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Home , Erythroderma, Pityriasis, Pityriasis rosea, Pityriasis rubra pilaris

International Journal of Pathology; 2005; 3(2): 91-93

Case Report

Erythroderma Due to Rubra Pilaris

Ikramullah Khan and Uzma Khalil

Department of , Pakistan Institute of Medical Sciences, Islamabad

Erythroderma can be due to a number of causes. 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 HAART, highly active anti retroviral therapy

Introduction and toe nails were shiny. Erythroderma is generalized of Nails were thickened and yellowish in colour skin affecting over 90% of body surface area. It with splinter hemorrhages and whitish longitudinal can be due to several causes. Pityriasis rubra pilaris bands. There was also subungual .. contributes to one percent of all cases of erythroderma. Palms and soles were hyperkeratotic named as PRP Pityriasis rubra pilaris is a heterogenous group of sandals. Usually PRP is associated with HIV infection disorders that have circumscribed follicular keratosis, but our patient was negative for it. brauny skin and orange red erythema. This is A skin taken from arm showed a rare disease with an incidence of one in 50001. It hyperkeratosis, parafollicular , follicular has a world wide distribution.15 It is one of the plugging, prominent granular layer (Figure 4 & 5). papulo squamous disorders that runs a chronic showed mild capillary dilatation and mild relapsing course.5,13 lymphohistiocytic infilterate. Some eosinophils were We report a case of pityriasis rubra also noted. pilaris which progressed into erythroderma. The A diagnosis of PRP was made and he was erythroderma subsided by the use of topical started on oral retinoids to which he responded emollients and oral retinoids. well. Our patient on follow up visits showed marked improvement. Case Report A 20 years old man presented with Discussion , itching and dryness of skin for Pityriasis rubra pilaris belongs to a group last 2 months. of rare erythemato squamous disorders of unknown The patient history dates back to 3 years when etiology. It is characterized by: he started having erythema, dryness and scaling of scalp and face which progressed to involve trunk and Pityriasis----prominent scaling limbs. The was itchy and the skin was dry. On examination there was generalized Rubra------perifollicular redness erythema of the body with an orangish tinge. There was mild bilateral pedal oedema. Cardiovascular Pilaris-----follicular plugging and respiratory systems were normal. The rest of systemic examination was unremarkable. There was It may become erythrodermic and is furaceous yellowish orange fine branny scaling on assocaiated with palmo-plantar hyper keratosis.1 whole of the body more marked on back. The finger Griffith described five subtypes and a new

91 International Journal of Pathology; 2005; 3(2): 91-93 sixth one has been described associated with HIV infection. labelling is increased from an average normal 3% to 27%. The growth is increased as well but not as fast as in

Type -1 Classical

Adult Form The condition is Fig. 4: Skin Biopsy showing Dilated severly itchy starts from Follicular Infundibulum Filled with head spreads towards Fig. 1: Generalized Erythema with Keratin and Parakeratosisat distal parts of body.6 It Follicular Shoulders Orangish Scaling 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 Fig. 2: Feet of the Patient showing hemorrhages. Scaling and Erythema It may progress to erythroderma7 ,8 All Fig. 5: Skin Biopsy showing Mitosis these findings were present in our patient. Indicating Increased Epidermal There is Squamous Cell Proliferation 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.

Fig. 3: Skin showing Perifolloicular Type –III -Classical Juvenile Form; Erythema and Scaling 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 There is increased epidermal resemble adult PRP but may follow an acute infection.2 overactivity as evidenced by increased It may clear spontaneously within one to two years2. labelling indices. Epidermal thymidine

92 International Journal of Pathology; 2005; 3(2): 91-93

Type IV –Juvenile Onset Circumscribed plugging are present on elbows and knees. It may involve Type; scalp. It remains localized to knees and elbows, does not progress Circumscribed persistent further. asymptomatic plaques of follicular Type –V –Atypical Juvenile Variant can also be used. It presents early in life or even at birth. There TYPE VI PRP responds to HAART.2 is erythema, hyper keratosis follicular plugging, Photo chemotherapy may exacerbate disease palmoplantar keratoderma. It is wide spread and but narrow band UVB, PUVA bath and extra corporeal shows little tendency to clear. Some cases are familial.2 photophoresis have been found to be beneficial.2 Our case presents classical PRP clinically with TYPE - VI PRP Associated with HIV; charactristic findings on skin biopsy. Thus PRP, PRP like eruption can occur with HIV . It although rare, should be kept in the list of differential responds to HAART i.e highly active anti retro diagnosis in cases of atypical erythrodermic and viraltherapy.for HIV . psoraisiform disorders. It shows features of classical PRP with filiform keratosis of scalp face and trunk. it is also associated with conglobata.2 4 References There are no definite laboratory tests for PRP. Skin histopathology shows basket weave 1. VivierAD,,pityriasis rubra pilaris.Atlas of clinicl hyperkeratosis, prominent follicular plugging, dermatology .third ed.spain elsivier,2004 91-93 2. MRjudge, W.H.I. Mclean, and C.S. Munro, Disorders of keratinization, ,acanthosis with exaggerated follicular shoulders, Rook, s text book of dermatology ,seventh edition italy Blackwell ,spotty parakeratosis and mild upper dermal science. 2004,64-66 3. Sanchez-Regana M,CreusL umbertP,pityriasi rubra pilaris a long term infiltrate. study of 25 cases,Eur j dermatol.1 1994 ;593-7 4. Weislo Dziadecka,D Krauze etal;diagnostic and therapeutic difficulties In our patient, since he was erythrodermic a in pityriasis rubra pilaris Case report PolMerkurtsz 2005; 19 (113 ): prominent scab was also noted above . 667-70. 5. Picnus D J,pityriasis rubra pilaris,a clinical review,Dermatol nurs 2005; As epidermal activity is increased so mitotic figures 17(6): 448-51. were also seen. 6. Booth A V, Ma L Pityriasis rubra pilaris type I Dermatol online J ,2005; J: 11(4) 9 The major differentials of the condition were 7. Pitman J,Muckusch.G pityriasis rubra pilaris Dermatol nurs 2005 17; psoriasis, but in an erythrodermic patient like ours (4): 302 8. Artik,S Khun A,Neumann N J, etal;pityriasis rubra pilaris ,Hautarzt other causes like eczema, , 2004; 55 (10): 980-3 fungoides, and Sezary syndrome must also be kept in 9. Whit K I pityriasis rubra pilaris Dermatol online J 2003; 9 (4): 6 10. Usuki K, Sekiaona T ,etal; Three cases of Pityriasis rubra pilaris mind. Successfully treated with ciclosporin A Since it is a disorder of keratinization ,it 11. Sehgal V N,Bajaj P,Jain S ,pityraisis rubra pilaris a report of four cases J Dermatol 2000; 27 (3): 174-7 responds well to oral retinoids. Like acitretin 12. Kirbey B , Watson ,R pityraisis rubra piilaris treated with Acitretin and isotertinoin12 can be used. Methotrexate is narrow band ultrvoilet B Brj Dermatol 2000; ,142 ( 8) 376-77 13. Caplan S,E Lowitt M,H ,Early presentation of pityriasis rubra pilaris, an alternative and as an adjunct to retinoids. Cutis 1997 6 (6) 291-6 10 14. Clayton B D, Jorlzzo J L et al; adult pityriasis rubra pilarisa 10 years CiyclosporinA and azathioprine have also case series J AMm AcadDermatol,1997; 3: 959 -64 been used Emollients help in reducing scales. In 15. Jacyk W,k pityriasis rubra pilaris in black south Africans clin Exp circumscribed variety topical vitamin D analogues Dermatol 1999; 24 (3): 160-30

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