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

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Presentation on theme: "Andrews’ Diseases of the Skin- Chapter 10-pg 239-253 & Chapter 11 Boris Ioffe, D.O."— Presentation transcript:

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

2 Recalcitrant Palmoplantar Eruptions Recalcitrant Palmoplantar Eruptions Recalcitrant pustular eruptions of the hands and feet are often examples of psoriasis 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) 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 Search also for a family history to confirm your suspicion

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

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

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

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

7 Palmoplantar Pustulosis Patches begin as erythematous areas in which pustules form Patches begin as erythematous areas in which pustules form Start as pinhead- sized, enlarge and coalesce to form small lakes of pus 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 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 Stages of quiescence and exacerbation characterize the condition Meds, such as lithium, have been reported to induce Meds, such as lithium, have been reported to induce

8 Palmoplantar Pustulosis Nails may become malformed, ridged, stippled, pitted and discolored Nails may become malformed, ridged, stippled, pitted and discolored May be associated with psoriasis vulgaris May be associated with psoriasis vulgaris Some regard palmoplantar pustulosis as a form of psoriasis, while others consider it a separate entity 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 Female predominance; lack of seasonal variation; different histopathologic features and Associated with thyroid disorders and cigarette smoking 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 May be predisposed to joint disease and possibly SAPHO syndrome-Synovitis, Acne, Pustulosis, Hyperostosis and Osteoarthritis It’s resistant to most treatments It’s resistant to most treatments Acitretin is reportedly effective(1mg/kg/day) Acitretin is reportedly effective(1mg/kg/day) Low-dose cyclosporine (1.25mg/kg/day- 3.75mg/kg/day) Low-dose cyclosporine (1.25mg/kg/day- 3.75mg/kg/day) Intramuscular Kenalog (40-60mg)may be effective for short-term relief 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 Characterized by a symmetric, grouped, vesicular or pustular eruption on palms and soles Marked by exacerbations and remissions over long periods Marked by exacerbations and remissions over long periods No involvement of webs of fingers or toes or flexion creases of toes No involvement of webs of fingers or toes or flexion creases of toes WBC may be elevated WBC may be elevated Scaling is usually present Scaling is usually present Etiology is thought to be a remote focus of infection; infection needs to be treated before resolution will occur 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 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 Toe webs are rarely involved; fingers may be Histologically, there is psoriasiform acanthosis and a sparse, lymphocytic infiltrate in the upper dermis Histologically, there is psoriasiform acanthosis and a sparse, lymphocytic infiltrate in the upper dermis Spongiosis is commonly present Spongiosis is commonly present Tx: bed rest, cotton socks and topical steroids Tx: bed rest, cotton socks and topical steroids Spontaneous resolution within 4 yrs is the rule Spontaneous resolution within 4 yrs is the rule

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15 Infantile Acropustulosis Intensely itchy vesicopustular eruption of hands and feet 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 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 Dapsone at 2mg/kg/day may help Potent topical steroids aid in symptomatic relief Potent topical steroids aid in symptomatic relief

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

17 Acropustulosis of infancy Acropustulosis of infancy

18 Pompholyx AKA dyshidrosis AKA dyshidrosis A vesicular eruption of palms and soles characterized by spongiotic intraepidermal vesicles and often accompanied by burning or itching A vesicular eruption of palms and soles characterized by spongiotic intraepidermal vesicles and often accompanied by burning or itching Hyperhidrosis may be present Hyperhidrosis may be present Usually bilateral and symmetrical Usually bilateral and symmetrical Bullae may form Bullae may form Contents are clear and colorless Contents are clear and colorless Attacks generally last a few weeks Attacks generally last a few weeks Lesions dry-up and desquamate rather than rupture Lesions dry-up and desquamate rather than rupture

19 Pomphylox Etiology- stress, atopy, and topical as well as ingested contactants Etiology- stress, atopy, and topical as well as ingested contactants Histopathology: spongiotic vesicles in the epidermis Histopathology: spongiotic vesicles in the epidermis Differential dx: 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 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 Tx: high potency corticosteroid creams Triamcinolone acetonide intramuscularly or a short course of oral prednisone is rapidly effective Triamcinolone acetonide intramuscularly or a short course of oral prednisone is rapidly effective Oral or topical psoralen + UVA (PUVA) is effective but costly & inconvenient Oral or topical psoralen + UVA (PUVA) is effective but costly & inconvenient In more severe forms, immunosuppressive mycophenolate mofetil has been effective In more severe forms, immunosuppressive mycophenolate mofetil has been effective

21 Lamellar Dyshidrosis AKA dyshidrosis lamellosa, keratolysis exfoliativa AKA dyshidrosis lamellosa, keratolysis exfoliativa A superficial exfoliative dermatosis of the palms and sometimes soles A superficial exfoliative dermatosis of the palms and sometimes soles Referred to as recurrent palmar peeling Referred to as recurrent palmar peeling Involvement is bilateral Involvement is bilateral Can occur in association with dyshidrosis Can occur in association with dyshidrosis Often exacerbated by environmental factors Often exacerbated by environmental factors Differential dx: dermatophytosis, chronic contact dermatitis Differential dx: dermatophytosis, chronic contact dermatitis

22 Lamellar Dyshidrosis Tx: difficult Tx: difficult Spontaneous involution can occur in a few weeks for some Spontaneous involution can occur in a few weeks for some Most tends to be chronic and relapsing Most tends to be chronic and relapsing Tar creams (Zetone cream) usually helps Tar creams (Zetone cream) usually helps 5% tar in gel (Estar Gel) is an excellent tx 5% tar in gel (Estar Gel) is an excellent tx Lac-Hydrin lotion and Carmol 10 or 20 are often effective Lac-Hydrin lotion and Carmol 10 or 20 are often effective NB-UVB may be helpful NB-UVB may be helpful

23 Lamellar Dyshidrosis

24 Palmoplantar Keratoderma AKA tylosis, keratosis, hyperkeratosis AKA tylosis, keratosis, hyperkeratosis Characterized by excessive formation of keratin on the palms and soles Characterized by excessive formation of keratin on the palms and soles Acquired Acquired –Keratosis Punctata of the Palmar Creases –Punctate Keratoses of the Palms and Soles –Porokeratosis Plantaris Discreta –Keratoderma Climactericum Congenital 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 Primary lesion is a 1-5mm round to oval, dome-shaped papule distributed over left hand and hypothenar eminence Main symptom is pruritis Main symptom is pruritis Lesions number from 1 to >40 Lesions number from 1 to >40 Affects mainly blacks Affects mainly blacks There’s a potential risk of developing lung and colon cancer 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 Common most often in black pts Primary lesion is a 1-5mm depression filled with a conical keratinous plug Primary lesion is a 1-5mm depression filled with a conical keratinous plug Primarily, in creases of palms or fingers, occasionally in soles Primarily, in creases of palms or fingers, occasionally in soles Lesions are multiple Lesions are multiple Friction aggravates lesions causing them to become verrucoid or surrounded by callus Friction aggravates lesions causing them to become verrucoid or surrounded by callus

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

29 Porokeratosis Plantaris Discreta Occurs in adults, Female:Male (4:1) Occurs in adults, Female:Male (4:1) Characterized by sharply marginated, rubbery, wide-based papule that does not bleed on removal Characterized by sharply marginated, rubbery, wide-based papule that does not bleed on removal Lesions are multiple, painful, 7-10mm in diameter Lesions are multiple, painful, 7-10mm in diameter Usually on wt bearing areas of sole, beneath metatarsal heads Usually on wt bearing areas of sole, beneath metatarsal heads Tx: foot pads to redistribute wt, surgical excision, blunt dissection 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 Characterized by hyperkeratosis of palms and soles beginning at about the time of menopause Descrete, thickened, hyperkeratotic patches most pronounced at pressure sites Descrete, thickened, hyperkeratotic patches most pronounced at pressure sites Fissuring may be present Fissuring may be present Tx: keratolytics -- 10% salicylic acid, lactic acid creams, etc. Tx: keratolytics -- 10% salicylic acid, lactic acid creams, etc.

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

32 Dominant inheritance; congenital thickening of epidermal horny layer of the palms and soles Dominant inheritance; congenital thickening of epidermal horny layer of the palms and soles Usually symmetrical Usually symmetrical Epidermis becomes thick, yellowish, verrucous, and horny Epidermis becomes thick, yellowish, verrucous, and horny Striate and punctate forms occur Striate and punctate forms occur Unna Thost

33 Occasionally nails become thickened Occasionally nails become thickened 5% salicylic acid may help 5% salicylic acid may help Lac Hydrin 12% may be tried Lac Hydrin 12% may be tried Acitretrin or isotretinoin may be considered, but need for lifetime tx makes them impractical 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 Focal palmoplantar keratosis of the striate type on the sole

36 Diffuse non- epidermolytic palmoplantar keratosis Diffuse non- epidermolytic palmoplantar keratosis

37 Diffuse epidermolytic palmoplantar keratosis with diffuse hyperkeratosis Diffuse epidermolytic palmoplantar keratosis with diffuse hyperkeratosis

38 Papillon-Lefevre Syndrome Palmoplantar hyperkeratosis with peridontosis Palmoplantar hyperkeratosis with peridontosis Usually develops within the first few months of life but may occur in childhood Usually develops within the first few months of life but may occur in childhood Well demarcated, erythematous, hyperkeratotic lesions on palms and soles Well demarcated, erythematous, hyperkeratotic lesions on palms and soles Transverse grooves of fingernails may occur Transverse grooves of fingernails may occur

39 Early onset peridontal disease has been attributed to damage and alteration in PMN function caused by Actinomyces actinomycetemcomitans 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 Disease associations include: acroosteolysis, and pyogenic liver abcesses There are asymptomatic ectopic calcifications in the choroid plexus and tentorium There are asymptomatic ectopic calcifications in the choroid plexus and tentorium Therapy may retard both dental and skin abnormalities Therapy may retard both dental and skin abnormalities Treatment with Acitretin in four siblings was reported to be effective Treatment with Acitretin in four siblings was reported to be effective Papillon-Lefevre Syndrome

40

41 Papillon-Lefevre syndrome: plantar keratoderma 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 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 than 30 cases have been reported world-wide More common in women and in whites More common in women and in whites Onset is in infancy or early childhood Onset is in infancy or early childhood

43 Vohwinkel’s mutilating syndrome: A.) diffuse keratoderma of palms with B.) pseudoaainhum formation 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 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 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 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 Focal PPK in association with carcinoma of the esophagus

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

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

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

51 Etiology Most common is preexisting dermatoses: (53%); 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%); Drug eruptions(5%); –allopurinol, gold, carbamazepine, phenytoin, and quinidine Cutaneous T-cell lymphoma(13%); Sezary syndrome and mycosis fungoides Cutaneous T-cell lymphoma(13%); Sezary syndrome and mycosis fungoides Paraneoplstic (2%); carcinoma of the lung and carcinoma of the stomach Paraneoplstic (2%); carcinoma of the lung and carcinoma of the stomach Leukemia cutis (1%) Leukemia cutis (1%) Idiopathic (26%) Idiopathic (26%) Mortality rate at a mean follow-up interval of 51 months was 43% Mortality rate at a mean follow-up interval of 51 months was 43%

52 Histology Most commonly, histology is nonspecific Most commonly, histology is nonspecific Hyperkeratosis & focal parakeratosis Hyperkeratosis & focal parakeratosis Epidermis shows mild acanthosis, scant superficial upper dermal infiltrate of mononuclear cells Epidermis shows mild acanthosis, scant superficial upper dermal infiltrate of mononuclear cells May be small areas of spongiosis May be small areas of spongiosis

53 Generali zation after withdra wal of methotr exate Generali zation after withdra wal of methotr exate

54 Exfoliation of scale with underlying erythema Exfoliation of scale with underlying erythema

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

56 Treatment Topical steroids, soaks, and compresses Topical steroids, soaks, and compresses Acitretin and cyclosporin-useful in psoriatic erythroderma, and isotretinoin in erythroderma caused by RPR; methotrexate Acitretin and cyclosporin-useful in psoriatic erythroderma, and isotretinoin in erythroderma caused by RPR; methotrexate Systemic corticosteroids in severe cases Systemic corticosteroids in severe cases Discontinuing the offending drug in drug- induced cases Discontinuing the offending drug in drug- induced cases

57 * Subungual hyperkeratosi s and distal dystrophy

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59 Parapsoriasis, Pityriasis Rosea, Pityriasis Rubra Pilaris

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

61 Pityriasis Lichenoides Chronica Erythematous, yellowish, scaly macules and lichenoid papules Erythematous, yellowish, scaly macules and lichenoid papules They persist indefinitely without change They persist indefinitely without change Mainly on sides of trunk, thighs, and upper arms Mainly on sides of trunk, thighs, and upper arms May be confused with psoriasis and secondary syphilis May be confused with psoriasis and secondary syphilis Tx- UV light is beneficial; however intense doses may be needed for good results Tx- UV light is beneficial; however intense doses may be needed for good results PUVA has been reported to be effective PUVA has been reported to be effective Oral tetracycline may be used with antihistamines Oral tetracycline may be used with antihistamines PLC is a benign disease that clears spontaneously in a few yrs to months PLC is a benign disease that clears spontaneously in a few yrs to months

62 Pityriasis Lichenoides Chronica

63 PLEVA AKA: parasoriasis lichenoides, Habermann’s disease, Mucha-Habermann disease and parapsoriasis varioliformis acuta 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 Sudden appearance of a polymorphous eruption composed of macules, papules, and occasional vesicles May run an acute, subacute, or chronic course 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 Papules are usually yellowish or brownish- red, round lesions, which tend to crust, become necrotic and hemorrhage

64 PLEVA

65 When exanthem heals it leaves a smooth, pigmented, depressed, varioliform scar When exanthem heals it leaves a smooth, pigmented, depressed, varioliform scar Favorite sites are anterior trunk, flexural arms, and axillae Favorite sites are anterior trunk, flexural arms, and axillae Palms and soles are involved infrequently- mucous membranes are not Palms and soles are involved infrequently- mucous membranes are not Generalized lymphadenopathy can occur 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 Maybe a spectrum of cutaneous T-cell lymophoma Differential dx: Differential dx: –leukocytoclastic angiitis, papulonecrotic tuberculid, psoriasis, lichen planus, varicella, PR, drug eruptions, maculopapular syphilid, viral, rickettsial diseases, lymphomatoid papulosis PLEVA

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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 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 Absence of neutrophils simplifies the distinction between leukocytoclastic angiitis Lymphomatoid papulosis differs by the presence of large, atypical mononuclear cells in the dermal infiltrate Lymphomatoid papulosis differs by the presence of large, atypical mononuclear cells in the dermal infiltrate

68 PLEVA-Tx No one tx is reliably effective No one tx is reliably effective Tetracycline and erythromycin are worth trying Tetracycline and erythromycin are worth trying UVB and PUVA UVB and PUVA Methotrexate, mg every 12 hrs for 3 doses 1 day each week 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 Several serious reactions a few of them fatal have occurred with simultaneous administration of methotrexate and NSAIDs Dapsone and pentoxifylline(Trental), 400mg twice daily 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 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 Has patches 5-15 cm; otherwise is similar to small-plaque type Prognosis is benign, especially if pruritis is severe Prognosis is benign, especially if pruritis is severe 10% may eventuate in T-cell lymphoma 10% may eventuate in T-cell lymphoma

71 Large plaques parasporiasis: large, variably erythematous and mildly poikilodermatous patches in the bathing trunk region 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 Small plaque parasporiasis: small(<5), erythematous, slightly scaly patches

73 Treatment First line: UV radiation -- either natural or UVB First line: UV radiation -- either natural or UVB Lubricants and Topical steroids Lubricants and Topical steroids PUVA but only if UVB fails PUVA but only if UVB fails Use of PUVA or high-potency topical streroids should be limited due to long-term adverse effects 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 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 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 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 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 Color is white (but never actually depigmented) or light pink Scales are fine and adherent Scales are fine and adherent Patches are usually sharply demarcated; edges may be erythematous and slightly elevated 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 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 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 Usually asymptomatic; however there may be mild pruritis Disease mainly occurs in children and teenagers Disease mainly occurs in children and teenagers It is particularly a cosmetic problem in dark- skinned individuals It is particularly a cosmetic problem in dark- skinned individuals

77 Pityriasis Alba Etiology unknown Etiology unknown Excessively dry skin appears to be contributory Excessively dry skin appears to be contributory Most lesions disappear with time Most lesions disappear with time Repigmentation can be accelerated with treatment Repigmentation can be accelerated with treatment Emollients and bland lubricants Emollients and bland lubricants Low-strength corticosteroids plus Lac-Hydrin are helpful Low-strength corticosteroids plus Lac-Hydrin are helpful Others have recommended PUVA Others have recommended PUVA

78 Pityriasis Rosea Mild inflammatory exanthem of unknown origin ?viral Mild inflammatory exanthem of unknown origin ?viral Characterized by salmon-colored papules and patches which are oval and covered with a collarette of scale 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 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 Appears rapidly and last from 3-8 weeks Peak: ages Peak: ages Typically in Spring and Autumn Typically in Spring and Autumn More common in women More common in women

80 Pityriasis Rosea Mainly affects the trunk Mainly affects the trunk Oral lesions are relatively uncommon, but present as aphthous lesions 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 Papular PR is an unusual form common in black chidren under age 5 Inverse PR is unusual, but not rare Inverse PR is unusual, but not rare Relapses and recurrences are frequently observed 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 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 Inverse pityriasis rosea: oval annular plaques in groin

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

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

86 PR: papules and annular plaques PR: papules and annular plaques

87 PR: oval and round plaques, some with central scale and others with a collarette of scale 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 hyperpigmentatio n PR in darkly pigmented skin: it tends to be more papular than in lightly pigmented skin-note associated hyperpigmentatio n

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

90 PRP Involvement is usually symmetrical and diffuse, with islands of normal Involvement is usually symmetrical and diffuse, with islands of normal Hyperkeratosis of palms and soles called, the “sandal” Hyperkeratosis of palms and soles called, the “sandal” Nails may be dull, rough, thick, and brittle Nails may be dull, rough, thick, and brittle Itching in some cases Itching in some cases Koebner’s phenomenon may be present 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 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 PRP may classified into familal or acquired types in respect to the onset of the disease in childhood or adulthood 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 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 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 Etiology unknown- ??AD Either sex affected Either sex affected Possible related to deficiency of Possible related to deficiency of vitamin A vitamin A Histology: hyperkeratosis, follicular plugging, and focal parakeratosis at follicular orifice Histology: hyperkeratosis, follicular plugging, and focal parakeratosis at follicular orifice Inflammatory infiltrate in dermis is composed of mononuclear cells Inflammatory infiltrate in dermis is composed of mononuclear cells

93 PRP: psoriasiform dermatitis with follicular plugging PRP: psoriasiform dermatitis with follicular plugging

94 Treatment Symptomatic: emollients-- Lac-Hydrin Symptomatic: emollients-- Lac-Hydrin A several-month course of isotretinoin in doses of 0.5 – 2 mg/kg/day 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 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 Methotrexate 2.5mg alternating with 5mg daily Monitor and treat secondary infections Monitor and treat secondary infections

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97 Pityriasis rubra pilaris: diffuse erythroderma with desquamation and follicular hyperkeratosis 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 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 Pityriasis rubra pilaris: orange-red waxy keratoderma of the palms


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