Andrews’ Diseases of the Andrews’ Diseases of the Skin-Chapter 10-pg 239-Skin-Chapter 10-pg 239-253 & Chapter 11253 & Chapter 11

Boris Ioffe, D.O.Boris Ioffe, D.O.

Recalcitrant Recalcitrant Palmoplantar Palmoplantar EruptionsEruptions 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 It is essentially unilateral in its beginning and asymmetrical beginning and asymmetrical throughout its entire coursethroughout its entire course

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 Acitretin, low dose cyclosporine, Acitretin plus calcipotriolplus calcipotriol

Palmoplantar Palmoplantar PustulosisPustulosis AKA pustular psoriasisAKA pustular psoriasis In contrast to dermatitis repens it In contrast to dermatitis repens it

is essentially bilateral and is essentially bilateral and symmetricalsymmetrical

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 Palmoplantar PustulosisPustulosis Nails may become malformed, ridged, Nails may become malformed, ridged,

stippled, pitted and discoloredstippled, pitted and discolored May be associated with psoriasis vulgarisMay be associated with psoriasis vulgaris Some regard palmoplantar pustulosis as a Some regard palmoplantar pustulosis as a

form of psoriasis, while others consider it a form of psoriasis, while others consider it a separate entityseparate entity

Female predominance; lack of seasonal Female predominance; lack of seasonal variation; different histopathologic features variation; different histopathologic features andand

Associated with thyroid disorders and Associated with thyroid disorders and cigarette smokingcigarette smoking

Palmoplantar Palmoplantar PustulosisPustulosis 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/dayeffective(1mg/kg/day))

Low-dose Low-dose cyclosporine 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 Palmoplantar PustulosisPustulosis

Pustular BacteridPustular Bacterid 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 flexion No involvement of webs of fingers or toes or flexion

creases of toes 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, Usually begins as a patchy, symmetrical,

smooth, red, glazed macule on great toes, smooth, red, glazed macule on great toes, sometimes with fissuring and desquamation sometimes with fissuring and desquamation in children aged 3-13in 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 and a sparse, lymphocytic infiltrate in the upper dermisupper dermis

• 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 Infantile AcropustulosisAcropustulosis Intensely itchy vesicopustular eruption Intensely itchy vesicopustular eruption

of hands and feetof 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 Should prompt an extensive workup to eliminate serious workup to eliminate serious infectious causes (i.e., Tzanck infectious causes (i.e., Tzanck prep, gram stain, KOH prep of prep, gram stain, KOH prep of pustule)pustule)

Some suspect that this condition Some suspect that this condition may be a persistent reaction to may be a persistent reaction to prior scabiesprior scabies

Infantile Infantile AcropustulosisAcropustulosis

Acropustulosis of infancyAcropustulosis of infancy

PompholyxPompholyx AKA dyshidrosisAKA dyshidrosis A vesicular eruption of palms and soles A vesicular eruption of palms and soles

characterized by spongiotic intraepidermal vesicles characterized by spongiotic intraepidermal vesicles and often accompanied by burning or itchingand often accompanied by burning or itching

Hyperhidrosis may be presentHyperhidrosis may be present Usually bilateral and symmetricalUsually bilateral and symmetrical Bullae may form Bullae may form Contents are clear and colorlessContents are clear and colorless Attacks generally last a few weeksAttacks generally last a few weeks Lesions dry-up and desquamate rather than ruptureLesions dry-up and desquamate rather than rupture

PomphyloxPomphylox

Etiology- stress, atopy, and topical as well as Etiology- stress, atopy, and topical as well as ingested contactantsingested contactants

Histopathology: spongiotic vesicles in the Histopathology: spongiotic vesicles in the epidermisepidermis

Differential dx: Differential dx: – dermatophytid, contact dermatitis, atopic dermatophytid, contact dermatitis, atopic

dermatitis, drug eruption, pustular psoriasis of dermatitis, drug eruption, pustular psoriasis of palms and soles, acrodermatitis continua, and palms and soles, acrodermatitis continua, and pustular bacteridpustular bacterid

Rarely, T-cell lymphoma can present with Rarely, T-cell lymphoma can present with similar clinical findings, but biopsy of the similar clinical findings, but biopsy of the vesicles will be diagnosticvesicles will be diagnostic

PomphyloxPomphylox

Tx: high potency corticosteroid creamsTx: high potency corticosteroid creams Triamcinolone acetonide Triamcinolone acetonide

intramuscularly or a short course of intramuscularly or a short course of oral prednisone is rapidly effectiveoral prednisone is rapidly effective

Oral or topical psoralen + UVA (PUVA) Oral or topical psoralen + UVA (PUVA) is effective but costly & inconvenientis effective but costly & inconvenient

In more severe forms, In more severe forms, immunosuppressive mycophenolate immunosuppressive mycophenolate mofetil has been effectivemofetil has been effective

Lamellar Lamellar DyshidrosisDyshidrosis

AKA dyshidrosis AKA dyshidrosis lamellosa, keratolysis lamellosa, keratolysis exfoliativaexfoliativa

A superficial exfoliative A superficial exfoliative dermatosis of the dermatosis of the palms and sometimes palms and sometimes solessoles

Referred to as Referred to as recurrent palmar recurrent palmar peelingpeeling

Involvement is bilateralInvolvement is bilateral Can occur in Can occur in

association with association with dyshidrosisdyshidrosis

Often exacerbated by Often exacerbated by environmental factorsenvironmental factors

Differential dx: Differential dx: dermatophytosis, dermatophytosis, chronic contact chronic contact dermatitisdermatitis

Lamellar DyshidrosisLamellar Dyshidrosis

Tx: difficult Tx: difficult Spontaneous involution can occur in a few Spontaneous involution can occur in a few

weeks for someweeks for some Most tends to be chronic and relapsingMost tends to be chronic and relapsing Tar creams (Zetone cream) usually helpsTar creams (Zetone cream) usually helps 5% tar in gel (Estar Gel) is an excellent tx5% tar in gel (Estar Gel) is an excellent tx Lac-Hydrin lotion and Carmol 10 or 20 are Lac-Hydrin lotion and Carmol 10 or 20 are

often effectiveoften effective NB-UVB may be helpfulNB-UVB may be helpful

Lamellar Dyshidrosis

Palmoplantar Palmoplantar KeratodermaKeratoderma

AKA tylosis, keratosis, hyperkeratosisAKA tylosis, keratosis, hyperkeratosis Characterized by excessive formation of Characterized by excessive formation of

keratin on the palms and soleskeratin on the palms and soles AcquiredAcquired

– Keratosis Punctata of the Palmar CreasesKeratosis Punctata of the Palmar Creases– Punctate Keratoses of the Palms and SolesPunctate Keratoses of the Palms and Soles– Porokeratosis Plantaris DiscretaPorokeratosis Plantaris Discreta– Keratoderma ClimactericumKeratoderma Climactericum

CongenitalCongenital

Punctate Keratosis of Punctate Keratosis of the Palms and Solesthe Palms and Soles Primary lesion is a 1-5mm round to Primary lesion is a 1-5mm round to

oval, dome-shaped papule distributed oval, dome-shaped papule distributed over left hand and hypothenar over left hand and hypothenar eminenceeminence

Main symptom is pruritisMain symptom is pruritis Lesions number from 1 to >40Lesions number from 1 to >40 Affects mainly blacksAffects mainly blacks There’s a potential risk of developing There’s a potential risk of developing

lung and colon cancerlung and colon cancer

Punctate Keratosis of Punctate Keratosis of the Palms and Solesthe Palms and Soles

Keratosis Punctata of Keratosis Punctata of the Palmar Creasesthe Palmar Creases Common most often in black ptsCommon most often in black pts Primary lesion is a 1-5mm depression Primary lesion is a 1-5mm depression

filled with a conical keratinous plugfilled with a conical keratinous plug Primarily, in creases of palms or Primarily, in creases of palms or

fingers, occasionally in solesfingers, occasionally in soles Lesions are multipleLesions are multiple Friction aggravates lesions causing Friction aggravates lesions causing

them to become verrucoid or them to become verrucoid or surrounded by callussurrounded by callus

Punctate Punctate keratoses of keratoses of the palmar the palmar creases in an creases in an African-African-AmericanAmerican

PPPK-PPPK-punctate punctate palmoplantar palmoplantar keratodermakeratoderma

Porokeratosis Porokeratosis Plantaris DiscretaPlantaris Discreta Occurs in adults, Female:Male (4:1)Occurs in adults, Female:Male (4:1) Characterized by sharply marginated, Characterized by sharply marginated,

rubbery, wide-based papule that does not rubbery, wide-based papule that does not bleed on removalbleed on removal

Lesions are multiple, painful, 7-10mm in Lesions are multiple, painful, 7-10mm in diameterdiameter

Usually on wt bearing areas of sole, Usually on wt bearing areas of sole, beneath metatarsal headsbeneath metatarsal heads

Tx: foot pads to redistribute wt, surgical Tx: foot pads to redistribute wt, surgical excision, blunt dissectionexcision, blunt dissection

Keratoderma Keratoderma ClimactericumClimactericum Characterized by hyperkeratosis of Characterized by hyperkeratosis of

palms and soles beginning at about palms and soles beginning at about the time of menopausethe time of menopause

Descrete, thickened, hyperkeratotic Descrete, thickened, hyperkeratotic patches most pronounced at pressure patches most pronounced at pressure sitessites

Fissuring may be presentFissuring may be present Tx: keratolytics -- 10% salicylic acid, Tx: keratolytics -- 10% salicylic acid,

lactic acid creams, etc.lactic acid creams, etc.

Hereditary syndromesHereditary syndromes

These have palmoplantar These have palmoplantar keratoderma as a featurekeratoderma as a feature– Unna-ThostUnna-Thost– Papillon-LefervePapillon-Leferve

Dominant inheritance; congenital thickening of epidermal Dominant inheritance; congenital thickening of epidermal horny layer of the palms and soleshorny layer of the palms and soles

Usually symmetricalUsually symmetrical Epidermis becomes thick, yellowish, verrucous, and hornyEpidermis becomes thick, yellowish, verrucous, and horny Striate and punctate forms occurStriate and punctate forms occur

Unna ThostUnna Thost

Unna ThostUnna Thost

Occasionally nails become Occasionally nails become thickenedthickened

5% salicylic acid may help5% salicylic acid may help Lac Hydrin 12% may be triedLac Hydrin 12% may be tried Acitretrin or isotretinoin may be Acitretrin or isotretinoin may be

considered, but need for lifetime considered, but need for lifetime tx makes them impracticaltx makes them impractical

Focal Focal palmoplantar palmoplantar keratosis of keratosis of the striate the striate type on the type on the solesole

Diffuse non-Diffuse non-epidermolytiepidermolytic c palmoplantapalmoplantar keratosisr keratosis

Diffuse epidermolytic palmoplantar Diffuse epidermolytic palmoplantar keratosis with diffuse hyperkeratosiskeratosis with diffuse hyperkeratosis

Papillon-Lefevre Papillon-Lefevre SyndromeSyndrome Palmoplantar hyperkeratosis with Palmoplantar hyperkeratosis with

peridontosisperidontosis Usually develops within the first few Usually develops within the first few

months of life but may occur in months of life but may occur in childhoodchildhood

Well demarcated, erythematous, Well demarcated, erythematous, hyperkeratotic lesions on palms and hyperkeratotic lesions on palms and solessoles

Transverse grooves of fingernails may Transverse grooves of fingernails may occuroccur

Early onset peridontal Early onset peridontal disease has been disease has been attributed to damage and attributed to damage and alteration in PMN function alteration in PMN function caused by Actinomyces caused by Actinomyces actinomycetemcomitansactinomycetemcomitans

Disease associations Disease associations include: acroosteolysis, include: acroosteolysis, and pyogenic liver and pyogenic liver abcessesabcesses

There are There are asymptomatic ectopic asymptomatic ectopic calcifications in the calcifications in the choroid plexus and choroid plexus and tentoriumtentorium

Therapy may retard Therapy may retard both dental and skin both dental and skin abnormalitiesabnormalities

Treatment with Treatment with Acitretin in four Acitretin in four siblings was reported siblings was reported to be effectiveto be effective

Papillon-Lefevre Papillon-Lefevre SyndromeSyndrome

Papillon-Lefevre syndrome: plantar Papillon-Lefevre syndrome: plantar keratodermakeratoderma

Mutilating Mutilating Keratoderma of Keratoderma of VohwinkelVohwinkel Palmoplantar hyperkeratosis of the Palmoplantar hyperkeratosis of the

honeycomb type-associated with starfish-honeycomb type-associated with starfish-like keratosis on backs of hands and feet; like keratosis on backs of hands and feet; linear keratoses of the elbows and knees, linear keratoses of the elbows and knees, and annular constriction (pseudo-ainhum) and annular constriction (pseudo-ainhum) of the digits, this may progress to of the digits, this may progress to autoamputationautoamputation

More than 30 cases have been reported More than 30 cases have been reported world-wideworld-wide

More common in women and in whitesMore common in women and in whites Onset is in infancy or early childhoodOnset is in infancy or early childhood

Vohwinkel’s mutilating syndrome: A.) Vohwinkel’s mutilating syndrome: A.) diffuse keratoderma of palms with B.) diffuse keratoderma of palms with B.) pseudoaainhum formationpseudoaainhum formation

Palmoplantar Palmoplantar Keratodermas & Keratodermas & MalignancyMalignancy Diffuse, waxy keratoderma of palms and Diffuse, waxy keratoderma of palms and

soles occurring as an AD trait soles occurring as an AD trait associated with esophageal carcinomaassociated with esophageal carcinoma

Other related factors are oral Other related factors are oral leukoplakia, esophageal srictures, leukoplakia, esophageal srictures, squamous carcinoma of tylotic skin, squamous carcinoma of tylotic skin, carcinoma of larynx and stomachcarcinoma of larynx and stomach

Acquired forms of palmoplantar Acquired forms of palmoplantar keratodermas have also been keratodermas have also been associated with carcinoma of associated with carcinoma of esophagus, lung, breast, bladder and esophagus, lung, breast, bladder and stomachstomach

Focal PPK in association Focal PPK in association with carcinoma of the with carcinoma of the esophagusesophagus

Acrokeratoelastoidosis Acrokeratoelastoidosis of Costaof Costa AD, more common in womenAD, more common in women Small, round, firm papules occurring over Small, round, firm papules occurring over

dorsal hands, knuckles, and lateral margins dorsal hands, knuckles, and lateral margins of palms and solesof palms and soles

Appears in early childhood and progress Appears in early childhood and progress slowlyslowly

Most often asymptomaticMost often asymptomatic Significant histologic finding is dermal Significant histologic finding is dermal

elastorrhexiselastorrhexis Therapies: liquid nitrogen, salicylic acid, Therapies: liquid nitrogen, salicylic acid,

tretinoin, and prednisone have been triedtretinoin, and prednisone have been tried

Focal Focal acrokeratoelastoidacrokeratoelastoides: multiple skin-es: multiple skin-colored papules at colored papules at the margin of the the margin of the palmar skinpalmar skin

Path: non-Path: non-epidermolytic epidermolytic palmoplantar palmoplantar keratosis, keratosis, acanthosis acanthosis and and hypergranulohypergranulosissis

Exfoliative DermatitisExfoliative Dermatitis

Universal or very extensive scaling and Universal or very extensive scaling and itching erythrodermaitching erythroderma

Often associated with hair lossOften associated with hair loss Initially with erythematous plaques, Initially with erythematous plaques,

which spread rapidlywhich spread rapidly Onset accompanied by general toxicityOnset accompanied by general toxicity Skin becomes scarlet and swollen and Skin becomes scarlet and swollen and

may ooze a straw-colored exudatemay ooze a straw-colored exudate Desquamation is evident within a few Desquamation is evident within a few

daysdays

EtiologyEtiology Most common is preexisting Most common is preexisting

dermatoses: (53%); dermatoses: (53%); – atopic dermatitis, chronic atopic dermatitis, chronic

actinic dermatitis, actinic dermatitis, psoriasis,seborrheic psoriasis,seborrheic dermatitis, vesicular dermatitis, vesicular palmoplantar eczema, palmoplantar eczema, pityriasis rubra pilaris, and pityriasis rubra pilaris, and contact dermatitiscontact dermatitis

Drug eruptions(5%);Drug eruptions(5%);– allopurinol, gold, allopurinol, gold,

carbamazepine, phenytoin, carbamazepine, phenytoin, and quinidineand quinidine

Cutaneous T-cell Cutaneous T-cell lymphoma(13%); Sezary lymphoma(13%); Sezary syndrome and mycosis syndrome and mycosis fungoidesfungoides

Paraneoplstic (2%); Paraneoplstic (2%); carcinoma of the carcinoma of the lung and carcinoma lung and carcinoma of the stomachof the stomach

Leukemia cutis Leukemia cutis (1%)(1%)

Idiopathic (26%)Idiopathic (26%) Mortality rate at a Mortality rate at a

mean follow-up mean follow-up interval of 51 interval of 51 months was 43%months was 43%

HistologyHistology

Most commonly, histology is Most commonly, histology is nonspecificnonspecific

Hyperkeratosis & focal Hyperkeratosis & focal parakeratosis parakeratosis

Epidermis shows mild acanthosis, Epidermis shows mild acanthosis, scant superficial upper dermal scant superficial upper dermal infiltrate of mononuclear cellsinfiltrate of mononuclear cells

May be small areas of spongiosisMay be small areas of spongiosis

GeneraGeneralization lization after after withdrwithdrawal of awal of methotmethotrexaterexate

ExfoliatioExfoliation of scale n of scale with with underlyinunderlying g erythemaerythema

GeneralizeGeneralized d erythema erythema with thick with thick scale and scale and crusted crusted fissures on fissures on the plantar the plantar surfacesurface

TreatmentTreatment

Topical steroids, soaks, and compresses Topical steroids, soaks, and compresses Acitretin and cyclosporin-useful in Acitretin and cyclosporin-useful in

psoriatic erythroderma, and isotretinoin psoriatic erythroderma, and isotretinoin in erythroderma caused by RPR; in erythroderma caused by RPR; methotrexatemethotrexate

Systemic corticosteroids in severe casesSystemic corticosteroids in severe cases Discontinuing the offending drug in Discontinuing the offending drug in

drug-induced casesdrug-induced cases

* Subungual * Subungual hyperkeratohyperkeratosis and sis and distal distal dystrophydystrophy

Parapsoriasis, Parapsoriasis, PityriasisPityriasisRosea, Pityriasis Rosea, Pityriasis Rubra PilarisRubra Pilaris

ParapsoriasisParapsoriasis

Group of macular scaly eruptions Group of macular scaly eruptions with slow evolutionwith slow evolution

These are all markedly chronic, These are all markedly chronic, resistant to treatment, and are resistant to treatment, and are without subjective symptomswithout subjective symptoms

They are divided into: pityriasis They are divided into: pityriasis lichenoides chronica, pityriasis lichenoides chronica, pityriasis lichenoides et varioliformis acuta, lichenoides et varioliformis acuta, and parapsoriasis en plaquesand parapsoriasis en plaques

Pityriasis Lichenoides Pityriasis Lichenoides ChronicaChronica

Erythematous, Erythematous, yellowish, scaly yellowish, scaly macules and macules and lichenoid papules lichenoid papules

They persist They persist indefinitely without indefinitely without changechange

Mainly on sides of Mainly on sides of trunk, thighs, and trunk, thighs, and upper armsupper arms

May be confused May be confused with psoriasis and with psoriasis and secondary syphilissecondary syphilis

Tx- UV light is beneficial; Tx- UV light is beneficial; however intense doses however intense doses may be needed for good may be needed for good resultsresults

PUVA has been reported PUVA has been reported to be effectiveto be effective

Oral tetracycline may be Oral tetracycline may be used with antihistaminesused with antihistamines

PLC is a benign disease PLC is a benign disease that clears spontaneously that clears spontaneously in a few yrs to monthsin a few yrs to months

Pityriasis Lichenoides Pityriasis Lichenoides ChronicaChronica

PLEVAPLEVA

AKA: parasoriasis AKA: parasoriasis lichenoides, Habermann’s lichenoides, Habermann’s disease, Mucha-disease, Mucha-Habermann disease and Habermann disease and parapsoriasis parapsoriasis varioliformis acutavarioliformis acuta

Sudden appearance of a Sudden appearance of a polymorphous eruption polymorphous eruption composed of macules, composed of macules, papules, and occasional papules, and occasional vesiclesvesicles

May run an acute, May run an acute, subacute, or chronic subacute, or chronic coursecourse

Papules are usually Papules are usually yellowish or yellowish or brownish-red, round brownish-red, round lesions, which tend to lesions, which tend to crust, become crust, become necrotic and necrotic and hemorrhagehemorrhage

PLEVAPLEVA

When exanthem heals it leaves a When exanthem heals it leaves a smooth, pigmented, depressed, smooth, pigmented, depressed, varioliform scarvarioliform scar

Favorite sites are anterior trunk, Favorite sites are anterior trunk, flexural arms, and axillaeflexural arms, and axillae

Palms and soles are involved Palms and soles are involved infrequently-mucous membranes infrequently-mucous membranes are notare not

Generalized lymphadenopathy Generalized lymphadenopathy can occurcan occur

* Usually a benign, self-limited * Usually a benign, self-limited disorder, but may be more disorder, but may be more chronic and severechronic and severe

Maybe a spectrum of cutaneous Maybe a spectrum of cutaneous T-cell lymophomaT-cell lymophoma

Differential dx: Differential dx: – leukocytoclastic angiitis, leukocytoclastic angiitis,

papulonecrotic tuberculid, psoriasis, papulonecrotic tuberculid, psoriasis, lichen planus, varicella, PR, drug lichen planus, varicella, PR, drug eruptions, maculopapular syphilid, eruptions, maculopapular syphilid, viral, rickettsial diseases, viral, rickettsial diseases, lymphomatoid papulosislymphomatoid papulosis

PLEVAPLEVA

PLEVAPLEVA

Histologically of PLEVA is characterized by Histologically of PLEVA is characterized by epidermal necrosis, with prominent epidermal necrosis, with prominent hemorrhage and primarily a dense hemorrhage and primarily a dense perivascular infiltrate of lymphocytes in perivascular infiltrate of lymphocytes in the superficial dermisthe superficial dermis

Absence of neutrophils simplifies the Absence of neutrophils simplifies the distinction between leukocytoclastic distinction between leukocytoclastic angiitis angiitis

Lymphomatoid papulosis differs by the Lymphomatoid papulosis differs by the presence of large, atypical mononuclear presence of large, atypical mononuclear cells in the dermal infiltratecells in the dermal infiltrate

PLEVA-TxPLEVA-Tx

No one tx is reliably effectiveNo one tx is reliably effective Tetracycline and erythromycin are worth Tetracycline and erythromycin are worth

tryingtrying UVB and PUVAUVB and PUVA Methotrexate, 2.5-7.5mg every 12 hrs for 3 Methotrexate, 2.5-7.5mg every 12 hrs for 3

doses 1 day each weekdoses 1 day each week Several serious reactions a few of them Several serious reactions a few of them

fatal have occurred with simultaneous fatal have occurred with simultaneous administration of methotrexate and NSAIDsadministration of methotrexate and NSAIDs

Dapsone and pentoxifylline(Trental), 400mg Dapsone and pentoxifylline(Trental), 400mg twice daily twice daily

Parapsoriasis en Parapsoriasis en PlaquesPlaques* Small-plaque parapsoriasis is * Small-plaque parapsoriasis is

characterized by non-indurated, characterized by non-indurated, brownish, hypopigmented, or yellowish brownish, hypopigmented, or yellowish red scaling patches, round to oval, red scaling patches, round to oval, with sharply defined borderswith sharply defined borders

* Most lesions occur on the trunk and * Most lesions occur on the trunk and are between are between

1 –5cm1 –5cm* Patches may persist for years to * Patches may persist for years to

decades and do not progress to decades and do not progress to lymphomalymphoma

Large Plaque Large Plaque ParapsoriasisParapsoriasis Has patches 5-15 cm; otherwise Has patches 5-15 cm; otherwise

is similar to small-plaque typeis similar to small-plaque type Prognosis is benign, especially if Prognosis is benign, especially if

pruritis is severepruritis is severe 10% may eventuate in T-cell 10% may eventuate in T-cell

lymphomalymphoma

Large plaques Large plaques parasporiasis: parasporiasis: large, variably large, variably erythematous erythematous and mildly and mildly poikilodermatopoikilodermatous patches in us patches in the bathing the bathing trunk regiontrunk region

Small plaque Small plaque parasporiasis: parasporiasis: small(<5), small(<5), erythematous, erythematous, slightly scaly slightly scaly patchespatches

TreatmenTreatmentt

First line: UV radiation -- either natural or UVB First line: UV radiation -- either natural or UVB Lubricants and Topical steroidsLubricants and Topical steroids PUVA but only if UVB failsPUVA but only if UVB fails Use of PUVA or high-potency topical streroids should be Use of PUVA or high-potency topical streroids should be

limited due to long-term adverse effectslimited due to long-term adverse effects LPPP has the potential to develop lymphoma – thus, LPPP has the potential to develop lymphoma – thus,

justifying more intense txjustifying more intense tx Vitamin D2 daily–250,000 units over 2-4 months has been Vitamin D2 daily–250,000 units over 2-4 months has been

effectiveeffective

Pityriasis Pityriasis AlbaAlba

AKA-pityriasis streptogenes, furfuraceous impetigo, AKA-pityriasis streptogenes, furfuraceous impetigo, pityriasis simplex, pityriasis sicca faciei, and erthema pityriasis simplex, pityriasis sicca faciei, and erthema streptogenesstreptogenes

Characterized by hypopigmented, round to oval, Characterized by hypopigmented, round to oval, scaling patches on face, upper arms, neck, or shouldersscaling patches on face, upper arms, neck, or shoulders

Color is white (but never actually depigmented) or light Color is white (but never actually depigmented) or light pinkpink

Scales are fine and adherentScales are fine and adherent Patches are usually sharply demarcated; edges may be Patches are usually sharply demarcated; edges may be

erythematous and slightly elevatederythematous and slightly elevated

Pityriasis AlbaPityriasis Alba Lack of any early specifically follicular localization helps Lack of any early specifically follicular localization helps

to distinguish this lesion from follicular mucinosisto distinguish this lesion from follicular mucinosis Vellus hairs are not lost in pityriasis alba, nor does Vellus hairs are not lost in pityriasis alba, nor does

hypesthesia to cold occur, as often happens in follicular hypesthesia to cold occur, as often happens in follicular mucinosismucinosis

Usually asymptomatic; however there may be mild Usually asymptomatic; however there may be mild pruritispruritis

Disease mainly occurs in children and teenagersDisease mainly occurs in children and teenagers It is particularly a cosmetic problem in dark-skinned It is particularly a cosmetic problem in dark-skinned

individualsindividuals

Pityriasis Pityriasis AlbaAlba

Etiology unknownEtiology unknown Excessively dry skin appears to be Excessively dry skin appears to be

contributorycontributory Most lesions disappear with timeMost lesions disappear with time Repigmentation can be accelerated Repigmentation can be accelerated

with treatmentwith treatment Emollients and bland lubricants Emollients and bland lubricants Low-strength corticosteroids plus Lac-Low-strength corticosteroids plus Lac-

Hydrin are helpfulHydrin are helpful Others have recommended PUVAOthers have recommended PUVA

Pityriasis RoseaPityriasis Rosea Mild inflammatory exanthem of Mild inflammatory exanthem of

unknown origin ?viralunknown origin ?viral Characterized by salmon-colored Characterized by salmon-colored

papules and patches which are oval and papules and patches which are oval and covered with a collarette of scalecovered with a collarette of scale

Disease frequently begins with a single Disease frequently begins with a single herald patch, which may persist a week herald patch, which may persist a week or more, then involutesor more, then involutes

PRPR

Appears rapidly and last from 3-8 Appears rapidly and last from 3-8 weeksweeks

Peak: ages 15-40Peak: ages 15-40 Typically in Spring and AutumnTypically in Spring and Autumn More common in womenMore common in women

Pityriasis RoseaPityriasis Rosea

Mainly affects the Mainly affects the trunktrunk

Oral lesions are Oral lesions are relatively relatively uncommon, but uncommon, but present as aphthous present as aphthous lesionslesions

Herald PatchHerald Patch

Pityriasis RoseaPityriasis Rosea

Papular PR is an unusual form common Papular PR is an unusual form common in black chidren under age 5in black chidren under age 5

Inverse PR is unusual, but not rareInverse PR is unusual, but not rare Relapses and recurrences are Relapses and recurrences are

frequently observedfrequently observed A PR-like eruption can occur as a rxn A PR-like eruption can occur as a rxn

to captopril, arsenicals, gold, bismuth, to captopril, arsenicals, gold, bismuth, clonidine, methoxypromazine, clonidine, methoxypromazine, tripelennaminehydrochloride, or tripelennaminehydrochloride, or barbituatesbarbituates

Inverse pityriasis rosea: oval Inverse pityriasis rosea: oval annular plaques in groinannular plaques in groin

TreatmentTreatment

SupportiveSupportive UVB should be used after acute UVB should be used after acute

inflammatory stage has passedinflammatory stage has passed Topical corticosteroidsTopical corticosteroids AntihistaminesAntihistamines EmollientsEmollients

PR: There is focal parakeratosis, mild acanthosis, PR: There is focal parakeratosis, mild acanthosis, spongiosis, perivascular lymphocytes, and focal spongiosis, perivascular lymphocytes, and focal erythrocyte extravasationerythrocyte extravasation

PR: papules and annular plaquesPR: papules and annular plaques

PR: oval and round plaques, some with PR: oval and round plaques, some with central scale and others with a collarette of central scale and others with a collarette of scalescale

PR in darkly PR in darkly pigmented skin: pigmented skin: it tends to be it tends to be more papular more papular than in lightly than in lightly pigmented skin-pigmented skin-note associated note associated hyperpigmentathyperpigmentationion

Pityriasis Rubra PilarisPityriasis Rubra Pilaris

Chronic skin disease characterized by Chronic skin disease characterized by small follicular papules, disseminated small follicular papules, disseminated yellowish pink scaling patches, and yellowish pink scaling patches, and often, solid confluent palmoplantar often, solid confluent palmoplantar hyperkeratosishyperkeratosis

Disease generally manifests itself first Disease generally manifests itself first by scaliness and erythema of the scalpby scaliness and erythema of the scalp

PRPPRP

Involvement is usually symmetrical and Involvement is usually symmetrical and diffuse, with islands of normaldiffuse, with islands of normal

Hyperkeratosis of palms and soles called, the Hyperkeratosis of palms and soles called, the “sandal”“sandal”

Nails may be dull, rough, thick, and brittleNails may be dull, rough, thick, and brittle Itching in some casesItching in some cases Koebner’s phenomenon may be presentKoebner’s phenomenon may be present A number of cases have been associated A number of cases have been associated

with Kaposi’s sarcoma, leukemia, basal cell, with Kaposi’s sarcoma, leukemia, basal cell, lung, unknown primary metastatic and lung, unknown primary metastatic and hepatocellular carcinomahepatocellular carcinoma

PRPPRP

PRP may classified into familal or PRP may classified into familal or acquired typesacquired types

in respect to the onset of the disease in respect to the onset of the disease in childhood or adulthoodin childhood or adulthood

Griffth’s classification: Type I, the Griffth’s classification: Type I, the classic adult type, is seen most classic adult type, is seen most commonly, with 80% involuting in 3 commonly, with 80% involuting in 3 yearsyears

Three types of juvenile-onset forms Three types of juvenile-onset forms account for up to 40% of cases and account for up to 40% of cases and have a poor prognosis for involutionhave a poor prognosis for involution

PRPPRP

Etiology Etiology unknown-??AD unknown-??AD

Either sex Either sex affectedaffected

Possible related Possible related to deficiency of to deficiency of

vitamin Avitamin A

Histology: Histology: hyperkeratosis, hyperkeratosis, follicular plugging, follicular plugging, and focal and focal parakeratosis at parakeratosis at follicular orificefollicular orifice

Inflammatory Inflammatory infiltrate in dermis is infiltrate in dermis is composed of composed of mononuclear cellsmononuclear cells

PRP: psoriasiform dermatitis with PRP: psoriasiform dermatitis with follicular pluggingfollicular plugging

TreatmentTreatment

Symptomatic: Symptomatic: emollients-- Lac-Hydrinemollients-- Lac-Hydrin

A several-month course A several-month course of isotretinoin in doses of isotretinoin in doses of 0.5 – 2 mg/kg/dayof 0.5 – 2 mg/kg/day

Vitamin A in doses of Vitamin A in doses of 300,00 to 500,000 untis 300,00 to 500,000 untis daily, with possible daily, with possible addtion of vitamin E, addtion of vitamin E, 400 units 2-3 times daily400 units 2-3 times daily

Methotrexate Methotrexate 2.5mg 2.5mg alternating with alternating with 5mg daily5mg daily

Monitor and treat Monitor and treat secondary secondary infectionsinfections

Pityriasis Pityriasis rubra pilaris: rubra pilaris: diffuse diffuse erythroderma erythroderma with with desquamation desquamation and follicular and follicular hyperkeratosihyperkeratosiss

Pityriasis rubra Pityriasis rubra pilaris: follicular pilaris: follicular papules and papules and confluent confluent orange-red orange-red scaly plaques scaly plaques with islands of with islands of sparingsparing

Pityriasis rubra pilaris: orange-red Pityriasis rubra pilaris: orange-red waxy keratoderma of the palmswaxy keratoderma of the palms