Other papulosquamous disorders

Pityriasis Rosea/
Pityriasis Rubra Pilaris (PRP) /
Parapsoriasis /
Pityriasis Lichenoides (Acute and chronic)

1. Other Papulosquamous Disorders

2. Psoriasis Pityriasis rosea Papulosquamous disorders Lichen Planus Lichen nitidus Seborrheic dermatitis Pityriasis rubra pilaris Exfoliative dermatitis Lichen striatus Parapsoriasis, small / large plaque Pityriasis lichenoides

3. Other important papulosquamous diseases Mycosis fungoides (cutaneous T-cell lymphoma) Discoid lupus erythematosus Subacute cutaneous lupus erythematosus Tinea corporis Nummular eczema Secondary syphilis Drug eruptions Erythema dyschromicum perstans Keratosis lichenoides chronica Lichen sclerosus Lichenoid dermatitis Lichenoid reaction of graft-versus-host disease Extramammary Paget’s disease

4. Today’s Topics Pityriasis Rosea Pityriasis Rubra Pilaris (PRP) Parapsoriasis Pityriasis Lichenoides (Acute and chronic)

5. Pityriasis Rosea

6. Pityriasis Rosea (PR) Definition Epidemiology Etiology Clinical Picture Differential Diagnoses Histological features Treatment

7. Definition IT is common, self limited acute papulosquamous disorder often with distinctive and constant course lasting from 4-10 weeks. Pityriasis : Any of several skin diseases marked by the formation and desquamation of fine scales. Rosea : rose colored or pink. Benign Self limiting but associated with increased miscarriage in first 15 wks. of pregnancy.

8. Epidemiology Worldwide distribution. AGE : Predominantly occur in adolescents and young adults 10-35 years but can occur rarely in infants and old persons. SEX : F>M. SEASON: more in spring & autumn . Most cases of pityriasis rosea (PR) are sporadic .

9. Etiology Exact cause unknown but may be due to; INFECTIONS : HHV-6 , HHV-7 infections but viral DNA couldn’t be detected from the lesion. Not contagious & gives life long immunity after outbreak. DRUGS : A rsenic, A dalimumab, B arbiturates, B ismuth, C aptopril, C lonidine , D -penicillamine, E tanercept, G old, I sotretinoin, K etotifen , L ithium, M etronidazole , N ortriptyline , O meprazole, T erbinafine.

10. Clinical Picture

11. Clinical Picture I. PRODROME May or may not be present only 5 % of patients. Fever Malaise Headache Mild constitutional symptoms Respiratory infection

12. Clinical Picture II. “HERALD PATCH” OR MOTHER PATCH Seen in 50-80 % of cases. Preceding exanthem. Raised plaque or patch. Large (2 – 10 cm). Usually single but may be multiple. Usually oval. Pink or salmon pink. Collarette scales points inwards just inside the well-demarcated border. Central clearing occasionally & slightly raised advancing margin (mimic tinea). Usually on the trunk.

13. Herald Patch

14. Herald Patch

15. Clinical Picture III. SECONDARY ERUPTION (EXANTHEM) Appear within 1-2 weeks. May be asymptomatic or pruritic 25-75 % . Mainly of 2 types: Small rounded papules with or w/o fine scaling that ↑ in number & spread peripherally. Similar lesions to herald patch but smaller (1-2 cm): Appear as discrete bilateral symmetrical oval salmon pink in color on the trunk and proximal aspects of the extremities with peripheral collarette scales. Run along lines of cleavage (Langer’s lines) /parallel to the ribs giving f i r tree or Christmas tree pattern.

16. Secondary eruption

17. Secondary eruption

18. Langer’s Lines

19. Christmas tree pattern

20. Christmas tree pattern

21. Clinical Picture IV. FADING OF THE LESIONS: Spontaneous remission occurs within 4 to 8 weeks. Occasionally lasts for 5 months or more. Postinflammatory pigment changes can be observed. Both hypopigmentation and hyperpigmentation can follow the rash. Recurrences are uncommon .

22. Mnemonic

23. Atypical Variants of Pityriasis Rosea 20 % of patients. Atypical morphology, distribution, or both.

24. Atypical Variants of Pityriasis Rosea

25. Atypical Variants of Pityriasis Rosea

26. Atypical Variants of PR

27. In dark individuals

28. Differential Diagnoses Guttate psoriasis Small plaque para psoriasis PL EVA PL C (especially if persistent ) P RP P . alba Tinea corporis Tinea Versicolor Seborrhoeic dermatitis Discoid dermatitis Viral Exanthems Drug eruptions Secondary syphilis Erythema multiforme

29. Histological features

30. Histological features Patchy epidermal spongiosis & lymphocytes Lymphocytes surrounding dermal vessels Extravasated red blood cells Patchy hyperkeratosis & parakeratosis

31. Histological features

32. Histological features

33. Histological features Focal (Patchy) or diffuse parakeratosis . Absence of granular layer. Mild acanthosis . Focal spongiosis . Occasional dyskeratotic cells with an eosinophilic homogeneous appearance. Exocytosis . Perivascular dermal infiltration with lymphocytes. Edema of dermis. Often some extravasated red blood cells. Increase in eosinophils in drug induced PR.

34. Treatment A ssurance: Since it is self limited no treatment is required. A ntipruritic lotions . A ntihistamines: Symptomatic relief from itching. Topical steroids : Low-mid strength. Systemic steroids : Short course. Erythromycin: 1 gm q.i.d for 2 weeks. Phototherapy : NB-UVB light may hasten the disappearance or natural sunlight if treatment is begun in the first week of eruption. A cyclovir : Standard dose  not effective. High dose 800 mg five times/d for 1 week may give rapid clearance of the lesions.

35. Pityriasis Rubra Pilaris

36. Pityriasis Rubra Pilaris ( PRP) Definition Epidemiology Etiology Clinical Picture Classification Differential Diagnoses Histological features Treatment

37. Definition A rare chronic papulosquamous skin disease characterized by the appearance of keratotic follicular papules, salmon-colored erythematous plaques interspersed with distinct islands of uninvolved skin, and palmoplantar keratoderma.

38. Epidemiology Rare . M=F . Nearly all cases are acquired sporadic , with occasional reports of a familial inherited form. Familial cases show autosomal dominant inheritance with incomplete penetrance and variable expression. Familial PRP usually presents at birth or appears during the first years of life and runs a chronic course .

39. Etiology The actual cause of PRP is unknown. May be due to: Dysfunction in keratinization or vitamin A metabolism. An autoimmune pathogenesis An abnormal immunologic response to particular antigens . Physical trigger .

40. Clinical Picture CRITERIA FOR SPOT DIAGNOSIS: Coalescence scaling orange–red ) salmon color) plaques with sharp borders covered with fine powdery scales & islands of sparing ( nappes   claires ) in-between . Follicular papules with an erythematous base especially on the dorsal aspect of the proximal fingers (nutmeg grater appearance). An orange–red waxy keratoderma of the palms and soles in most patients.

41. Clinical Picture The plaques may progress to an erythroderma with varying degrees of exfoliation. Erythema with a fine diffuse scale is often seen on the scalp . Nail changes include Distal yellow-brown discoloration Subungual hyperkeratosis Longitudinal ridging The mucous membranes are rarely involved , but they may show features similar to oral lichen planus . Pruritus and burning in 20 % of cases. Mild ectropion may develop when the face becomes uniformly erythematous. Both photoaggravated and phototriggered forms of PRP can also occur. Nail plate thickening Splinter hemorrhages Nail dystrophy and shedding may occur.

42. Classification According to Griffiths classification there are 6 types of PRP. According to age group and typicality of clinical presentation.

43. Type I/Classic adult PRP

44. Type I/Classic adult PRP

45. Type I/Classic adult PRP

46. Type I/Classic adult PRP

47. Type I/Classic adult PRP

48. Type I/Classic adult PRP

49. Type I/Classic adult PRP

50. Type I/Classic adult PRP

51. Type II/Atypical adult PRP

52. Type III/Classic juvenile PRP

53. Type III/Classic juvenile PRP

54. Type I V/Circumscribed juvenile PRP

55. Type V /Atypical juvenile PRP

56. Clinical type % of patients Age at onset Distribution Skin findings Course I (classical adult ) 50 Peak in the 6th decade Generalized , beginning on the head & neck then spreading caudally •Red-orange plaques, confluent with islands of sparing •Perifollicular papules with keratotic plugs •Diffuse, waxy PPK Often resolves within 3 years II (atypical adult ) 5 Adults of various ages Generalized •Areas of eczematous dermatitis • Ichthyosiform scale on the lower extremities •PPK with lamellated scale •Alopecia Chronic intractable III (classic juvenile ) 10 Peaks in the first few years of life and the late teens Generalized •Similar to type I (see above) •<50% have palmoplantar involvement Often resolves within 3 years IV (circumscribed juvenile ) 25 Prepubertal Focal, favoring the elbows and knees •Well-circumscribed, scaly, erythematous plaques •Perifollicular papules with keratotic plugs Variable Some cases clear in the late teens V (atypical juvenile ) 5 First few years of life Generalized • Ichthyosiform dermatitis •Perifollicular papules with keratotic plugs •Scleroderma-like appearance of hands & feet •Accounts for most familial cases of PRP Chronic intractable VI (HIV-associated ) NA Variable Generalized •Similar to type I (see above) •Associated with acne conglobata , hidradinitis suppurativa, and lichen spinulosus Refractory May respond to HAART

57. Differential Diagnoses Psoriasis. Erythroderma. Seborrheic dermatitis ( Early cases). Dermatomyositis. Cutaneous T-cell lymphoma. Erythrokeratodermia variabilis Kawasaki disease (Children with acute-onset PRP).

58. Histological features

59. Histological features

60. Histological features

61. Histological features

62. Histological features

63. Histological features

64. Histological features PSORIASIFORM DERMATITIS: Biopsy from non-follicular lesion consists of distinctive orthokeratosis and parakeratosis alternating in both vertical and horizontal directions ( checkerboard pattern ). The hair follicles are dilated and filled with a keratinous plug , while the “ shoulder effect ” of stratum corneum surrounding the follicular opening frequently shows parakeratosis . Focal or confluent hypergranulosis . Thick suprapapillary plate . Occasionally , mild spongiosis. Scattered intraepidermal lymphocytes. Acantholysis and focal acantholytic dyskeratosis , may be present. Broad slightly elongated epidermal rete ridges , narrow dermal papillae . Perivascular lymphohistiocytic infiltrate in the superficial dermis . Small numbers of plasma cells and eosinophils may be present.

65. Treatment Empiric as the actual cause of PRP is unknown & in many patients there is spontaneous resolution . The goals of treatment are to reduce morbidity and to prevent complications. Consider combination treatment.

66. FIRST LINE SECOND LINE Topical Emollients reduce fissuring and dryness . Keratolytics (salicylic acid, urea). Vitamin D3 ( calcipotriol ). Glucocorticoids (medium to high potency). Topical retinoids ( tazarotene ). Calcineurin inhibitors . Photo(chemo)therapy (May respond well or may flare) Narrowband UVB. Extracorporeal photopheresis . Topical or Systemic PUVA. Ultraviolet A1. Broadband UVB. Systemic Oral retinoids: Currently they are the first line of therapy. Isotretinoin (1 to 1.5 mg/kg/day for 3–6 months), although acitretin (0.5 to 0.75 mg/kg per day) may be more effective in clearing lesions. Methotrexate (10 to 25 mg weekly, IM or orally, once a week) has shown variable rates of success may be combination with a systemic retinoid. Triple antiretroviral therapy (for HIV-associated variant). Oral vitamin A: sometimes in combination with vitamins B and D. Azathioprine (100 to 150 mg/day) but its effect is also inconsistent. Cyclosporine ( 5 mg/kg/day )  Several cases of adult-type PRP showed significant clearance in 2-4 wks. Fumaric acid esters. Biological agents: TNF- α inhibitors and Ustekinumab.

67. Treatment Erythroderma with islands of sparing on the chest and abdomen and yellow palmar keratoderma with fissuring Erythema and scaling subsided following 3 infusions of infliximab.

68. Parapsoriasis

69. Parapsoriasis Definition Classification Epidemiology Etiology Clinical Picture Differential Diagnoses Histological features Treatment Prognosis

70. Definition Group of idiopathic chronic cutaneous diseases that can be characterized by scaly patches or slightly elevated papules and/or plaques that have a resemblance to psoriasis.

71. Classification Small plaque parapsoriasis Large plaque parapsoriasis

72. Epidemiology Presentation most frequently is in middle age ; peak incidence is in the fifth decade of life. Small plaque parapsoriasis the male-to-female ratio is 3:1 .

73. Etiology Exact cause is unknown . T-cell–predominantly CD4 + infiltrates in the skin . Small plaque parapsoriasis shows multiple dominant clones . Large plaque parapsoriasis is a may be due to long-term antigen stimulation & it is associated with a dominant T-cell clone , one that may represent up to 50 % of the T-cell infiltrate. Human herpesvirus type 8 may be detected in skin lesions of large plaque parapsoriasis and the significance is unclear .

74. Clinical Picture Onset of parapsoriasis is indolent . Asymptomatic or mildly pruritic . Composed of patches rather than plaques. Typically persistent and can slowly progress to become more extensive . Favor more sun-protected sites .

75. Clinical Picture SMALL PLAQUE PARAPSORIASIS Can last months to several years ; the disease often resolves spontaneously . Lesions are well-circumscribed , round–oval slightly scaly , light salmon-colored patches that measure <5 cm in diameter and are scattered over the trunk and extremities .

76. Clinical Picture SMALL PLAQUE PARAPSORIASIS Digitate pattern “ Digitate Dermatosis ” is a distinctive form of small plaque disease that consists of palisading elongated fingerlike patches that follow the dermatome and are most prominently displayed symmetrically on the lateral flank and abdomen . They may measure 10 cm or more along their long axis .

77. Digitate dermatosis

78. Clinical Picture LARGE PLAQUE PARAPSORIASIS It is chronic and progresses over many years, sometimes decades . It may progress to CTCL . Manifests as faint erythematous patches with arcuate geographic borders. Each lesion often is >5 cm in diameter. Lesions are scattered on the proximal extremities and the trunk and often show a bathing-suit distribution . Surfaces of the lesions have a faint red-to-salmon color; show flaky thin scales ; and have an atrophic , cigarette-paper or tissue-paper, wrinkling quality or may show poikiloderma or retiform (all retiform cases progress to overt MF).

79. Large plaque parapsoriasis

80. Retiform parapsoriasis

81. Differential Diagnoses SMALL PLAQUE PARAPSORIASIS Pityriasis rosea Drug eruption , esp. PR-like Guttate psoriasis PLC MF 2 ry syphilis Nummular dermatitis LARGE PLAQUE PARAPSORIASIS MF Drug eruption , esp. MF-like Psoriasis Causes of Poikiloderma Poikilodermatous autoimmune connective tissue disease ( e .g . dermatomyositis ) Poikilodermatous genodermatoses Chronic radio dermatitis

82. Laboratory studies Complete blood cell count with differential should be performed, and a high lymphocyte count or the presence of Sézary cells suggests mycosis fungoides.

83. Histological features SMALL PLAQUE PARAPSORIASIS Exhibit a mild, nonspecific spongiotic dermatitis , focal hyperkeratosis, parakeratosis mild superficial perivascular lymphocytic infiltrate , scale crust, and occasional exocytosis . Lymphocytes are small and do not show atypical features. do not show histologic atypia to suggest malignant transformation. LARGE PLAQUE PARAPSORIASIS May have similar histologic findings or an interface lymphocytic infiltrate with a variable degree of lichenoid features. Blood vessels are dilated, and melanophages can be present. The epidermis shows flattening of the rete ridges when epidermal atrophy is prominent on clinical examination. Acanthosis of the epidermis and irregular hyperkeratosis of the cornified layer are present. In contrast to small plaque parapsoriasis, spongiosis is absent .

84. Histological features

85. Histological features

86. Histological features

87. Treatment FIRST LINE Assurance (small plaque parapsoriasis) Emollients Topical corticosteroids ( mid- to high-potency) Topical coal tar products Phototherapy: Sunlight, Broadband or Narrowband ultraviolet B Antihistamines ( If there is pruritus) SECOND LINE (Mainly for large-plaque parapsoriasis cases considered to be early MF & must be treated) Topical bexarotene Topical imiquimod PUVA Topical mechlorethamine ( nitrogen mustard ) Topical carmustine (BCNU) Subcutaneous interferon- α

88. Prognosis SMALL PLAQUE PARAPSORIASIS Is a stable benign disorder that rarely if ever progresses. It lasts several months to years and can spontaneously resolve . LARGE PLAQUE PARAPSORIASIS Is chronic & more ominous in that approximately 10-35 % of patients progress to CTCL . It does not enter remission without treatment & requires closer follow-up . Induration or epidermal atrophy should prompt a repeat skin biopsy to consider a diagnosis of MF in evolution. The 5-year survival rate, however, still remains high and is greater than 90%.

89. Pityriasis Lichenoides

90. Pityriasis Lichenoides Definition Types Epidemiology Etiology Clinical Picture Differential Diagnoses Histological features Treatment

91. Definition They are papular clonal T-cell disorders characterized by recurrent crops of spontaneously regressing erythematous papules.

92. Types Pityriasis lichenoides et varioliformis acuta (PLEVA/ Mucha – Habermann disease ) Pityriasis lichenoides chronica (PLC )

93. Epidemiology Both are more prevalent in the pediatric population, but it affects patients in all age groups, races and geographic regions. There is a male predominance.

94. Etiology The exact etiology is unknown . May be response to foreign antigens such as infectious agents or drugs . The infiltrate is predominantly T cells (CD8 + ) that are often monoclonal thus they are lymphoproliferative disorders .

95. Clinical Picture The acute and chronic forms of pityriasis lichenoides exist on a disease spectrum with variable presentations so many patients have intermediate or mixed manifestations , either serially or concurrently . Can resolve spontaneously after weeks to months or it may pursue a chronic relapsing course .

96. Clinical Picture PLEVA PLC Lesions Vesicular , ulcerative , crusted or pustular . Scaly erythematous to red–brown papules . Course Rapid . More indolent . Resolution Within weeks may  varioliform scars if dermal damage is extensive. Over weeks to months  hypopigmented macules (more obvious in darkly pigmented individuals and may be the presenting C/O ). Extracut . manifestation Malaise , fever , lymphadenopathy , arthritis and/or bacteremia . “ Febrile ulceronecrotic Mucha– Habermann disease (FUMHD)”  severe variants with mucosal, gastrointestinal and pulmonary involvement. Absent .

97. PLEVA

98. PLEVA

99. PLEVA

100. PLEVA

101. PLC

102. PLC

103. PLC

104. PLC

105. PLC

106. Differential Diagnoses PLEVA Lymphomatoid papulosis Cutaneous small vessel vasculitis Lichenoid drug eruption Arthropod reactions Varicella, enteroviral exanthems Folliculitis Erythema multiforme Dermatitis herpetiformis PLC Small plaque parapsoriasis Guttate psoriasis Lichen planus Pityriasis rosea Secondary syphilis Lymphomatoid papulosis Papular dermatitis Lichenoid drug eruption

107. Histological features Pityriasis lichenoides exhibits a superficial perivascular interface dermatitis in all cases.

108. Histological features PLEVA PLC S. corneum Hyperkeratosis Foci of parakeratosis Few neutrophils Laminated Hyperkeratosis Foci of thin flat parakeratosis w/o neutrophils Granular cell layer Deficient beneath mound of parakeratosis Well formed Prickle cell layer From edema to extensive epidermal necrosis in well-developed lesions. No or focal necrotic keratinocytes Basal cell layer & dermo -epidermal junction. Marked vacuolar changes Denser interface lymphocytic infiltrate . Very focal mild basal cell vacuolization which may not be evident in well-developed or resolving lesions. Milder interface lymphocytic infiltrate. Dermis Shows denser superficial & deep perivascular wedge-shaped lymphocytic infiltrates. Numerous erythrocyte extravasation. Only a superficial perivascular lymphocytic infiltrate. Mild erythrocyte extravasation.

109. PLEVA

110. PLC

111. Treatment FIRST LINE Topical corticosteroids Topical coal tar preparations Antibiotics ( erythromycin 500 mg PO 2-4× daily ; azithromycin ; tetracycline 500 mg PO 2-4× daily, minocycline 100 mg PO twice daily) Phototherapy (Sunlight, ultraviolet A, broadband or narrowband UVB ) SECOND LINE Topical tacrolimus Methotrexate (10-25 mg PO weekly) Phototherapy (ultraviolet AI, PUVA) Cyclosporine Biological agents : etanercept IVIg

112. Treatment If a drug association is suspected , stop the offending drug . SPECIAL TREATMENTS: Prednisone: (60/40/20 mg PO taper, 5 days each) If there is fever, arthritis or other systemic findings. Antihistamines: if pruritus is present. Other Systemic antibiotics: If there is secondary infection.

113. REFERENCES Bolognia 3 rd ed http://dermnetnz.org Google images Husein Oozeerally (Presentation) Jonathan Faulkner (Presentation ) emdicine.com dermis.net

114. THANK YOU