Lichenoid dermatoses can be divided into 2 categories: Lichenoid Interface Lichenoid inflammation is seen in entities that are usually benign and self

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1 Lichenoid reaction pattern Nathan C. Walk, M.D.

2 Lichenoid dermatoses can be divided into 2 categories: Lichenoid Interface Lichenoid inflammation is seen in entities that are usually benign and self limited and shows: Prominent band-like inflammatory infiltrate Less prominent basal vacuolar change Invert this scenario and you have those entities with more morbidity and mortality: Prominent basal vacuolar change Less prominent inflammatory infiltrate

3 Predominately. Lichenoid Lichen planus, and variants Lichen nitidus Lichen striatus LPLK Lichenoid drug eruption Interface Fixed drug eruption* Erythema multiforme/ten Graft versus host disease Eruption of lymphocyte recovery Lupus erythematosus Dermatomyositis

4 Other lichenoid entities: **Poikilodermas **Lichen sclerosus et atrophicus **Pityriasis lichenoides **Persistent viral Perniosis Paraneoplastic pemphigus Lichenoid purpura **Lichenoid contact Late syphillis Porokeratosis Drug eruptions **Phototoxic dermatitis Prurigo pigmentosa MF **Vitiligo Lichen amyloid Lichenoid tattoo

5 Cases 10 & 11

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10 Lichen planus Clinical: Cutaneous % population, oral form 1-4% 40s-50s, children uncommon, elderly rare Small, polygonal-shaped shaped, violaceous, flat-topped topped pruritic papules that favor the extremities. Shiny or transparent surface, with fine white lines called Wickham s striae + Koebbner phenomenon. Flexor surfaces of wrists and forearms, dorsal hands, anterior lower legs, g, neck,,p presacral, oral >1/2 of cases sometimes only site. Course depends on subtype resolution in 3-12 months in acute form, oral usually lifetime. Spontaneous resolution UNCOMMON.

11 Histology: Prominent Civatte bodies Band-like inflammatory infiltrate presses against epidermis & usually does not obscure D-E interface or extend into mid epidermis (EM, fixed drug). Hyperkeratosis, NO parakeratosis, wedge-shaped hypergranulosis related to acrosyringia and acrotrichia, c a, variable ab basal vacuolar change, variable acanthosis. Pointed or saw tooth rete. Oh Other: Colloid bodies (PAS positive) in papillary dermis. DIF shows colloid bodies stain for complement and IgM. Some pigment incontinence. Max-Joseph spaces = small clefts at D- E jnx from coalescence of BV change.

12 Clinical Differential: LP variants atrophic, ulcerative, hypertrophic, linear, actinic, acute, annular, bullous Lichenoid drug eruption (LDE) more eczematous/psoriasisform, more likely in sun exposed areas Lichen nitidus, lichen striatus, lichen sclerosus Pityriasis rosea, EDP Psoriasis Secondary syphilis Paraneoplastic pemphigus, if erosive LP-like LE most difficult to differentiate especially in patients with only oral or scalp lesions try looking for lupus band test

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17 Hypertrophic LP Clinical: Usually on shins persist for many years, may develop SCC. Histology: Looks LSC-like with compact orthokeratosis, psoriasiform hyperplasia....but, has basal cell damage w/ civatte bodies at rete tips. Inflammatory band less pronounced.

18 Case 13

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25 Lichen planopilaris (LPP) Clinical: Keratotic follicular lesions are present in a/w other manifestations of flp LP. Most important clinical group is characterized by scarring alopecia. Keratotic spines surrounded by violaceous rim scalp, other hair-bearing surfaces. Alopecia + scarring late.

26 Histology: Lichenoid reaction pattern involving follicular epithelium Infundibulum and isthmus ***Unlike lupus: Not much PVLI Not as deep Interfollicular epidermis characteristically NOT involved, but in reality can be in up to 1/3 of cases involving scalp.

27 Case 14

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33 Lichen striatus Clinical: Continuous or interrupted band of discrete or clustered pink, skin-colored papules p that are flat topped, smooth or scaly, and range from 2-4 mm. Predilection for female children. Follow Blaschko s lines. Usually along one side of body, usually length of extremity. Spontaneous resolution in 1-2 years.

34 Histology: Lichenoid id reaction pattern occupying 3or 4 adjacent dermal papillae; irregular and discontinuous. +/- around follicles, sweat glands c/w LE Overlying epidermis changes secondary - acanthotic with exocytosis of inflammatory cells, may have dyskeratotic cells. +/- intraepidermal vesicles Infiltrate less dense than in LP, may extend around hair follicles or eccrine glands.

35 Clinical differential: LP versus LS Differ in size and color hypopigmentation is frequent sequela of LS, while hyperpigmentation appears in the wake of LP. LS MINIMAL pruritus.

36 Case 15

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40 Lichen nitidus Clinical: Asymptomatic, numerous, tiny, discrete, skin-colored, uniform,,p pinhead sized papules. Papules are flat with a shiny surface. Flexor aspects of upper extremities, genitalia, chest, abdomen, dorsal hands. + Koebbner phenomenon

41 Histology: Well circumscribed, subepidermal infiltrate, limited to 1-2 dermal papillae. Claw-like like acanthotic rete grasp the infiltrate Thinned epidermis +/- parakeratosis Lymphocytes, histiocytes, t melanophages, giant cells. Sometimes frankly granulomatous. Clinical DDx: LP, verruca plana, papular sarcoid, papular eczema, lichen scrofulosorum

42 Case 16

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46 Lichen planus like keratosis Pathogenesis: Arise from pre-existing existing solar lentigo of SK. OR Inflamed actinic keratosis. Unidentified epidermal antigen stimulated Langerhaans cells T cells; i.e. similar to LP Clinical: Solitary pink to pink-brown, often scaly, papules from cm. Caucasians, 30s-60s. Asymptomatic, ti or pruritic. Forearm, upper chest, > H/N; middle aged women. May appear suddenly.

47 Histology: Lichenoid reaction pattern with numerous civatte bodies in the basal layer and mild basal vacuolar change. Infiltrate usually dense, may obscure D-E interface. Prominent PI Contiguous solar lentigo may be seen. May be subepidermal cleft from confluent apoptosis of keratinocytes.

48 Cases 17 & 18

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55 Erythema multiforme HSV in minor forms. Mycoplasma and drugs in major forms, SJS/TEN. Clinical: Primary lesion = Round, red papule which remains fixed for 7 days may evolve into target lesion. Target lesions: central purple, outer red. Iris lesions: dusky center, then white, then red. Symmetric, predilection for the extremities. Abrupt onset, pruritic or burning sensations. + koebbner

56 Histology: Interface obscuring interface dermatitis; mainly lymphocytes. Prominent epidermal cell death, not confined to basal layer. Basal vacuolar change. Some spongiosis. Vesicular lesions are characterized by clefting at the D- E junction. Dermal infiltrate is lymphocytes and macrophages. Eosinophils are usually not prominent. DIF granular C3, IgM at D-E junction, vessels.

57 Toxic epidermal necrolysis >30% epidermal detachment (helpful guide, not strict rule). NECROSIS and apoptosis. Subepidermal bullae with overlying confluent necrosis of epidermis. SPARSE inflammatory infiltrate. Satellite cell necrosis early c/w GVHD. More inflammation in those cases a/w EM.

58 Fixed drug Looks a LOT like EM, but. Deeper inflammatory infiltrate Neutrophils (NOT in EM) More pigment pg incontinence

59 Cases 19 & 20

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67 Clinical: Graft versus host disease Acute form, 1-3 weeks after transplantation usually could be as long as 3 months Morbilliform (maculopapular) exanthem with sudden onset begins acrally, may be generalized. Folliculotropic. If severe, diffuse erythroderma with bulla formation. Chronic form, usually after mean of 4 months, as soon as 40 days Divided into: Lichenoid: erythematous or violaceous lichenoid papules and plaques affecting dorsal hands, forearms, and trunk. Sclerodermoid: plaques of morphea, eventually generalized scleroderma.

68 Histology: Grade 0 normal skin Grade 1 basal vacuolar change Grade 2 dyskeratotic cells at all levels in the epidermis and/or follicle, dermal lymphocytic y infiltrate Grade 3 fusion of basilar vacuoles to form clefts and microvesicles Grade 4 separation of epidermis from dermis Satellite cell necrosis said to be characteristic. ti In early chronic lesions, looks like LP or LPP, although infiltrate is less dense; PI. Sclerodermoid phase mild epidermal changes, such as atrophy, BV change, dermal fibrosis that extends into subcutis (septal hyalinization) and may involve skin appendages; few dyskeratotic cells.

69 A tough differential GVHD Lymphocyte recovery Drug reaction Viral Also Erythema multiforme What can help. Eosinophils? Inflammatory pattern? Does is obscure interface? Timing? Severity? erit

70 Cases 21 & 22

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78 Lupus erythematosus Clinical: There are several variants of cutaneous lupus, defined in part by the location and depth of the inflammatory infiltrate. ACLE primarily epidermis and upper dermis + systemic disease SCLE DLE primarily epidermis and upper dermis - 15% systemic disease; a/w anti-ro Midface spared, V neck, extensor forearms, sides of face; confined to sun exposed areas, Hypo/hyperpigmentation p after, no scar epidermis, upper and lower dermis, adnexal structures w/ scarring NO systemic dz Face almost always +/- trunk, not related to sun, scarring LE tumidus dermis, no prominent adnexal involvement LE panniculitis subcutaneous tissue, w/ depressed scars

79 Discoid lupus erythematosus Sharply demarcated, erythematous, scaly patches with follicular plugging Face almost always +/- trunk, not related to sun, scarring With scarring NO systemic dz

80 Subacute cutaneous lupus 15% systemic disease; a/w anti-ro Erythematous, slightly scaly eczematous or psoriasiform appearance. Midface spared, V neck, extensor forearms, sides of face; confined to sun exposed areas Hypo/hyperpigmentation after, no scar erythematosus

81 Histology: Lichenoid reaction pattern with S+D PVLI Tendency to accumulate around pilosebaceous follicles. Mostly vacuolar change w/ scattered Civatte bodies. Thick BM in older lesions PAS stain. Epidermal atrophy Follicular plugging which psoriasiform disease characterized by prominent follicular plugging? Increased dermal mucin Lupus band test IgG, IgM > C3 in broad band along BMZ

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87 Dermatomyositis Clinical: Bimodal age distribution Proximal inflammatory myopathy + photodistributed violaceous poikiloderma favoring scalp, periocular, extensor skin sites, nailfold telangiectasias.

88 Histology: Looks like LE, but NO deep inflammation Basal vacuolar change, occasional civattte bodies, dermal mucin, thickened BMZ May also have poikilodermatous changes

89 Case 24

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92 Lichen sclerosus et atrophicus Chronic disorder, men and women Usually asymptomatic, may p/w intractable pruritus/soreness Hypopigmentation, thin, wrinkled atrophic skin. Scarring disease Increased risk of SCC? Relation to morphea?

93 Lichenoid Clues If predominately. Lichen planus, and variants Lichen nitidus Lichen striatus LPLK Lichenoid drug eruption Interface Fixed drug eruption Erythema multiforme/ten Graft versus host disease Eruption of lymphocyte recovery Lupus erythematosus Dermatomyositis

94 Clues Folliculotropic? GVHD Deep inflammation? DLE FDE Lichen striatus t Interface obscuring inflammation? EM FDE PLEVA but also has lymph vasculitis Prominent pigment incontinence? Drugs Poikilodermas Eccrine duct involvement? EM (drug induced) L. striatus