Andrews’ Diseases of the Skin

Andrews’ Diseases of the Skin
By:Boris Ioffe, D.O.

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
* 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
* Histology
o similar to those seen in psoriasis
o the primary lesion is epidermal
o An intraepithelial spongiform pustule is formed by infiltration of pmn’s
* Treatment
o topical mechlorethamine, topical steroids, PUVA, fluorouracil, and sulfapyridine
o 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
* Patches begin as erythematous areas in which pustules form
* Start as pinhead-sized, enlarge and coalesce to form small lakes of pus
* 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
* 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
* 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

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

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
* 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

Infantile Acropustulosis
* Acropustulosis of infancy
Pompholyx
* AKA dyshidrosis
* A vesicular eruption of palms and soles characterized by spongiotic intraepidermal vesicles and often accompanied by burning or itching
* Hyperhidrosis may be present
* Usually bilateral and symmetrical
* Bullae may form
* Contents are clear and colorless
* Attacks generally last a few weeks
* Lesions dry-up and desquamate rather than rupture
* Etiology- stress, atopy, and topical as well as ingested contactants
* Histopathology: spongiotic vesicles in the epidermis
* Differential dx:
o dermatophytid, contact dermatitis, atopic dermatitis, drug eruption, pustular psoriasis of palms and soles, acrodermatitis continua, and pustular bacterid
* Rarely, T-cell lymphoma can present with similar clinical findings, but biopsy of the vesicles will be diagnostic
* Tx: high potency corticosteroid creams
* Triamcinolone acetonide intramuscularly or a short course of oral prednisone is rapidly effective
* Oral or topical psoralen + UVA (PUVA) is effective but costly & inconvenient
* In more severe forms, immunosuppressive mycophenolate mofetil has been effective

Lamellar Dyshidrosis
* AKA dyshidrosis lamellosa, keratolysis exfoliativa
* A superficial exfoliative dermatosis of the palms and sometimes soles
* Referred to as recurrent palmar peeling
* Involvement is bilateral
* Can occur in association with dyshidrosis
* Often exacerbated by environmental factors
* Differential dx: dermatophytosis, chronic contact dermatitis
* Tx: difficult
* Spontaneous involution can occur in a few weeks for some
* Most tends to be chronic and relapsing
* Tar creams (Zetone cream) usually helps
* 5% tar in gel (Estar Gel) is an excellent tx
* Lac-Hydrin lotion and Carmol 10 or 20 are often effective
* NB-UVB may be helpful

Palmoplantar Keratoderma
* AKA tylosis, keratosis, hyperkeratosis
* Characterized by excessive formation of keratin on the palms and soles
* Acquired
o Keratosis Punctata of the Palmar Creases
o Punctate Keratoses of the Palms and Soles
o Porokeratosis Plantaris Discreta
o Keratoderma Climactericum
* Congenital


Punctate Keratosis of the Palms and Soles
* Primary lesion is a 1-5mm round to oval, dome-shaped papule distributed over left hand and hypothenar eminence
* Main symptom is pruritis
* Lesions number from 1 to >40
* Affects mainly blacks
* There’s a potential risk of developing lung and colon cancer

Keratosis Punctata of the Palmar Creases
* Common most often in black pts
* Primary lesion is a 1-5mm depression filled with a conical keratinous plug
* Primarily, in creases of palms or fingers, occasionally in soles
* Lesions are multiple
* Friction aggravates lesions causing them to become verrucoid or surrounded by callus
* Punctate keratoses of the palmar creases in an African-American
* PPPK-punctate palmoplantar keratoderma

Porokeratosis Plantaris Discreta
* Occurs in adults, Female:Male (4:1)
* Characterized by sharply marginated, rubbery, wide-based papule that does not bleed on removal
* Lesions are multiple, painful, 7-10mm in diameter
* Usually on wt bearing areas of sole, beneath metatarsal heads
* Tx: foot pads to redistribute wt, surgical excision, blunt dissection

Keratoderma Climactericum
* Characterized by hyperkeratosis of palms and soles beginning at about the time of menopause
* Descrete, thickened, hyperkeratotic patches most pronounced at pressure sites
* Fissuring may be present
* Tx: keratolytics -- 10% salicylic acid, lactic acid creams, etc.

Hereditary syndromes
* These have palmoplantar keratoderma as a feature
o Unna-Thost
o Papillon-Leferve
* Dominant inheritance; congenital thickening of epidermal horny layer of the palms and soles
* Usually symmetrical
* Epidermis becomes thick, yellowish, verrucous, and horny
* Striate and punctate forms occur

Unna Thost
* Occasionally nails become thickened
* 5% salicylic acid may help
* Lac Hydrin 12% may be tried
* Acitretrin or isotretinoin may be considered, but need for lifetime tx makes them impractical
* Focal palmoplantar keratosis of the striate type on the sole
* Diffuse non-epidermolytic palmoplantar keratosis
* Diffuse epidermolytic palmoplantar keratosis with diffuse hyperkeratosis

Papillon-Lefevre Syndrome
* Palmoplantar hyperkeratosis with peridontosis
* Usually develops within the first few months of life but may occur in childhood
* Well demarcated, erythematous, hyperkeratotic lesions on palms and soles
* Transverse grooves of fingernails may occur
* Early onset peridontal disease has been attributed to damage and alteration in PMN function caused by Actinomyces actinomycetemcomitans
* Disease associations include: acroosteolysis, and pyogenic liver abcesses
* There are asymptomatic ectopic calcifications in the choroid plexus and tentorium
* Therapy may retard both dental and skin abnormalities
* Treatment with Acitretin in four siblings was reported to be effective

Papillon-Lefevre Syndrome
* Papillon-Lefevre syndrome: plantar keratoderma

Mutilating Keratoderma of Vohwinkel
* Palmoplantar hyperkeratosis of the honeycomb type-associated with starfish-like keratosis on backs of hands and feet; linear keratoses of the elbows and knees, and annular constriction (pseudo-ainhum) of the digits, this may progress to autoamputation
* More than 30 cases have been reported world-wide
* More common in women and in whites
* Onset is in infancy or early childhood
* Vohwinkel’s mutilating syndrome: A.) diffuse keratoderma of palms with B.) pseudoaainhum formation

Palmoplantar Keratodermas & Malignancy
* Diffuse, waxy keratoderma of palms and soles occurring as an AD trait associated with esophageal carcinoma
* Other related factors are oral leukoplakia, esophageal srictures, squamous carcinoma of tylotic skin, carcinoma of larynx and stomach
* Acquired forms of palmoplantar keratodermas have also been associated with carcinoma of esophagus, lung, breast, bladder and stomach
* Focal PPK in association with carcinoma of the esophagus

Acrokeratoelastoidosis of Costa
* AD, more common in women
* Small, round, firm papules occurring over dorsal hands, knuckles, and lateral margins of palms and soles
* Appears in early childhood and progress slowly
* Most often asymptomatic
* Significant histologic finding is dermal elastorrhexis
* Therapies: liquid nitrogen, salicylic acid, tretinoin, and prednisone have been tried
* Focal acrokeratoelastoides: multiple skin-colored papules at the margin of the palmar skin
* Path: non-epidermolytic palmoplantar keratosis, acanthosis and hypergranulosis

Exfoliative Dermatitis
* Universal or very extensive scaling and itching erythroderma
* Often associated with hair loss
* Initially with erythematous plaques, which spread rapidly
* Onset accompanied by general toxicity
* Skin becomes scarlet and swollen and may ooze a straw-colored exudate
* Desquamation is evident within a few days

Etiology
* Most common is preexisting dermatoses: (53%);
o atopic dermatitis, chronic actinic dermatitis, psoriasis,seborrheic dermatitis, vesicular palmoplantar eczema, pityriasis rubra pilaris, and contact dermatitis
* Drug eruptions(5%);
o allopurinol, gold, carbamazepine, phenytoin, and quinidine
* Cutaneous T-cell lymphoma(13%); Sezary syndrome and mycosis fungoides
* Paraneoplstic (2%); carcinoma of the lung and carcinoma of the stomach
* Leukemia cutis (1%)
* Idiopathic (26%)
* Mortality rate at a mean follow-up interval of 51 months was 43%
Histology
* Most commonly, histology is nonspecific
* Hyperkeratosis & focal parakeratosis
* Epidermis shows mild acanthosis, scant superficial upper dermal infiltrate of mononuclear cells
* May be small areas of spongiosis
* Generalization after withdrawal of methotrexate
* Exfoliation of scale with underlying erythema
* Generalized erythema with thick scale and crusted fissures on the plantar surface
Treatment
* Topical steroids, soaks, and compresses
* Acitretin and cyclosporin-useful in psoriatic erythroderma, and isotretinoin in erythroderma caused by RPR; methotrexate
* Systemic corticosteroids in severe cases
* Discontinuing the offending drug in drug-induced cases
* Subungual hyperkeratosis and distal dystrophy

Parapsoriasis, Pityriasis Rosea, Pityriasis Rubra Pilaris
Parapsoriasis
* Group of macular scaly eruptions with slow evolution
* These are all markedly chronic, resistant to treatment, and are without subjective symptoms
* They are divided into: pityriasis lichenoides chronica, pityriasis lichenoides et varioliformis acuta, and parapsoriasis en plaques

Pityriasis Lichenoides Chronica
* Erythematous, yellowish, scaly macules and lichenoid papules
* They persist indefinitely without change
* Mainly on sides of trunk, thighs, and upper arms
* May be confused with psoriasis and secondary syphilis
* Tx- UV light is beneficial; however intense doses may be needed for good results
* PUVA has been reported to be effective
* Oral tetracycline may be used with antihistamines
* PLC is a benign disease that clears spontaneously in a few yrs to months
PLEVA
* AKA: parasoriasis lichenoides, Habermann’s disease, Mucha-Habermann disease and parapsoriasis varioliformis acuta
* Sudden appearance of a polymorphous eruption composed of macules, papules, and occasional vesicles
* May run an acute, subacute, or chronic course
* Papules are usually yellowish or brownish-red, round lesions, which tend to crust, become necrotic and hemorrhage
* When exanthem heals it leaves a smooth, pigmented, depressed, varioliform scar
* Favorite sites are anterior trunk, flexural arms, and axillae
* Palms and soles are involved infrequently-mucous membranes are not
* Generalized lymphadenopathy can occur
* Usually a benign, self-limited disorder, but may be more chronic and severe
* Maybe a spectrum of cutaneous T-cell lymophoma
* Differential dx:
o leukocytoclastic angiitis, papulonecrotic tuberculid, psoriasis, lichen planus, varicella, PR, drug eruptions, maculopapular syphilid, viral, rickettsial diseases, lymphomatoid papulosis
* Histologically of PLEVA is characterized by epidermal necrosis, with prominent hemorrhage and primarily a dense perivascular infiltrate of lymphocytes in the superficial dermis
* Absence of neutrophils simplifies the distinction between leukocytoclastic angiitis
* Lymphomatoid papulosis differs by the presence of large, atypical mononuclear cells in the dermal infiltrate

PLEVA-Tx
* No one tx is reliably effective
* Tetracycline and erythromycin are worth trying
* UVB and PUVA
* Methotrexate, 2.5-7.5mg every 12 hrs for 3 doses 1 day each week
* Several serious reactions a few of them fatal have occurred with simultaneous administration of methotrexate and NSAIDs
* Dapsone and pentoxifylline(Trental), 400mg twice daily


Parapsoriasis en Plaques
* Small-plaque parapsoriasis is characterized by non-indurated, brownish, hypopigmented, or yellowish red scaling patches, round to oval, with sharply defined borders
Large Plaque Parapsoriasis
* Has patches 5-15 cm; otherwise is similar to small-plaque type
* Prognosis is benign, especially if pruritis is severe
* 10% may eventuate in T-cell lymphoma
* Large plaques parasporiasis: large, variably erythematous and mildly poikilodermatous patches in the bathing trunk region
* Small plaque parasporiasis: small(<5), erythematous, slightly scaly patches
Treatment
* First line: UV radiation -- either natural or UVB
* Lubricants and Topical steroids
* PUVA but only if UVB fails
* Use of PUVA or high-potency topical streroids should be limited due to long-term adverse effects
* LPPP has the potential to develop lymphoma – thus, justifying more intense tx
* Vitamin D2 daily–250,000 units over 2-4 months has been effective

Pityriasis Alba
* AKA-pityriasis streptogenes, furfuraceous impetigo, pityriasis simplex, pityriasis sicca faciei, and erthema streptogenes
* Characterized by hypopigmented, round to oval, scaling patches on face, upper arms, neck, or shoulders
* Color is white (but never actually depigmented) or light pink
* Scales are fine and adherent
* Patches are usually sharply demarcated; edges may be erythematous and slightly elevated
* Lack of any early specifically follicular localization helps to distinguish this lesion from follicular mucinosis
* Vellus hairs are not lost in pityriasis alba, nor does hypesthesia to cold occur, as often happens in follicular mucinosis
* Usually asymptomatic; however there may be mild pruritis
* Disease mainly occurs in children and teenagers
* It is particularly a cosmetic problem in dark-skinned individuals
* Etiology unknown
* Excessively dry skin appears to be contributory
* Most lesions disappear with time
* Repigmentation can be accelerated with treatment
* Emollients and bland lubricants
* Low-strength corticosteroids plus Lac-Hydrin are helpful
* Others have recommended PUVA

Pityriasis Rosea
* Mild inflammatory exanthem of unknown origin ?viral
* Characterized by salmon-colored papules and patches which are oval and covered with a collarette of scale
* Disease frequently begins with a single herald patch, which may persist a week or more, then involutes
* Appears rapidly and last from 3-8 weeks
* Peak: ages 15-40
* Typically in Spring and Autumn
* More common in women
* Mainly affects the trunk
* Oral lesions are relatively uncommon, but present as aphthous lesions

Herald Patch
Pityriasis Rosea
* Papular PR is an unusual form common in black chidren under age 5
* Inverse PR is unusual, but not rare
* Relapses and recurrences are frequently observed
* A PR-like eruption can occur as a rxn to captopril, arsenicals, gold, bismuth, clonidine, methoxypromazine, tripelennaminehydrochloride, or barbituates
* Inverse pityriasis rosea: oval annular plaques in groin

Treatment
* Supportive
* UVB should be used after acute inflammatory stage has passed
* Topical corticosteroids
* Antihistamines
* Emollients
* PR: There is focal parakeratosis, mild acanthosis, spongiosis, perivascular lymphocytes, and focal erythrocyte extravasation
* PR: papules and annular plaques
* PR: oval and round plaques, some with central scale and others with a collarette of scale
* PR in darkly pigmented skin: it tends to be more papular than in lightly pigmented skin-note associated hyperpigmentation

Pityriasis Rubra Pilaris
* Chronic skin disease characterized by small follicular papules, disseminated yellowish pink scaling patches, and often, solid confluent palmoplantar hyperkeratosis
* Disease generally manifests itself first by scaliness and erythema of the scalp

PRP
* Involvement is usually symmetrical and diffuse, with islands of normal
* Hyperkeratosis of palms and soles called, the “sandal”
* Nails may be dull, rough, thick, and brittle
* Itching in some cases
* Koebner’s phenomenon may be present
* A number of cases have been associated with Kaposi’s sarcoma, leukemia, basal cell, lung, unknown primary metastatic and hepatocellular carcinoma
* PRP may classified into familal or acquired types

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

* Griffth’s classification: Type I, the classic adult type, is seen most commonly, with 80% involuting in 3 years
* Three types of juvenile-onset forms account for up to 40% of cases and have a poor prognosis for involution
* Etiology unknown-??AD
* Either sex affected
* Possible related to deficiency of

vitamin A
* Histology: hyperkeratosis, follicular plugging, and focal parakeratosis at follicular orifice
* Inflammatory infiltrate in dermis is composed of mononuclear cells
* PRP: psoriasiform dermatitis with follicular plugging
Treatment
* Symptomatic: emollients-- Lac-Hydrin
* A several-month course of isotretinoin in doses of 0.5 – 2 mg/kg/day
* Vitamin A in doses of 300,00 to 500,000 untis daily, with possible addtion of vitamin E, 400 units 2-3 times daily
* Methotrexate 2.5mg alternating with 5mg daily
* Monitor and treat secondary infections
* Pityriasis rubra pilaris: diffuse erythroderma with desquamation and follicular hyperkeratosis
* Pityriasis rubra pilaris: follicular papules and confluent orange-red scaly plaques with islands of sparing
* Pityriasis rubra pilaris: orange-red waxy keratoderma of the palms

Andrews’ Diseases of the Skin.ppt

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Cutaneous Vascular Diseases

Cutaneous Vascular Diseases
by:Rick Lin, DO MPH
KCOM Dermatology Residency Program

Raynaud’s Phenomenon
* Intermittent constriction of the small digital arteries and arterioles
* Persistently cyanotic and painful
* Aggrevated by cold weather
* Young middle aged women
* Assoc c scleroderma, dermatomyositis, LE, Mixed connective tissue diseases, Sjogren’s RA, and paroxysmal hemoglobinuria.
* Scleroderma is the underlying condition for more than half of the patients
* Maybe caused by medications, ie bleomycin

The LDI images below graphically illustrate the vasospasm of Raynaud’s phenomenon following a cold provocation.

Raynaud’s Disease
* Primary disorders
* Pallor, cyanosis, hyperemia, and numbness of the finger
* Precipitated by cold.
* Present for 2 years with out associated disease finding
* Good prognosis
* Multifactorial.
* Increase alpha-2 sympathetic receptor activity on vessels.
* Endothelia dysfunction
* Deficiency in calcitonin gene related protein
* Central thermoregulatory defects
* Treatment include avoidance of aggravating factor, ie cold.
* Vasodilating drugs, nifedipine, 10-20 mg tid; prazosin 1-3 mg tid
* Nitroglycerin 2% local application

Erythromelalgia
* Aka erythermalgia and acromelalgia
* May be secondary to myeloproliferative disease such as polycythemia vera, TTP
* Responds to treatment of primary disorders
* Cold water immersion

Livedo Reticularis
* Mottled or reticulated pink/reddish/blue discoloration
* Assoc c LE, DM, scleroderma, RA
* Side effect of amantadine

Necrotizing livedo reticularis
* Assoc c nodules and ulcerations
* Result from sever atherosclerotic disease
* Sneddon’s syndrome

Livedoid Vasculitis
* atrophie blanche
o White stellate scars of ulcers
* PURPLE (Painful purpuric ulcer with reticular pattern of the lower extremity)
* Histologically shows chronic perivascular hemorrhage.

Treatment
* Low Dosage of Aspirin 325mg qd
* Nifedipine 10mg TID
* Pentoxifylline 400mg BID-TID

Marshall-White Syndrome
* Bier’s Spot: White spot appear on hand with blood pressure cuff
* Consist of Bier’s spot and is associated with insomnia and tachycardia
* White middle age men

Purpura
* Multifocal extravasation of blood into the skin
* Petechiae <3mm
* Ecchymosis
* Vobices (vibex) – Linear
* Hematoma – pool-like collection
* Complete blood count
* PT and PTT

Thrombocytopenic Purpura
* Three Large Categories:
o Accelerated platelet destruction
o Deficient platelet production
o Unknown pathogenesis

Idiopathic Thrombocytopenic Purpura
* Aka autoimmune thrombocytopenic purpura
* Aka Werlhof’s disease
* Bleeding occurs when platelet count drops below 50,000
* Risk greatly increased for serious hemorrhage when count goes below 10,000
* Acute variety occurs in children following season viral illness in 50% of the patient.
* Lag between illness and onset of purpura is 2 weeks
* Resolve spontaneously with minimal therapy
* Chronic case may result in death.
* Chronic form most often occur in adult
* Evaluate patient with Tc99M radionuclide scan to look for accessory spleen
* Result of platelet injury by antibodies of IgG class
* Treatment include Splenectomy, systemic corticosteroid, IVIg

These are the kidneys from a case of idiopathic thrombocytopenic purpura. Petechiae are found throughout the renal parenchyma.

Drug-Induced Thrombocytopenia
* Drug induced antiplatelet antibodies
* May be caused by sulfonamides, digoxin, quinine, quinidine, PCN, furosemide, Lidocaine, methyldopa

Thrombotic Thrombocytopenic Purpura
* Aka Moschcowitz syndrome
* Pentad of thrombocytopenia, hemolytic anemia, renal abnormalities, fever, CNS disturbance.
* Delay in diagnosis may lead to a mortality rate as high as 90%
* Positive histologic diagnosis require gingival biopsies looking for subendothelial hyaline deposits
* Exchange plasmapheresis is required for treatment. 80% patient survive if treatment is instituted.

Dysproteinemic Purpura
* Aka Nonthrombocytopenic purpura
* Aka purpura cryoglobulinemica
* Aka cryofibrinogenemia
* Occur most frequently in multiple myeloma and macroglobulinemia of monoclonal IgM, IgG, or Bence Jones cryoglobulin.
* Tx with plasmaphoresis, systemic steroid, and immunosuppressors.

Purpura Hyperglobulinemica
* Aka Waldenstrom’s hyperglobulinemic purpura
* Consist of episodic showers of petechiae occuring on all parts of body
* Diffuse peppery distribution, resembling Schamberg’s
* Most useful labtest is protein electrophoresis
* Hyperglobulinemic purpura occurs most commonly in women.
* Frequently seen with Hepatitis C and Sjogren’s syndrome, keratoconjunctivitis sicca, RA
* Histologically: derma vessels with perivascular infiltrate of mononuclear cells.
* Benign and chronic course. Assoc c various of connective tissue diseases.

Waldenstrom’s Macroglobulinemia
* Bleeding from mucous membrane of the mouth and nose, lymphadenopathy, hepatomegaly, retina hemorrhage, and RARELY the purpura
* Perivasular infiltrate containing lymphocytes and neutrophils and eosinophils
* Plasmaphoresis until adequate dose of chlorambucil is administered. Cyclophosphamide and corticosteriods are treatment options as well

A Skull X Ray showed a single, small, left-sided lytic Defect.
Drug- and Food Induced Purpura
* Drug induced purpura may occurs without platelet destruction.
* Cocaine induced thrombosis with infarct skin assoc c skin popping.
* Rumpel-Leede sign: distal shower of petechiae that occurs immediately after the release of pressure from a tourniqut release. Associated with capillary fragility.
* Topical EMLA can induce purpura in 30m.
Solar Purpura
* Large, sharply outlined 1-5 cm dark purplish red ecchymoses on dormsum of the forearm
* Less frequently, back of the hand

Purpura Fulminans
* Aka purpura gangrenosa
* Severe, rapidly fatal reaction occurring most commonly in children after infectious illness
* May follows scarlet fever, strep pharyngitis, and meningococcal meningitis.
* Assoc c Protein C or S deficiency in Neonates
* Management is supportive
* Protein C replacement if protein C deficiency is present
* Fresh frozen plasma maybe useful

Disseminated Intravascular Coagulation
* Up to 2/3 of DIC patients have skin lesions
* Minute, widspread petechiae, ecchymoses, ischemic necrosis of the skin and hemorrhage bullae.
* Elevated PT and PTT, fibrin degradation products
* Decrease platelets, decreased fibrinogen

Disseminated Intravascular Coagulation
* All patient needs to receive vitamin K replacement to exclude vitamin K deficiency.

Fibrinolysis Syndrome
* An acute hemorrhagic state brought by inability of the blood to clot
* Massive hemorrhages into the skin produce blackish, purplish swelling.
* Can be a complication of pregnancy in cases of placental previa, eclampsia, and fatal death
* Excessive fibrinolysis

Blue Muffin Baby
* Purpuric lesions observed in newborns with congenital rubella
* Assoc c disease that produce extramedullary erythropoesis
* Generalized dark blue to magenta nonblanchable, indurated, round, oval hemispheric papules 1-7mm
* Evaluation c biopsy, TORCH serology, CBC, viral culture.
Itching Purpura

* Aka diseminated pruriginous angiodermatitis
* Orange-purpleish-red petechiae evolve completely and may become confluent in 2 weeks
* Runs its course in 3-6 months. May become chronic
* Unknown etiology

Deep Vein Thrombosis
* Almost always affect femoral vein
* Leads to Reversible ischemia or frank gangrene
* Significant superficial vein thrombosis is a risk factor for DVT
* Pulmonary Embolism is a major concern
* Malignant Neoplasms are the most common underlying condition
* Pulmonary Embolism has 40% mortality
* DVT is assoc c 35% of cancer associated causes as the first sign
* Patient younger than 40 with DVT prompt for search for cancer

Superficial Thrombophlebitis
* Painful induration with erythema
* Linear or branching configuration forming cords
* Assoc c hypercoagulable state
* Need to be evaluated for possibility of deep veous disease

Mondor’s Disease
* 3:1 = women:men
* Age range 30-60
* Sudden appearance of a cord like thrombosed vein along the anterior-lateral chest wall
* First red and tender and subsequently change into a painless tough, fibrous band.
* No systemic symptom associated
* Treatment of the symptom: hot moist dressing with NSAID
* Runs its course for 3-6 months.

Calciphylaxis
* End-stage renal disease patients with metastatic calcification are most exclusively affected by this disease.
* Reticulated violaceous, mottled patches
* Progress into ecchymosis, central necrosis, and ulceration
* 50%+ morbidity and mortality
* Death is usually caused by staphylococcal sepsis after infection
* Hyperbaric oxygen has used with some success

* On low magnification, basophilic alteration of a fibrous septum can be seen Fibrin thrombi are also present within many of the blood vessels of the subcutaneous adipose tissue (Figure 3). Higher magnification identifies calcium deposition within the fibrous septum, primarily on elastic fibers (Figure 4, Figure 5). Higher magnification of the same area with Verhoeff-van Gieson stain confirms the presence of fragmented elastic fibers (Figure 6). Special stain for calcium (von-Kossa) identifies calcium deposition both within the septum of the fat lobule (Figure 7) and within the walls of blood vessels (Figure 8).
Fibrin thrombi are also present within many of the blood vessels of the subcutaneous adipose tissue

Higher magnification of the same area with Verhoeff-van Gieson stain confirms the presence of fragmented elastic fibers

Special stain for calcium (von-Kossa) identifies calcium deposition both within the septum of the fat lobule

Scorbutic Purpura
* Bleeding gums
* Deficiency in Vitamin C
Achenbach’s Syndrome
* Aka Paroxysmal Hand Hematoma
* Spontaneous focal hemrhage into palm or volar suface
* Transitory localized pain followed by rapid swelling and bluish discoloration
* Acute nature with rapid resolution

Painful Bruising Syndrome
* Aka Autoerythrocyte Sensitization
* Aka Gardner-Diamond Syndrome
* Distinctive localized purpuric reaction
* Young and middle-aged women with some emotional disturbance
* Emotional upset is the precipitating factor
* Intracutaneous injections of erythrocytes stroma evoke lesions
* Some believe the symptome to be artifactual.

Psychogenic Purpura
* Similar purpura as Painful bruising syndrome
* Absence of erythrocytes sensitivity
* Secretan’s syndrome: factitial lymphedema of the hand
* L’oedeme bleu: factitial lymphadema of the arm

Pigmentary Purpuric Eruption
* Pigmented purpuric eruptions of the lower extremities
* Similar histologic finding
* Shamberg’s
* Majocci’s
* Gougerot-Blum

Shamberg Diseases
* Aka progressive pigmentary dermatosis
* Grains of cayenne pepper
* Lesions seldom itch
* Favors lower shins and ankles

Majocchi’s Disease
* Aka purpura annularis telangiectodes
* Bluish annular macules 1-3cm in diameter with telangiectatic puncta
* Begins symmetrically at lower extremities
* Involution requires as long as a year, and may prolong indefinitely
* Asymptomatic.
Gougerot-Blum
* Pigmented purpuric lichenoid dermatitis
* Minute, rust-colored lichenoid papules that fuse into plaques of various hues
* Legs, thighs, and lower trunk
* Differentiate from Schamberg based on distribution and lichenoid lesions.
* Lichen Aureus

Ducas Kapetanakis pigmented purpura
* Histologically present with distinguished from others by presence of spongiosis
* Must be distinguished from mycosis fungoides
Histology

* Purupra Majocchi-Schamberg is characterized by slight alteration of superficial capillaries with hemorrhage and perivascular lymphocytic infiltrate.

Cutaneous Vascular Diseases Part-1.ppt
Cutaneous Vascular Diseases Part-2.ppt

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Syphilis

Syphilis
by: Erik Austin, D.O., M.P.H.

Syphilis
* AKA lues
* Contagious, sexually transmitted disease caused by the
* Spirochete: Treponema pallidum
* Enters through skin or mucous membrane where primary manifestations are seen

Treponema pallidum
* Spiral spirochete that is mobile
* # of spirals varies from 4 to 14
* Length is 5 to 20 microns
* Can be seen on fresh primary or secondary lesions by darkfield microscopy or fluorescent antibody techniques

Syphilis epidemiology
* Major health problem throughout world
* 2.6 cases per 100,000 in 1999 in the US
* Lowest level ever recorded
* Concentrated in 28 counties in the SE U.S.
* Mainly gay men and crack cocaine users
* Enhances risk of transmission of HIV
* HIV testing recommended in all patients with syphilis
* Reportable disease

Serologic Tests
* Testing reveals patients immune status not whether they are currently infected
* Non-treponemal antigen test uses lipoidal antigens rather than T. pallidum or components of it
* RPR = rapid plasma reagin
* VDRL = Venereal Disease Research Laboratory
* Positive within 5 to 6 weeks after infection
* Strongly positive in secondary phase
* Strength of reaction is stated in dilutions
* May become negative with treatment or over decades
* MHA-TP: microhemagglutination assay for T. pallidum
* FTA-ABS: fluorescent treponemal antibody absorption test
* All positive nontreponemal test results should be confirmed with a specific treponemal test
* Treponemal tests become positive early, useful in confirming primary syphilis
* Remain positive for life, useful in diagnosing late disease
* Treatment results in loss of positivity in 13-24% of patients

Biologic False-Positive Test Results
* Positive test with no history or clinical evidence of syphilis
* Acute BFP: those that revert to negative in less than 6 months
* Chronic BFP: those that persist > 6 months

BFP Test Results in Syphilis
* Acute BFP
* Vaccinations
* Infections
* pregnancy
* Chronic BFP
* Connective tissue disease (SLE)
* Liver disease
* Blood transfusions
* IVDA

Cutaneous Syphilis
* Chancre is usually the first cutaneous lesion
* 18 to 21 days after infection
* Round indurated papule with an eroded surface that exudes a serous fluid
* Usually painless and heals without scarring

Chancre
* Inguinal adenopathy 1-2 weeks after chancre
* Generally occur singly, but may be multiple
* Diameter mm to cm

Chancres
* In women, the genital chancre is less often observed due to location within the vagina and cervix
* Edema of labia may occur
* Untreated, the chancre heals spontaneously in 1 to 4 months
* Constitutional symptoms begin just as chancres disappear
* Extragenital chancre: may be larger, frequently on lips, rarely tongue, tonsil, breast, finger, anus.

Chancre Histology
* Ulcer covered by neutrophils and fibrin
* Dense infiltrate of lymphocytes and and plasma cells
* Spirochetes seen with with silver stains; Warthin-Starry
* Direct fluorescent antibody tissue test (DFAT-TP) = serous exudate collected on a slide sent for exam

Serology
* Nontreponemal tests positive 50%
* Treponemal tests positive 90%
* Positivity depends upon duration of infection, if chancre has been present for several weeks, test is usually positive

Chancre vs. Chancroid
* Incubation 3 weeks
* Painless
* Hard
* Lymphadenopathy may be bilateral, nontender, nonsuppurative
* Incubation 4-7 days
* Painful
* Soft
* Lymphadenopathy unilateral, tender, suppurative

DDx in Syphilis
* Chancroid - multiple lesions, may coexist with chancre, must r/o syphilis
* Granuloma Inguinale - indurated nodule that erodes, soft red granulation tissue, Donovan bodies in macrophages with Wright or Giemsa stain
* Lymphogranuloma Venereum - small, painless, superficial non indurated ulcer, primary lesions followed in 7 to 30 days by adenopathy
* HSV - grouped vesicles, burning pain

Secondary Syphilis
* Skin manifestations in 80% called syphilids
* Symmetric, generalized, superficial, macular - later papular, pustular
* May affect face, shoulders, flanks, palms and soles, anal or genital areas

Secondary Syphilis Macular Eruptions
* Exanthematic erythema 6-8 weeks after chancre - may last hours to months
* Round, slightly scaly ham-colored macules
* Pain and pruritus may be present
* Generalized adenopathy

Secondary Syphilis Papular Eruptions
* Occurs on face and flexures of arms, legs, and trunk
* Yellowish-red spots may appear on palmar and plantar surfaces
* Ollendorf’s sign = tender papule
* May produce a psoriasiform eruption
* May appear as minute scale-capped papules
* Tend to be disseminated, but may be localized, asymmetrical, configurate, hypertrophic or confluent.
* Annular syphilid - mimics sarcoidosis and is more common in blacks
* Pustular syphilid – rare - face, trunk, extremities red small crust-covered ulceration
* Rupial syphilid - superficial ulceration is covered with a pile of terraced crusts resembling an oyster shell.
* Lues Maligna - rare, severe ulcerations, pustules, or rupioid lesions, accompanied by severe constitutional symptoms.
* Condylomata lata - papular mass, weeping, gray 1-3cm, groin, anus (not vegetative like condylomata acuminata)
* Syphilitic alopecia - irregular, scalp has a moth-eaten appearance 5% of pts

Secondary Syphilis Mucous Membrane
* Present in 1/3 of secondary syphilis
* Most common is “syphilitic sore throat”
* Diffuse pharyngitis, hoarseness
* Tongue may show patches of desquamation of papillae
* Ulcerations of tongue and lips in late stages
* Mucous patches are the most characteristic mucous membrane lesions; macerated, flat. Grayish, rounded erosions covered by a delicate, soggy membrane.

Secondary Syphilis Systemic Involvement
* Lymphadenopathy common.
* Acute glomerulonephritis, gastritis, proctitis, hepatitis, meningitis, iritis, uveitis, optic neuritis, Bell’s palsy, pulmonary nodular infiltrates, osteomyelitis, polyarthritis.

Secondary Syphilis Diagnosis
* Nontreponemal serologic tests for syphilis are strongly reactive (seronegativity rarely in AIDS)
* Spirochetes on darkfield exam

Secondary Syphilis DDx “Great Imitator”
* Pityriasis rosea
* Drug eruptions (pruritic)
* Lichen planus; Wickham’s striae, Koebner’s, pruritic
* Psoriasis; no adenopathy
* Sarcoidosis; need serology and silver staining of biopsy
* Infectious mononucleosis, false pos RPR
* Geographic tongue
* Aphthous stomatitis

Latent Syphilis
* After the lesions of secondary syphilis have involuted, a latent period occurs where the patient has no clinical signs, but positive serological tests
* May last a few months or a lifetime
* 60-70% of pts that are untreated remain asymptomatic for life
* Women may infect unborn child for 2 years
Late Syphilis
* Defined by CDC as infection of greater than 1 years duration
* Tertiary Cutaneous Syphilis
* Late Osseous Syphilis
* Neurosyphilis
* Late Cardiovascular Syphilis

Tertiary Cutaneous Syphilis
* Tertiary syphilids usually occur 3-5 years after infection
* 16% of untreated pts will develop lesions of skin, mucous membrane, bone or joints
* Skin lesions are localized, destructive, heal with scarring

Tertiary Syphilids
* Two main types; Nodular syphilid and the Gumma
* Nodular - reddish brown firm papules or nodules 2mm or larger, scales.
* Gumma - larger

Nodular Tertiary Syphilid
* Lesions tend to form rings and undergo involution as new lesions develop
* Characteristic circular or serpiginous pattern
* “kidney-shaped” lesion occurs on the extensor surfaces of the arms and on back
* Patches have scars and fresh ulcerated lesions
* Process may last for years, slowly marching across large areas of skin

Gumma
* May occur as unilateral, isolated, single or disseminated lesions, or serpiginous
* May be restricted to the skin, or originate in deeper tissues, and break down the skin
* Lesions begin as small nodules, enlarge to several centimeters
* Central necrosis, deep ulcer with a gummy base, most frequent site is lower legs

Diagnosis of Tertiary Syphilis
* Histopathology - tuberculoid granules with multinucleated giant cells
* Nontreponemal tests (VDRL, RPR) positive in 75%
* Treponemal tests (FTA-ABS, MHA-TP, TPI) positive in nearly 100%
* Darkfield negative, PCR may be positive

DDx Tertiary Syphilis
* R/O tumors; SCCA tongue, leukemic infiltrates, sarcoidosis
* Ulcerated syphilids resemble scrofula, atypical mycobacterium, sporotrichosis, blastomycosis
* Mycosis fungoides (CTCL) has eczema and pruritus
* Perforation of hard palate and septum

Late Osseous Syphilis
* Gummatous lesions can involve the periosteum and bone
* Head, face, tibia
* Periostitis, osteomyelitis, osteitis, gummatous osteoarthritis
* “Osteocope” - bone pain often at night
* Charcot joint - loss of contours of joint, hypermobility, painless
* Associated with tabes dorsalis

Neurosyphilis
* CNS involvement with syphilis can occur at any stage
* Most are asymptomatic; CSF shows pleocytosis
* 4-10% of untreated pts will develop neurosyphilis

Early Neurosyphilis
* First year of infection - meningeal
* Headache, stiff neck, cranial nerve disorders, seizures, delirium, increased ICP

Meningovascular Neurosyphilis
* 4-7 years after infection
* Thrombosis of vessels in the CNS
* Hemiplegia, aphasia, hemianopsia, transverse myelitis, progressive muscular atrophy
* CN palsies; CN IIX, III, IV, VI
* “Argyll Robertson Pupil” accommodates, but doesn’t react

Late Neurosyphilis
* Parenchymatous neurosyphilis occurs more than 10 years after infection
* Two classical patterns; Tabes Dorsalis, and General Paresis

Tabes Dorsalis
* Degeneration of the dorsal roots of the spinal nerves and posterior columns of the the spinal cord
* Gastric crisis with severe pain and vomiting is most common
* Pain, urination problems, paresthesias, ataxia, diplopia, vertigo, deafness
* Signs: Argyll Robertson pupil, reduced lower cord reflexes, Romberg sign, sensory loss, atonic bladder, Charcot’s joints, optic atrophy
* Personality changes, memory loss, apathy, megalomania, delusions, dementia

Late Cardiovascular Syphilis
* Occurs in 10% of untreated pts
* Aortitis, aortic insufficiency, coronary disease, aortic aneurysm

Congenital Syphilis
* Prenatal syphilis acquired in utero
* Infection through the placenta usually does not occur before the fourth month, so treatment of the mother before this time will almost always prevent infection in the fetus.
* If infection occurs after the fourth month 40% risk of fetal death

* 40% of pregnancies in women with untreated early syphilis will result in a syphilitic infant.
* Most neonates with congenital syphilis are normal at birth.
* Early congenital syphilis - lesions occurring within first two years of life
* Late congenital syphilis - lesion occur after two years

Early Congenital Syphilis
* Cutaneous manifestations appear most commonly during 3rd week
* Snuffles (a form of Rhinitis) is most frequent, bloody drainage, ulcers may develop, later septal perfs
* 30-60% of infants develop cutaneous lesions similar to secondary syphilis
* Red to copper maculopapular, become large, scaling, pustules, crusting
* Face, arms, buttocks, legs, palms and soles

Early Congenital Syphilis
* Face, perineum, and intertriginous areas, usually fissured lesions resembling mucous patches. Radial scarring results leading to Rhagades
* Bone lesions occur in 70-80% , epiphysitis is common and causes pain on motion, leading to infant refusing to move; Parrot’s pseudoparalysis.
* Radiologic features of the bone lesions in congenital syphilis during the first 6 months are characteristic.
* Bone lesions occur at the epiphyseal ends of long bones.
* Lymphadenopathy, hepatomegaly, nephrotic syndrome, meningitis, nerve palsies may all occur

Late Congenital Syphilis
* Lesions are two types - malformations of tissue affected at critical growth periods (Stigmata) and persistent inflammatory foci
* Inflammatory - lesions of the cornea, bones, and central nervous system, i.e., interstitial keratitis in 20-50%, perisynovitis of knees (Clutton’s joints), tabes dorsalis, seizures, and paresis

Late Congenital Syphilis
* Malformations (Stigmata) - destructive effects leave scars or developmental defects
* Hutchinson’s Triad - Changes in incisors, corneal scars, and eighth nerve deafness
* Also, saber shins, rhagades of the lips, saddle nose, mulberry molars

Hutchinson’s Teeth
* Malformation of the central upper incisors that appears in the second or permanent teeth. Teeth are cylindrical rather than flattened, cutting edge narrower than base, notch may develop
* Mulberry molar - first molar hyperplastic, flat occlusal surface covered with knobs representing abortive cusps

Treatment of Syphilis
* PCN is drug of choice for treatment of all stages of syphilis.
* HIV testing is recommended in all patients
* If less than one year; 2.4M U of Benzathine PCN G
* PCN-allergic; Tetracycline 500mg QID for 14 days

Jarisch- Herxheimer Reaction
* Febrile reaction occurs after the initial dose of antisyphilitic tx, 60-90% of pts
* 6-8 hours after dose - chills, fever, myalgia, increase in inflammation (neurosyphilis)

Treatment of Sex Partners
* Persons exposed to a patient with early syphilis within the previous 3 months should be treated, even if seronegative
* Single dose azithromycin effective in treating incubating syphilis

Serologic Testing after Tx
* VDRL or RPR repeated every 3 months in first year, every 6 months in second year, than annually
* A fourfold decrease in titer should be seen at 6 months, if not then 3 weekly PCN IM injections
* Response for latent syphilis is slower, 12-24 months
* If not responding; HIV and CSF testing repeated
* Pts with late syphilis may be “serofast”, and titers may not improve
* Neurosyphilis pts should have CSF every 6 months

Syphilis and HIV
* Most HIV pts exhibit the classic clinical manifestations and course, and respond similarly to tx
* More likely to present with secondary syphilis and have a persistent chancre

Yaws
* Treponema pallidum subsp. Pertenue
* Endemic in some tropical, rural regions
* Overcrowding, poor hygiene, transmitted by contact with infected lesions
* Children, disabling course, affects skin, bones, and joints

Early Yaws
* Primary papule or group of papules appear at site of inoculation after 3 week incubation period, initial lesion becomes larger and crusted (Mother Yaw, maman pian )
* Feet, legs, buttocks, face, not genitals
* Mother yaw disappears after a few months
* Secondary Yaws – appears weeks or months after mother yaw appears. May be smaller and appear around primary lesion; may be annular (ringworm yaws)
* Condylomata may develop around body orifices and creases
* Palms and soles may form hyperkeratotic plaques leading to a painful crab-like gait (crab yaws)

Late Yaws
* 10% progress to late stage where gummas occur
* Ulcer with clean edges that tend to fuse to form con figurate and serpiginous patterns similar to tertiary syphilis
* Bone, joint, saddle nose, saber shin, Gangosa (destruction of palate and nose)
* Diagnosis = Darkfield, VDRL or RPR

Endemic Syphilis (Bejel)
* Bejel is a Bedouin term for nonvenereal treponematosis, nomadic tribes of North Africa, Southwest Asia, Eastern Mediterranean
* T. pallidum subsp. Endemicum
* Usually occurs in childhood through skin contact
* May affect the skin, oral mucosa, and skeletal system

Bejel
* Primary lesions rare, probably go undetected in the oral mucosa
* Secondary oral lesions - shallow, painless ulcers, laryngitis
* Condyloma of axillae and groin, lymphadenopathy, osteoperiostitis causes night leg pain
* Untreated secondary bejel heals in 6-9 months
* Tertiary stage - gummatous ulcerations of the skin, nasopharynx, and bone.
* Neuro - uveitis, choritis, optic atrophy

Pinta
* T. carateum; nonvenereal, endemic
* Only skin lesions occur
* All ages, Brazilian rain forest
* Primary Stage - 7 to 60 days after inoculation. Lesion begins as a tiny red papules and become an elevated erythematous infiltrated plaque 10cm in diameter over 2-3 months. Legs -satellite lesions, no erosion or ulceration as in chancres.
* Secondary Stage - 5 months to 1 year
* Small, scaling papules that enlarge and coalesce – affects extremities and face
* Red to blue, black with postinflammatory hyperpigmentation
* Nontreponemal tests reactive in 60%
* Late Dyschromic Stage - young adults – may appear as hyperpigmented and depigmented macules resembling vitiligo
* Face, waist, wrist, trochanteric areas
* Histo - acanthosis, lichenoid, spirochetes in epidermis

Treatment of Yaws, Bejel, and Pinta
* Benzathine PCN G 1.2 to 2.4 M units IM
* Tetracycline 500mg QID for adults
* EES 10mg/kg children QID for 14 days
Nonvenereal Treponematoses
* Yaws
* Endemic Syphilis
* Pinta

Treatment
* Syphilis >1year; 2.4M PCN G weekly for 3 weeks Pcn-allergic; Tetra 500mg QID for 30 days
* Neurosyphilis; IV
* Infant 100,000 to 150,000 units/kg/day Procaine PCN BID for first seven days of life

Syphilis.ppt

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