Pityriasis Rubra Pilaris

Explanation:

A i.e. Isolated patches of normal skin are found; B i.e. Cephalo caudal distribution

PRP presents with erythematous slightly scaly macules (or dermatitis) and perifollicular hyperkeratotic papules, erythroderma with characteristic orange hue, diagnostic islands of normal – unaffected skin (nappes claires)Q; waxy­hyperkeratotic – thickened and yellow palm & soles (PRP sandal) and follicular accentuationQ.

Nonscaly Hypopigmented macule

• Indeterminate leprosy   •

More common in children
– More frequent in patients

‑ belonging to high leprosy prevalence (endemic) state as Tamil Nadu, Bihar, Orrisa etc.

– Anaesthetic/Non anaesthetic lesion

– Epidermal atrophyQ

• Tuberous sclerosis

–    Ash leaf maculeQ

–    Shagreen patchQ

–    Adenoma sebaciumQ

–    Seizure, mental retardationQ                          

• Vitiligo

–  Sharply defined

– Non pigmented patches – Anywhere

Recurrent Scaly Nonanesthetic

Hypopigmented macule

Pityriasis alba (Predominant face involvement)

Self limiting benign condition mostly affecting head & neck regions (most common on cheeks, around the mouth & chin) of preadolescent childrenQ b/w the ages of 3 & 16 years (before puberty).

More frequent in children of hill station exposed to excessive & unprotected sun, and hot baths.

Presents with multiple ill defined, irregular, round or oval, erythematous or hypopigmented macule without or with fine powdery white scaleQ. Pityriasis versicolor (Predominant trunk involvement)

Common in young adults (rare in children) Q. Cushing’s syndrome steroids, oral anticoagulants, hot & humid climate aggrevate lesions. Multiple small scaly hypopigmented (pale, non erythematous) maculesQ (may also be hyperpigmented/ brown, pink or salmon colored) develop insidiously over trunk, chest, back and shouldersQ. Macules start around the hair follicle. Face involvement is rare. Hands & lower limbs are usually not involved.

Scales are dust like (fine, branny) or furfaceousQ.

Erythematous Scaly

Lesions

Pityriasis rubra pilaris (starts from face) Sudden onset without precipitating factor in late middle aged or elderly (40-60 yr) Q

Begins on face and scalp & spreads in cephalo caudal directionQ to involve rest of body within weeks.

Patient shows erythematous slightly scaly macules and perifollicular hyperkeratotic papules. Gradually erythematous scaling dermatitis with characteristic orange hueQ appears which often progresses to generalized erythroderma.

Diagnostic hallmark is sharply demarcated islands of sparing or normal/unaffected skin (k/a nappes claires) Q.

Waxy, thickened, hyperkeratitic & yellow palms & soles (PRP sandal), follicular accentuationQ and diffuse bran scaling are other features. Pityriasis rosea (predominant trunk involvement)

Self limiting disorderQ of sudden onset mostly affecting children & young adults (10-35 years). May be a/ w reactivation of HHV 7 and 6. Ist feature is development of larger, single, sharply (well) demarcated, annular, erythematous or lzyperpigmented, asymptomatic, scaly patch usually on trunkQ (k/a herald/ primary/ mother patch).

Lesions show fine cigarette paper collarette of scale just inside the periphery of plaqueQ. Similar but smaller secondary eruptions appear in crops in christmas or fir tree patternQ.

Explanation:

A i.e. Pityriasis rosea

Explanation:

C i.e. Pityriasis rubra pilaris

Keratoderma is seen in PRP, psoriasis, Reiter’s syndrome (keratoderma blenorrhagica), arsenic and Haxthausen’s disease.

Explanation:

Ans. is ‘d’ i.e., Pityriasis rubra pilaris 

Pityriasis rubra pilaris

• It is a chronic papulosquamous disorder of unknown etiology characterized by : –

1. Scaling (Pityriasis means scaling Skin disorder)
2. Erythmatous plaque (Rubra)
3. Follicular papules (Pilaris)

• Hence the name pityriasis rubra pilaris.
• Two peaks of age are seen : –

1. 5-10 years in juvenile type
2. 40 – 60 years in adult type

Clinical presentation

• Characteristic lesions are erythmatous (orange to pink), follicular, scaly plaques. A characteristic feature of plaques is the presence of distinct islands of normal skin. Sites of predilection
• Trunk —> Lesions on the trunk evolve in craniocaudal (Cephalo caudal) direction. Typically, follicular lesions are seen on the dorsum of hands on knuckles —> Nutmeg papules.

Associated features

• Diffuse erythema and scaling of face.
• Orangish thickening of palms and soles (Keratodermic sandals).
• Nails —> Distal yellow brown discoloration and nail plate thickening.
• Complications –> Erythroderma

Treatment

• Localized lesions —> Topical corticosteroids + Keratolytics (Salicylic acid, urea)
• Erythroderma –> Vitamin A, Acitretin (Retinoids), oral methotrexate.

Explanation:

Ans. is `d’ i.e., Pityriasis rubra pilaris

Pityriasis rubra pilaris

• It is a chronic papulosquamous disorder of unknown etiology characterized by : ‑

1. Scaling (Pityriasis means scaling Skin disorder)
2. Erythmatous plaque (Rubra)
3. Follicular papules (Pilaris)

• Hence the name pityriasis rubra pilaris.
• Two peaks of age are seen : ‑

1. 5-10 years in juvenile type
2. 40 – 60 years in adult type

Clinical presentation

• Characteristic lesions are erythmatous (orange to pink), follicular, scaly plaques. A characteristic feature of plaques is the presence of distinct islands of normal skin.

Sites of predilection

• Trunk —> Lesions on the trunk evolve in craniocaudal (Cephalo caudal) direction. Typically, follicular lesions are seen on the dorsum of hands on knuckles –> Nutmeg papules. Associated features

1. Diffuse erythema and scaling of face.
2. Orangish thickening of palms and soles (Keratodermic sandals).
3. Nails —> Distal yellow brown discoloration and nail plate thickening.

Complications Erythroderma

Treatment

• Localized lesions —> Topical corticosteroids + Keratolytics (Salicylic acid, urea)
• Erythroderma -4 Vitamin A, Acitretin (Retinoids), oral methotrexate