthritis afflicts synovial joints, synovial joints, especially those that especially those that are weight bearingare weight bearing
Onset is sudden with Onset is sudden with heat and tenderness heat and tenderness and swellingand swelling
Pain in one or both Pain in one or both heels is a frequent heels is a frequent symptomsymptom
Cutaneous lesions of Cutaneous lesions of Reiter’sReiter’s
The skin lesions start as multiple, The skin lesions start as multiple, small, yellowish vesicles that break, small, yellowish vesicles that break, become confluent, and form become confluent, and form superficial erosionssuperficial erosions
Develop frequently on the genitals Develop frequently on the genitals and palmsand palms
Eruption on the glans penis occurs Eruption on the glans penis occurs in 25% of patientsin 25% of patients
Cutaneous featuresCutaneous features The eruption is known The eruption is known
as keratoderma as keratoderma blennorrhagicumblennorrhagicum
Penile lesions are Penile lesions are frequentfrequent Balanitis circinataBalanitis circinata
Characterized by Characterized by perimeatal balanitisperimeatal balanitis
Similar lesion are Similar lesion are seen on the vaginal seen on the vaginal mucosa of womenmucosa of women
EtiologyEtiology
Syndrome has been attributed to many Syndrome has been attributed to many different agents, such as: different agents, such as: Shigella flexneri, Shigella flexneri, Salmonella spp., yersinia spp., Salmonella spp., yersinia spp., Ureaplasma urealyticum, Borrelia Ureaplasma urealyticum, Borrelia burgdorferi, Cryptosporidia,burgdorferi, Cryptosporidia, and and Campylobacter fetusCampylobacter fetus
May be responsible for the infectious May be responsible for the infectious enteritis that precedes onset in a small enteritis that precedes onset in a small percentage of patientspercentage of patients
In cases that follow an infection of the In cases that follow an infection of the genitourinary tract, genitourinary tract, Chlamydia Chlamydia trachomatis trachomatis may be associatedmay be associated
HLA-B27 positivity is present in 80%HLA-B27 positivity is present in 80%
Laboratory findingsLaboratory findings
Non-specificNon-specific A leukocytosis of 10, 000 to 20, A leukocytosis of 10, 000 to 20,
000/mm3 and an elevated sed rate000/mm3 and an elevated sed rate
HistopathologyHistopathology
Identical to psoriasisIdentical to psoriasis
Differential diagnosisDifferential diagnosis
May be confused with rheumatoid May be confused with rheumatoid arthritis, ankylosing spondylitis, arthritis, ankylosing spondylitis, gout, psoriatic arthritis, gonococcal gout, psoriatic arthritis, gonococcal arthritis, acute rheumatic fever, arthritis, acute rheumatic fever, chronic mucocutaneous candidiasis, chronic mucocutaneous candidiasis, and serum sicknessand serum sickness
TreatmentTreatment
Usually mucocutaneous lesions are Usually mucocutaneous lesions are self- limited and clear within a few self- limited and clear within a few monthsmonths
Topical steroidsTopical steroids NSAIDsNSAIDs MethotrexateMethotrexate Cyclosporine Cyclosporine AcitretinAcitretin
Subcorneal pustular Subcorneal pustular dermatosisdermatosis
(Sneddon-Wilkinson Disease(Sneddon-Wilkinson Disease)) Chronic pustular disease, Chronic pustular disease,
which occurs chiefly in which occurs chiefly in middle-aged womenmiddle-aged women
Pustules are superficial and Pustules are superficial and arranged in annular and arranged in annular and serpiginous patterns on the serpiginous patterns on the abdomen, axillae and groinabdomen, axillae and groin
Vesicles may be presentVesicles may be present Cultures from pustules are Cultures from pustules are
sterilesterile Oral lesions are rareOral lesions are rare Some cases occur in Some cases occur in
association with an IgA association with an IgA monoclonal gammopathymonoclonal gammopathy
Desmocollin 1 autoantigen Desmocollin 1 autoantigen for Iga pemphigusfor Iga pemphigus
Subcorneal pustular Subcorneal pustular dermatosisdermatosis
(Sneddon-Wilkinson Disease)(Sneddon-Wilkinson Disease) Dapsone appears to be Dapsone appears to be
effective in most caseseffective in most cases SulfapyridineSulfapyridine AcitretinAcitretin NB-UVBNB-UVB Corticosteroids, Corticosteroids,
colchicine, and TCN colchicine, and TCN with niacinamidewith niacinamide
Without treatment this Without treatment this is a chronic condition is a chronic condition with remissions of with remissions of variable durationvariable duration
Pathology of Sneddon-Pathology of Sneddon-WilkinsonWilkinson
Eosinophilic pustular Eosinophilic pustular folliculitisfolliculitis
Also referred to as Also referred to as sterile sterile eosinophilic pustulosiseosinophilic pustulosis
AKA Ofuji’s diseaseAKA Ofuji’s disease Males, AsiaMales, Asia HIVHIV Characterized by pruritic, Characterized by pruritic,
follicular papulopustules arranged follicular papulopustules arranged in groupsin groups
Histo: eos in or around folliclesHisto: eos in or around follicles
Eosinophilic pustular Eosinophilic pustular folliculitisfolliculitis
Distribution is usually asymmetrical – Distribution is usually asymmetrical – affects face, trunk, and upper affects face, trunk, and upper extremitiesextremities
Cause is unknown – numerous studies Cause is unknown – numerous studies have implicated chemotactic have implicated chemotactic substances, ICAM-1, and substances, ICAM-1, and cyclooxygenase-generated metabolitescyclooxygenase-generated metabolites
Typical course – spontaneous Typical course – spontaneous remissions and exacerbations for a few remissions and exacerbations for a few months to several yearsmonths to several years
Eosinophilic pustular Eosinophilic pustular folliculitisfolliculitis
Dapsone or systemic steroids are the Dapsone or systemic steroids are the treatment of choicetreatment of choice
Success with intralesional steroids, Success with intralesional steroids, clofazimine, minocycline, clofazimine, minocycline, isotretinoin, UVB therapy, isotretinoin, UVB therapy, indomethacin, colchicine, indomethacin, colchicine, cyclosporine, and cetirizinecyclosporine, and cetirizine
Recalcitrant Recalcitrant Palmoplantar EruptionsPalmoplantar Eruptions
Recalcitrant pustular eruptions of Recalcitrant pustular eruptions of the hands and feet are often the hands and feet are often examples of psoriasisexamples of psoriasis
Need to then search for lesions Need to then search for lesions elsewhere on the body(e.g., scalp, elsewhere on the body(e.g., scalp, ears, glans penis)ears, glans penis)
Search also for a family history to Search also for a family history to confirm your suspicionconfirm your suspicion
Dermatitis RepensDermatitis Repens Aka- acrodermatitis continua and Aka- acrodermatitis continua and
acrodermatits perstansacrodermatits perstans It’s a chronic inflammatory disease of It’s a chronic inflammatory disease of
hands and feethands and feet Rarely, can become generalizedRarely, can become generalized Usually, as a pustule or paronychiaUsually, as a pustule or paronychia
Dermatitis RepensDermatitis Repens Occasionally, mucous membranes are Occasionally, mucous membranes are
involvedinvolved Nails are often dystrophic or destroyedNails are often dystrophic or destroyed Lesions cause skin atrophyLesions cause skin atrophy Crusted, eczematoid, and psoriasiform Crusted, eczematoid, and psoriasiform
lesions may occur, and there may be lesions may occur, and there may be moderate itchingmoderate itching
It is essentially unilateral in its beginning It is essentially unilateral in its beginning and asymmetrical throughout its entire and asymmetrical throughout its entire coursecourse
Dermatitis RepensDermatitis Repens HistologyHistology
similar to those seen in psoriasissimilar to those seen in psoriasis the primary lesion is epidermalthe primary lesion is epidermal An intraepithelial spongiform pustule is An intraepithelial spongiform pustule is
formed by infiltration of pmn’sformed by infiltration of pmn’s TreatmentTreatment
topical mechlorethamine, topical steroids, topical mechlorethamine, topical steroids, PUVA, fluorouracil, and sulfapyridinePUVA, fluorouracil, and sulfapyridine
Acitretin, low dose cyclosporine, Acitretin plus Acitretin, low dose cyclosporine, Acitretin plus calcipotriolcalcipotriol
Palmoplantar PustulosisPalmoplantar Pustulosis
AKA pustular psoriasisAKA pustular psoriasis In contrast to dermatitis repens it is In contrast to dermatitis repens it is
essentially bilateral and symmetricalessentially bilateral and symmetrical Locations include: Locations include:
thenar/hypothenar eminences or thenar/hypothenar eminences or central portion of the palms and central portion of the palms and solessoles
Palmoplantar Palmoplantar PustulosisPustulosis
Patches begin as Patches begin as erythematous erythematous areas in which areas in which pustules formpustules form
Start as pinhead-Start as pinhead-sized, enlarge sized, enlarge and coalesce to and coalesce to form small lakes form small lakes of pusof pus
In the course of a week, In the course of a week, they tend to dry up, they tend to dry up, leaving punctate brown leaving punctate brown scabs that eventually scabs that eventually exfoliateexfoliate
Stages of quiescence and Stages of quiescence and exacerbation characterize exacerbation characterize the conditionthe condition
Meds, such as lithium, Meds, such as lithium, have been reported to have been reported to induceinduce
Palmoplantar PustulosisPalmoplantar Pustulosis Nails may become malformed, ridged, stippled, Nails may become malformed, ridged, stippled,
pitted and discoloredpitted and discolored May be associated with psoriasis vulgarisMay be associated with psoriasis vulgaris Some regard palmoplantar pustulosis as a form Some regard palmoplantar pustulosis as a form
of psoriasis, while others consider it a separate of psoriasis, while others consider it a separate entityentity
Female predominance; lack of seasonal variation; Female predominance; lack of seasonal variation; different histopathologic features anddifferent histopathologic features and
Associated with thyroid disorders and cigarette Associated with thyroid disorders and cigarette smokingsmoking
Palmoplantar PustulosisPalmoplantar Pustulosis May be predisposed May be predisposed
to joint disease and to joint disease and possibly SAPHO possibly SAPHO syndrome-syndrome-SSynovitis, ynovitis, AAcne, cne, PPustulosis, ustulosis, HHyperostosis and yperostosis and OOsteoarthritissteoarthritis
It’s resistant to most It’s resistant to most treatmentstreatments
Acitretin is reportedly Acitretin is reportedly effective(1mg/kg/day)effective(1mg/kg/day)
Low-dose cyclosporine Low-dose cyclosporine (1.25mg/kg/day-(1.25mg/kg/day-3.75mg/kg/day)3.75mg/kg/day)
Intramuscular Intramuscular Kenalog (40-Kenalog (40-60mg)may be 60mg)may be effective for short-effective for short-term reliefterm relief
Palmoplantar PustulosisPalmoplantar Pustulosis
Pustular Pustular BacteridBacterid
Characterized by a symmetric, grouped, vesicular Characterized by a symmetric, grouped, vesicular or pustular eruption on palms and solesor pustular eruption on palms and soles
Marked by exacerbations and remissions over long Marked by exacerbations and remissions over long periodsperiods
No involvement of webs of fingers or toes or No involvement of webs of fingers or toes or flexion creases of toes flexion creases of toes
WBC may be elevatedWBC may be elevated Scaling is usually presentScaling is usually present Etiology is thought to be a remote focus of Etiology is thought to be a remote focus of
infection; infection needs to be treated before infection; infection needs to be treated before resolution will occurresolution will occur
Juvenile Plantar Juvenile Plantar DermatosisDermatosis
• Usually begins as a patchy, symmetrical, smooth, Usually begins as a patchy, symmetrical, smooth, red, glazed macule on great toes, sometimes with red, glazed macule on great toes, sometimes with fissuring and desquamation in children aged 3-13fissuring and desquamation in children aged 3-13
• Toe webs are rarely involved; fingers may beToe webs are rarely involved; fingers may be• Histologically, there is psoriasiform acanthosis Histologically, there is psoriasiform acanthosis
and a sparse, lymphocytic infiltrate in the upper and a sparse, lymphocytic infiltrate in the upper dermisdermis
• Spongiosis is commonly presentSpongiosis is commonly present• Tx: bed rest, cotton socks and topical steroidsTx: bed rest, cotton socks and topical steroids• Spontaneous resolution within 4 yrs is the ruleSpontaneous resolution within 4 yrs is the rule
Infantile AcropustulosisInfantile Acropustulosis
Intensely itchy vesicopustular Intensely itchy vesicopustular eruption of hands and feeteruption of hands and feet
Begins at any age up to 10 months, Begins at any age up to 10 months, clearing in a few weeks and recurring clearing in a few weeks and recurring repeatedly until final resolution at 6 – repeatedly until final resolution at 6 – 36 months of age36 months of age
Dapsone at 2mg/kg/day may helpDapsone at 2mg/kg/day may help Potent topical steroids aid in Potent topical steroids aid in
symptomatic reliefsymptomatic relief
Should prompt an extensive workup Should prompt an extensive workup to eliminate serious infectious to eliminate serious infectious causes (i.e., Tzanck prep, gram causes (i.e., Tzanck prep, gram stain, KOH prep of pustule)stain, KOH prep of pustule)
Some suspect that this condition Some suspect that this condition may be a persistent reaction to prior may be a persistent reaction to prior scabiesscabies
Infantile AcropustulosisInfantile Acropustulosis
Acropustulosis of infancyAcropustulosis of infancy
THE ENDTHE END