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

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1 Andrews’ Diseases of the Skin-Chapter 10-pg 239-253 & Chapter 11
Boris Ioffe, D.O.

2 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

3 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

4 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

5 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

6 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

7 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

8 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

9 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

10 Palmoplantar Pustulosis

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12 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

13 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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15 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

16 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

17 Acropustulosis of infancy

18 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

19 Pomphylox Etiology- stress, atopy, and topical as well as ingested contactants Histopathology: spongiotic vesicles in the epidermis Differential dx: 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

20 Pomphylox 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

21 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

22 Lamellar Dyshidrosis 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

23 Lamellar Dyshidrosis 10-38.jpg

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

25 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

26 Punctate Keratosis of the Palms and Soles

27 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

28 Punctate keratoses of the palmar creases in an African-American
PPPK-punctate palmoplantar keratoderma

29 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

30 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.

31 Hereditary syndromes These have palmoplantar keratoderma as a feature
Unna-Thost Papillon-Leferve

32 Unna Thost 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

33 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

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35 Focal palmoplantar keratosis of the striate type on the sole

36 Diffuse non-epidermolytic palmoplantar keratosis

37 Diffuse epidermolytic palmoplantar keratosis with diffuse hyperkeratosis

38 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

39 Papillon-Lefevre Syndrome
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

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41 Papillon-Lefevre syndrome: plantar keratoderma

42 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

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

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45 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

46 Focal PPK in association with carcinoma of the esophagus

47 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

48 Focal acrokeratoelastoides: multiple skin-colored papules at the margin of the palmar skin

49 Path: non-epidermolytic palmoplantar keratosis, acanthosis and hypergranulosis

50 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

51 Etiology Most common is preexisting dermatoses: (53%); atopic dermatitis, chronic actinic dermatitis, psoriasis,seborrheic dermatitis, vesicular palmoplantar eczema, pityriasis rubra pilaris, and contact dermatitis Drug eruptions(5%); 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%

52 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

53 Generalization after withdrawal of methotrexate

54 Exfoliation of scale with underlying erythema

55 Generalized erythema with thick scale and crusted fissures on the plantar surface

56 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

57 * Subungual hyperkeratosis and distal dystrophy

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59 Parapsoriasis, Pityriasis Rosea, Pityriasis Rubra Pilaris

60 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

61 Pityriasis Lichenoides Chronica
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 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

62 Pityriasis Lichenoides Chronica

63 PLEVA 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 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

64 PLEVA 11-6.jpg

65 PLEVA * Usually a benign, self-limited disorder, but may be more chronic and severe Maybe a spectrum of cutaneous T-cell lymophoma Differential dx: leukocytoclastic angiitis, papulonecrotic tuberculid, psoriasis, lichen planus, varicella, PR, drug eruptions, maculopapular syphilid, viral, rickettsial diseases, lymphomatoid papulosis 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

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67 PLEVA 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

68 PLEVA-Tx No one tx is reliably effective
Tetracycline and erythromycin are worth trying UVB and PUVA Methotrexate, mg 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

69 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 * Most lesions occur on the trunk and are between 1 –5cm * Patches may persist for years to decades and do not progress to lymphoma

70 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

71 Large plaques parasporiasis: large, variably erythematous and mildly poikilodermatous patches in the bathing trunk region

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

73 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

74 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

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76 Pityriasis Alba 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

77 Pityriasis Alba 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

78 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

79 PR Appears rapidly and last from 3-8 weeks Peak: ages 15-40
Typically in Spring and Autumn More common in women

80 Pityriasis Rosea Mainly affects the trunk
Oral lesions are relatively uncommon, but present as aphthous lesions

81 Herald Patch

82 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

83 Inverse pityriasis rosea: oval annular plaques in groin

84 Treatment Supportive UVB should be used after acute inflammatory stage has passed Topical corticosteroids Antihistamines Emollients

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

86 PR: papules and annular plaques

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

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

89 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

90 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

91 PRP 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

92 PRP 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

93 PRP: psoriasiform dermatitis with follicular plugging

94 Treatment Methotrexate 2.5mg alternating with 5mg daily
Monitor and treat secondary infections 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

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97 Pityriasis rubra pilaris: diffuse erythroderma with desquamation and follicular hyperkeratosis

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

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

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