TOP DERMATOLOGIST PRESENTED A CASE STUDY OF ERYTHRODERMA DUE TO PITYRIASIS RUBRA PILARI
Top dermatologist presented a case study regarding Erythroderma due to Pityriasis Rubra Pilari. This study was published in the prestigious ‘International Journal of Pathology; 2005; 3(2): 91-93 91’
Erythroderma Due to Pityriasis Rubra Pilaris
Ikramullah Khan and Uzma Khalil
Department of Dermatology, Pakistan Institute of Medical Sciences, Islamabad
Erythroderma can be due to a number of causes. Pityriasis Rubra Pilaris contributing to 1% of all the cases. Pityriasis Rubra Pilaris belongs to a rare erythematosquamous disorders of unknown etiology. We report here a case of Pityriasis Rubra Pilaris that progressed to erythroderma.
Key words: pityriasis rubra pilaaris, PRP, HIV human immunodeficiency virus HAART, highly active anti retroviral therapy
Introduction Erythroderma is generalized erythema of skin affecting over 90% of body surface area. It can be due to several causes. Pityriasis rubra pilaris contributes to one percent of all cases of erythroderma. Pityriasis rubra pilaris is a heterogenous group of disorders that have circumscribed follicular keratosis, brauny skin and orange red erythema. This is a rare disease with an incidence of one in 50001. It has a world wide distribution.15 It is one of the papulo squamous disorders that runs a chronic relapsing course.5,13
We report a case of pityriasis rubra pilaris which progressed into erythroderma. The erythroderma subsided by the use of topical emollients and oral retinoids.
Case Report A 20 years old man presented with generalized erythema, itching and dryness of skin for last 2 months.
The patient history dates back to 3 years when he started having erythema, dryness and scaling of scalp and face which progressed to involve trunk and limbs. The rash was itchy and the skin was dry.
On examination there was generalized erythema of the body with an orangish tinge. There was mild bilateral pedal oedema. Cardiovascular and respiratory systems were normal. The rest of systemic examination was unremarkable. There was furaceous yellowish orange fine branny scaling on whole of the body more marked on back. The finger and toe nails were shiny.
Nails were thickened and yellowish in colour with splinter hemorrhages and whitish longitudinal bands. There was also subungual hyperkeratosis.. Palms and soles were hyperkeratotic named as PRP sandals. Usually PRP is associated with HIV infection but our patient was negative for it.
A skin biopsy taken from arm showed hyperkeratosis, parafollicular parakeratosis, follicular plugging, prominent granular layer (Figure 4 & 5). Dermis showed mild capillary dilatation and mild lymphohistiocytic infilterate. Some eosinophils were also noted.
A diagnosis of PRP was made and he was started on oral retinoids to which he responded well. Our patient on follow up visits showed marked improvement.
Discussion Pityriasis rubra pilaris belongs to a group of rare erythemato squamous disorders of unknown etiology. It is characterized by:
Type – I Classical Adult Form; The condition is severely itchy starts from head spreads towards distal parts of body.6 It has powdery scaling and pilosebaceous follicles are plugged with keratin.
They are surrounded by erythema, the over all colour is orangish yellow.
The nails show longitudinal ridges, sub ungual hyperkeratosis and splinter hemorrhages.
It may progress to erythroderma7 ,8 All these findings were present in our patient.
There is spontaneous remission with in two to three years without recurrence.9,11
Type – II Adult Atypical Form; In this form scales are coarser and more lamellated. The legs are particularly involved. Five percent of the patients belong to this type.
Type – III Classical Juvenile Form; It is not present at birth and appears at the end of first decade .One third of the patients may have family history. It may resemble adult PRP but may follow an acute infection.2
It may clear spontaneously within one to two years2.
Type – IV Juvenile Onset CircumscribedType;Circumscribed persistent asymptomatic plaques of follicular plugging are present on elbows and knees. It may involve scalp. It remains localized to knees and elbows, does not progress further.
Type – V Atypical Juvenile Variant; It presents early in life or even at birth. There is erythema, hyper keratosis follicular plugging, palmoplantar keratoderma. It is wide spread and shows little tendency to clear. Some cases are familial.2
Type – VI PRP Associated with HIV; PRP like eruption can occur with HIV. It responds to HAART i.e highly active anti retro viraltherapy for HIV.
It shows features of classical PRP with filiform keratosis of scalp face and trunk. it is also associated with acne conglobata.2
There are no definite laboratory tests for PRP.4
Skin histopathology shows basket weave hyperkeratosis, prominent follicular plugging, ,acanthosis with exaggerated follicular shoulders, ,spotty parakeratosis and mild upper dermal infiltrate.
In our patient, since he was erythrodermic a prominent scab was also noted above epidermis. As epidermal activity is increased so mitotic figures were also seen.
The major differentials of the condition were psoriasis, but in an erythrodermic patient like ours other causes like eczema, drug eruption, Mycosis fungoides, and Sezary syndrome must also be kept in mind.
Since it is a disorder of keratinization ,it responds well to oral retinoids. Like acitretin and isotertinoin12 can be used. Methotrexate is an alternative and as an adjunct to retinoids. CiyclosporinA 10 and azathioprine have also been used Emollients help in reducing scales. In circumscribed variety topical vitamin D analogues can also be used.
TYPE VI PRP responds to HAART.2
Photo chemotherapy may exacerbate disease but narrow band UVB, PUVA bath and extra corporeal photophoresis have been found to be beneficial.2
Our case presents classical PRP clinically with charactristic findings on skin biopsy. Thus PRP, although rare, should be kept in the list of differential diagnosis in cases of atypical erythrodermic and psoraisiform disorders.
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