PAPULOSQUAMOUS DISORDERS

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Presentation transcript:

PAPULOSQUAMOUS DISORDERS DR. HADAF A. HASAN

LICHEN PLANUS A common, pruritic, inf. Disease of skin, mucosa & hair follicles. Aetiology: 1)immune: association with autoimmune disorders & graft vs. host dis. 2) drugs: as gold, antimalarials, thiazides, anti-TB, sulphonamide & others. 3) ass. With chronic active hepatitis & hepatitis B,C. 4) Color developers.

Presentation of L.P Characteristic; almost pathognomonic primary lesions: small, violaceous, flat-topped, polygonal papules. Erythema. then violaceous, then hyperpigmented Glistening dry surface with whitish streaks or puncta (wickham stria) Sites: flexor wrists, med. Thighs, trunk, shins, dorsal hands & glans penis. Positive Koebner, prominent itching

Lichen planus Mucous membrane involvement: 50% of patients, asymptomatic White lacy lines & dots, or small plaques Usually inside cheeks, lips Sometimes may be the sole manifestation (oral LP) Genital mucosa may show same picture

Lichen planus Nail changes: In 5-10% of patients May be the only manifestation esp. children Longitudinal ridging, splitting , onychlysis, red lunula Pterygium formation is characteristic of LP

Clinical types of LP 1) Linear: one band or streak, children more 2) annular: 90% are males, axillae & genitalia, asymptomatic, few lesions. 3) Hypertrophic: mostly shins, verrucous plaques + scales. 4) Ulcerative: rare on skin, more on mucous membrane, on sole give bullae + ulcers+ permanent toenail loss

Clinical types of LP 5) Follicular (lichen planopilaris): 70-80% are females leading to scarring alopecia. 6) Actinic LP: mostly middle east, sun exposed skin, in spring or summer. Adolescents & young adults Face is target organ; forehead, cheeks, lips Annular, hyperpigmented plaques+ hypopigmented margin, minimal itching.

Histopathology of LP 1)Interface dermatitis along DEJ leading to destruction of basal layer. 2) A saw-tooth hyperplasia of epidermis+ beaded hypergranulosis. 3) A band-like inflammatory infiltrate in the superficial dermis of lymphocytes & macrophages. Civatte bodies: necrotic keratinocytes in superficial dermis

Differential diagnosis of LP Skin lesions: from lichenoid drug rashes, guttate psoriasis, lichenoid syphilid, pit. rosea. Mucosal lesions: From leukoplakia, candidiasis, oral LE, oral pemphigus, syphilitic mucous patches

Lichen planus course Individual lesions may last months The disease itself may last for 1 year Hypertrophic lesions are more chronic Hyperpigmentation usually follow resolution Recurrence rate is 1 in 6

Treatment of LP May be difficult 1)Limited lesions by superpotent topical or intralesional steroids 2)Systemic steroids in certain cicumstances: Extensive lesions, nail destrucion, painful erosive oral lesions. 3)phototherpy: PUVA+ narrow-band UVB 4)Retinoids: in hypertrophic types 5) Antihistamines to relieve itching

Oral LP management Superpotent steroids in orabase or gel form Intralesional steroids may be given for focal unresponsive lesions 0.1% tacrolimus ointment is now the standard treatment for erosive LP of oral & genital mucosa.

PITYRIASIS ROSEA Mild inflammatory exanthem characterized by pinkish, macular,& papular lesions; 1st discrete, then confluent. Mainly children & young adults. More in spring & autumn Women more than men Herpes virus 6 & 7 implicated in etiology Not contagious, 2% recurrence rate

presentation Begin with herald patch which is larger, redder, more scaly than remaining rash Which consist of oval or circinate patches covered with dry, crinkled, surface, desquamates leaving a collarette scale Mainly trunk, extremities, neck Inverted Christmas tree pattern Mild pruritis & constitutional symptoms

Clinical types of P.R Papular PR more in children under 5 years Inverse P.R opposite sites of predilection Purpuric type with purpura & ecchymosis at sites of typical P.R Last 2-10 weeks & resolves spontaneously leaving sometimes a post inflammatory hyper pigmentation

DIFFERENTIAL DIAGNOSIS P.R may closely resemble: Tinea corporis, pityriasis vesicolor, secondary syphilis, seborrheic dermatitis, drug rash , scabies, viral exanthem, lichen planus & psoriasis

Treatment Most cases no treatment is required To shorten the course or decrease itching: UVB in erythema doses Corticosteroids are the standard therapy Oral antihistamines Emollients to relieve dryness & irritation Erythromycine 250mg q.d.s for 2 weeks

Reactive erythema erythema multiformi A reaction pattern of multiform erythematous lesions. May be precipitated by certain factors: Viral: preceding oral HHV 1-2 weeks previously, also orf, mycoplasma, hepatitis A,B,C. bacterial, fungal, & parasitic infection. Drugs: sulfa, NSAID, anticonvulsants, & others Pregnancy Malignancy or its treatment with radiotherapy

Presentation of erythema multiformi Sharply marginated, erythematous macule. Become raised edematous papules over 24-48 hours. A ring of erythema forms around the periphery, with flatter, purpuric dusky center, giving” target lesions” of 3 zones Bilateral, symmetrical, & acral distribution Mostly starting on dorsa of hands

Presentation of E.M Sites of predilection are extensor limbs, face, elbows, knees, palms & soles. Oral mucosal involvement in 25% of cases Stevens-Johnson syndrome is a severe variant with bullous lesions, fever & extensive mucosal involvement. May be complicated by asphyxia, blindness

Treatment of E.M Most cases are self-limited with symptomatic treatment. Remove precipitating factors. Recurrent HHV infection may be prevented by oral acyclovir 200mg 3-5 times/day. Steven-Johnson may require aggressive treatment: I.V. gamma globuline, good nutrition, fluid & electrolyte balance, prevent secondary infection,+ high dose short course systemic steroids & good nursing care for eyes & mouth.

Erythema nodosum Inflammation of subcutaneous fat (panniculitis), elicited by many factors: 1) Bacterial infection: as T.B, strept., brucella, leprosy, yersinia. 2) viral, mycoplasma, rickettsia,& chlamydia 3)Fungal as coccidiomycosis 4)malignancy, sarcoidosis, ulcerative colitis, Behcet disease 5)drugs: sulfa & oral contraceptives.

Presentation of erythema nodosum Characteristic lesion is a tender red nodule , alone or grouped on shins or forearms. Other areas as thighs, face, breast. Bilateral, symmetrical lesions. Mostly in young adult women. Acute onset, frequently with const. symp. Resolve within few days leaving bruise like Course last 6-8 weeks

Investigations & treatment Thorough history +physical examination Chest x-ray, throat culture, ASO titre Treatment is simple consisting of bed rest, NSAID as aspirin, indomethacin, ibuprofen Systemic steroids not used Potassium iodide in a dose of 400-900 mg/day but not for more than 6 months