Papulosquamous Disorders: Seb Derm, Psoriasis, Palmoplantar, Pust Derm, Erythroderma Chris Weyer D.O. Dermatology Resident, PGY-1 Northeast Regional Medical Center

Seborrheic Dermatitis 2-5 % of the population Chronic, superficial, inflammatory disease caused by the yeast, Malassezia globosa Most commonly affects the scalp Also, affects the eyebrows, ears, eyelids, nasolabial creases, lips, sternal area, axillae, submammary folds, umbilicus, groin, and gluteal crease Characterized by itchy, pink, yellow or erythematous patches that may be dry, moist, or greasy

Infantile Seborrheic Dermatitis Presents around 1 week after birth AKA “Cradle cap” Superimposed Candida may occur

Adult Seborrheic Dermatitis Erythematous, greasy yellow scale Symmetrical If extensive, think about immunosuppression: HIV, diabetes, and Parkinson’s disease

Histology of Seb Derm Epidermis is acanthotic Focal scale crust adjacent to follicular ostia Slight spongiosis Adult seb derm has superficial perivascular & perifollicular lymphocytic infiltrate composed mainly of lymphocytes Older lesions show irregular acanthosis & focal parakeratosis Histo features are combination of psoriasis & spongiotic derm

Treatment-Adolescent & Adult Regular use of medicated shampoos -- over-the-counter: sulfide, tar, zinc, pyrithionate Nizoral shampoo & cream Loprox shampoo & solution Elidel cream Corticosteroids for quick control or flares Key: leave shampoo on scalp for 3 minutes daily; apply before going into shower, as patient will not leave on shampoo for 3 minutes while in shower

Infantile seborrheic dermatitis Responds well to topical therapy Hydrocortisone 1% on the face & skin folds This may be combined with an antifungal agent for skin folds Remove scale after softening with an oil preparation or a weak keratolytic agent Reassurance

Psoriasis A common, chronic, recurrent, inflammatory disease of the skin characterized by round, circumscribed, erythematous, dry, scaling plaques of various sizes, covered by grayish white or silvery white, imbricated and lamellar scales Predilection for the scalp, nails, extensor surfaces, elbows, knees, umbilical, and sacral region

Psoriasis nail changes onycholysis, nail pitting, and oil spot phenomenon Psoriasis nail changes including onycholysis, nail pitting, and oil spot phenomenon

Psoriasis clinical features Koebner’s phenomenon – the appearance of typical lesions of psoriasis at sites of injury Auspitz’s sign – pinpoint bleeding when the psoriatic scale is forcibly removed, this occurs because of severe thinning of the epidermis over the tips of the dermal papilla Woronoff ring – is concentric blanching of the erythematous skin at or near the periphery of the healing psoriatic plaque

Pathogenesis Hyperproliferative disorder driven by inflammatory mediators TH1 cytokines- IL-2,6,8,12; IFN-g, TNF-a IL-8: accumulation of neutrophils IL-12: Primary TH1 signal, promotes IFN-g production

Inheritence Heredity has a high influence, incidence increases with generations Evidence that susceptibility to psoriasis is linked to class I and II MHC chromo 6 Genetic loci: PSORS1 (6); PSORS2 (17q) HLA asso: early onset -Cw6, -B57, and -DR7 late onset –Cw2

Inheritance cont It is also believed that any individual that has -B13 or –B17 has a fivefold risk of developing psoriasis pustular psoriasis: HLA-B27 guttate and erythrodermic psoriasis: -B13,-B17 Palmarplantar pust: -B8, -Bw35, -Cw7, -DR3

Epidemiology of psoriasis Seen in 1-2% of US population Equal frequency in both sexes Mean age of onset is 27 years Sunlight may improve psoriasis by decreasing T-cells Emotional stress, smoking, and alcohol aggravates psoriasis Increased incidence of celiacs, lymphoma

Drug-induced psoriasis May be induced by many drugs: beta blockers, lithium, and antimalarials, terbinafine, calcium channel blockers, captopril, glyburide, and lipid lowering agents, such as gemfibrozil

Pathology Regular epidermal hyperplasia with long, test-tube-shaped rete ridges Thinning of dermal papillae Granular layer is thin or absent Overlying parakeratosis Small collections of neutrophils (Munro microabcesses) may be present in the stratum corneum There is a perivascular mononuclear cell infiltrate

Types of Psoriasis Seborrheic-Like, “sebopsoriasis” Inverse Napkin Arthritis Guttate Generialized Pustular (von Zumbusch) Acro dermatitis Continua of Hallopeau Impetigo Herpetiformis Keratoderma Blennorrhagica Erythrodermic

Inverse Psoriasis Flexural or intertrigenous location Shiny erythematous plaques in the axilla that lack scale.

“Napkin” Psoriasis Infants 2-8 mo Erythematous sharply demarcated in diaper area Lesions typically clear w/ topical tx Infants may be at risk for psoriasis in adulthood

Five clinical patterns of psoriatic arthritis Asymmetrical DIP joint involvement with nail damage, 16% Arthritis mutilans with osteolysis of phalanges and metacarpals, 5% Symmetrical polyarthritis-like rheumatoid arthritis, with claw hands, 15% Oligoarthritis with swelling and tenosynovitis of one or a few hand joints, 70% Ankylosing spondylitis alone or with peripheral arthritis, 5%

Radiographic findings of psoriatic arthritis Erosion of terminal phalangeal tufts; tapering of phalanges or metacarpals; “cupping’ of proximal ends of phalanges; bony ankylosis; osteolysis of metatarsals; predilection for distal and proximal interphalangeal joints; paravertebral ossification; asymmetrical sacroiliitis; and “bamboo spine” Nearly half the patients with psoriatic arthritis have HLA-B27 Tx- biologics, MTX, cyclosporin- dz modifying prevent deformity

Guttate Psoriasis Usually, occurs as an abrupt eruption following an acute infection, such as streptococcal pharyngitis Occurs mostly in patients under age 30 Recurrent episodes are likely This type of psoriasis is usually rapidly responsive to topical steroids or UVB Tx 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 psoriasis (von Zumbusch) Typical patients have plaque psoriasis and often psoriatic arthritis The onset is sudden, with formation of lakes of pus periungally, on the palms, and at the edge of psoriatic plaques Pruritus, pain, fever, and malaise Fetid odor develops

Generalized pustular psoriasis (von Zumbusch) Etiology unclear Iodides, coal tar, steroid withdrawal, terbinafine, minocycline, hydroxychloroquine, acetazolamide, and salicylates may trigger the attacks May occur in infants Acitretin is drug of choice, with a rapid and predictable response Isotretinoin, cyclosporine, methotrexate, dapsone

Acrodermatitis Continua of Hallopeau Acral erythematious plaques with nail involvment Fingernails float away on lakes of pus: anonychia Fingers taper to long keratotic points

Impetigo Herpetiformis Generalized pustular psoriasis of pregnancy Retinoids not indicated Prednisone: controversial, may induce pustular flare, also matures fetal lung Early delivery strongly encouraged

Treatment- Topicals Corticosteroids- class I- 2wks, pulse, occlusion; rapid return when stopped Calcipotriene- keratinocyte differentiation; plaque and scalp type Tars- oils and shmp; stinks (literally) Macrolactams (tacrolimus, pimicrolimus)- prevent steroid atrophy/acne Salicylic acid- keratolytic Tazorac- modulate differentiation/proliferation Anthralin- SCAT, supresses neut superoxide, inhib mono IL-6,8/TNF-a

Treatment- Light NB-UVB (311-313nm), MED, 70% response Goeckerman Tech- 2-5% tar bath QD + UV; clear 18 d w/ long term response Ingram- tar bath QD/carbonisdetergens/ UV/ anthalin/talcum/stocking dressing PUVA- 2x/wk, clear 20-25 tx; risk cataracts, SCC, MM

Methotrexate First effective systemic drug for psoriasis and is the standard for systemic therapy Blocks synthesis of deoxyribonucleic acid, which decreases cell division Indications- erythroderma, arthritis, pustular, lg BSA

MTX For patients with no risk factors for liver disease, the first liver biopsy should be obtained at approximately 1.5g of cumulative methotrexate Weekly dosing for oral or IM – divided in three doses, 12 hours apart IM injections have a much lower risk of hepatotoxicity

Cyclosporine May down-modulate proinflammatory epidermal cytokines Rapid clearing and return Low risk of renal tox w/ <6mo tx Follow BP, Cr

Diet Most recent trials have demonstrated the antiinflammatory effects of fish oils rich in n-3 polyunsaturated fatty acids in rheumatoid arthritis, inflammatory bowel disease, psoriasis and asthma Gluten-free diet Koo J., Lee E., Lee C.S., Lebwohl M.. Psoriasis. J Am Acad Dermatol 2004;50:613-22.

Oral antimicrobial therapy Recent evidence suggest that Staphylococcus aureus and streptococci secrete a large family of exotoxins that are superantigens, producing massive T-cell activation Oral antibiotic for psoriasis patients infected with these organisms is imperative

Retinoids Treatment with 13-cis-retinoic acid can produce good results, especially in pustular psoriasis It is a potent teratogen Combinations of retinoic acids with photochemotherapy for chronic plaque psoriasis may also be very affective Etretinate- long ½ life, EtOH changes acitretin Acitretin – avoid pregnancy for up to 3 years following cessation of therapy

Dapsone Use is limited largely to palmoplantar eruptions 2nd-3rd line therapy

Combination therapy Patient on methotrexate may have their dose minimized with concomitant topical steroid use PUVA with acitretin, cyclosprine, or methotrexate Topical Dovonex with Acitretin, cyclosporine, methotrexate, and phototherapy

Biologic Agents Alefacept (Amevive) Efalizumab (Raptiva) Etanercept (Enbrel) Infliximab (Remicaide) Adalimumab (Humira) These are indicated for moderate-to-severe plaque psoriasis

Journal of the American Academy of Dermatology Volume 53 • Number 2 • August 2005 Copyright © 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

Journal of the American Academy of Dermatology Volume 53 • Number 2 • August 2005 Copyright © 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 syndrome Triad: urethritis, conjunctivitis, and arthritis Can’t see, can’t pee, can’t climb a tree! May also be other features that involve the skin , mucous membranes, GI tract, and cardiovascular system American Rheumatism Association - criterion of peripheral arthritis of more than 1 month duration, in association with urethritis and/or cervicitis Young men of HLA-B27 genotype

Clinical features May also have fever, weakness, and weight loss A nonbacterial urethritis may develop with painful and bloody urination and pyuria About 1/3 of patients develop conjunctivitis Keratitis is usually superficial and very painful. Iritis is common.

Reiter’s syndrome An asymmetric arthritis afflicts synovial joints, especially those that are weight bearing Onset is sudden with heat and tenderness and swelling Pain in one or both heels is a frequent symptom Sclerosing of the SI joints

Cutaneous lesions of Reiter’s The skin lesions start as multiple, small, yellowish vesicles that break, become confluent, and form superficial erosions Develop frequently on the genitals and palms Eruption on the glans penis occurs in 25% of patients

Cutaneous features The eruption is known as keratoderma blennorrhagicum Penile lesions are frequent Balanitis circinata Characterized by perimeatal balanitis Similar lesion are seen on the vaginal mucosa of women

Etiology Syndrome has been attributed to many different agents, such as: Shigella flexneri, Salmonella spp., yersinia spp., Ureaplasma urealyticum, Borrelia burgdorferi, Cryptosporidia, and Campylobacter fetus May be responsible for the infectious enteritis that precedes onset in a small percentage of patients In cases that follow an infection of the genitourinary tract, Chlamydia trachomatis may be associated HLA-B27 positivity is present in 80%

Laboratory findings Non-specific A leukocytosis of 10, 000 to 20, 000/mm3 and an elevated sed rate

Histopathology Identical to psoriasis

Differential diagnosis May be confused with rheumatoid arthritis, ankylosing spondylitis, gout, psoriatic arthritis, gonococcal arthritis, acute rheumatic fever, chronic mucocutaneous candidiasis, and serum sickness

Treatment Usually mucocutaneous lesions are self- limited and clear within a few months Topical steroids NSAIDs Methotrexate Cyclosporine Acitretin

Subcorneal pustular dermatosis (Sneddon-Wilkinson Disease) Chronic pustular disease, which occurs chiefly in middle-aged women Pustules are superficial and arranged in annular and serpiginous patterns on the abdomen, axillae and groin Vesicles may be present Cultures from pustules are sterile Oral lesions are rare Some cases occur in association with an IgA monoclonal gammopathy Desmocollin 1 autoantigen for Iga pemphigus

Subcorneal pustular dermatosis (Sneddon-Wilkinson Disease) Dapsone appears to be effective in most cases Sulfapyridine Acitretin NB-UVB Corticosteroids, colchicine, and TCN with niacinamide Without treatment this is a chronic condition with remissions of variable duration

Pathology of Sneddon-Wilkinson

Eosinophilic pustular folliculitis Also referred to as sterile eosinophilic pustulosis AKA Ofuji’s disease Males, Asia HIV Characterized by pruritic, follicular papulopustules arranged in groups Histo: eos in or around follicles

Eosinophilic pustular folliculitis Distribution is usually asymmetrical – affects face, trunk, and upper extremities Cause is unknown – numerous studies have implicated chemotactic substances, ICAM-1, and cyclooxygenase-generated metabolites Typical course – spontaneous remissions and exacerbations for a few months to several years

Eosinophilic pustular folliculitis Dapsone or systemic steroids are the treatment of choice Success with intralesional steroids, clofazimine, minocycline, isotretinoin, UVB therapy, indomethacin, colchicine, cyclosporine, and cetirizine

Recalcitrant Palmoplantar Eruptions Recalcitrant pustular eruptions of the hands and feet are often examples of psoriasis Need to then search for lesions elsewhere on the body(e.g., scalp, ears, glans penis) Search also for a family history to confirm your suspicion

Dermatitis Repens Aka- acrodermatitis continua and acrodermatits perstans It’s a chronic inflammatory disease of hands and feet Rarely, can become generalized Usually, as a pustule or paronychia

Dermatitis Repens Occasionally, mucous membranes are involved Nails are often dystrophic or destroyed Lesions cause skin atrophy Crusted, eczematoid, and psoriasiform lesions may occur, and there may be moderate itching It is essentially unilateral in its beginning and asymmetrical throughout its entire course

Dermatitis Repens Histology Treatment similar to those seen in psoriasis the primary lesion is epidermal An intraepithelial spongiform pustule is formed by infiltration of pmn’s Treatment topical mechlorethamine, topical steroids, PUVA, fluorouracil, and sulfapyridine Acitretin, low dose cyclosporine, Acitretin plus calcipotriol

Palmoplantar Pustulosis AKA pustular psoriasis In contrast to dermatitis repens it is essentially bilateral and symmetrical Locations include: thenar/hypothenar eminences or central portion of the palms and soles

Palmoplantar Pustulosis In the course of a week, they tend to dry up, leaving punctate brown scabs that eventually exfoliate Stages of quiescence and exacerbation characterize the condition Meds, such as lithium, have been reported to induce Patches begin as erythematous areas in which pustules form Start as pinhead-sized, enlarge and coalesce to form small lakes of pus

Palmoplantar Pustulosis Nails may become malformed, ridged, stippled, pitted and discolored May be associated with psoriasis vulgaris Some regard palmoplantar pustulosis as a form of psoriasis, while others consider it a separate entity Female predominance; lack of seasonal variation; different histopathologic features and Associated with thyroid disorders and cigarette smoking

Palmoplantar Pustulosis May be predisposed to joint disease and possibly SAPHO syndrome-Synovitis, Acne, Pustulosis, Hyperostosis and Osteoarthritis It’s resistant to most treatments Acitretin is reportedly effective(1mg/kg/day) Low-dose cyclosporine (1.25mg/kg/day-3.75mg/kg/day) Intramuscular Kenalog (40-60mg)may be effective for short-term relief

Palmoplantar Pustulosis 10-32.jpg

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Pustular Bacterid Characterized by a symmetric, grouped, vesicular or pustular eruption on palms and soles Marked by exacerbations and remissions over long periods No involvement of webs of fingers or toes or flexion creases of toes WBC may be elevated Scaling is usually present Etiology is thought to be a remote focus of infection; infection needs to be treated before resolution will occur

Juvenile Plantar Dermatosis Usually begins as a patchy, symmetrical, smooth, red, glazed macule on great toes, sometimes with fissuring and desquamation in children aged 3-13 Toe webs are rarely involved; fingers may be Histologically, there is psoriasiform acanthosis and a sparse, lymphocytic infiltrate in the upper dermis Spongiosis is commonly present Tx: bed rest, cotton socks and topical steroids Spontaneous resolution within 4 yrs is the rule

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Infantile Acropustulosis Intensely itchy vesicopustular eruption of hands and feet Begins at any age up to 10 months, clearing in a few weeks and recurring repeatedly until final resolution at 6 – 36 months of age Dapsone at 2mg/kg/day may help Potent topical steroids aid in symptomatic relief

Infantile Acropustulosis Should prompt an extensive workup to eliminate serious infectious causes (i.e., Tzanck prep, gram stain, KOH prep of pustule) Some suspect that this condition may be a persistent reaction to prior scabies

Acropustulosis of infancy

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