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

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 • 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. 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 Palmoplantar Pustulosis

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

10. Palmoplantar Pustulosis

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

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. 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 Lamellar Dyshidrosis

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

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

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. 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

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.) diffuse keratoderma of palms with B.) pseudoaainhum formation

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