Skin Problems Pityriasis rubra pilaris

January 17, 2003

Thnx fof info marilyn i really hope i dont have this skin condition too just

nother thing dang.Did join nother group for it though just in case its support

group like this one.Got message from one maybe thnking she has stills i replied

to her with sum of the possible symtoms and told her if she thnks she might she

should find a good rhumy.The PRP thing was just sumthing the dermatolgist said

it looks like i go on weds again.Seen sum really nasty rashes of wht this could

turn into if it is and it dont look good a few of the pictures did look like my

rash tho.Just like stills there is no specific test for it as far as i know.But

its late i better get sum rest,OH by way we got sum snow now but still not

enough for good snomobiling lol take care all g-nite. d. Canada --

Original Message -----

From: Marilyn Hogg

stillsdisease

Sent: Thursday, January 16, 2003 11:20 PM

Subject: Skin Problems Pityriasis rubra pilaris

I think this was for Docken, if I'm correct. Hope it sheds some

light on this skin problem you are now dealing with....Marilyn, Canada

Skin Problems Pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) is a fairly uncommon dermatological

condition. Its etiology is currently unknown. Rare forms are inherited via

an autosomal dominant route. Most forms, however, are acquired. The major

presentation is that of " generalized erythroderma with islands of sparing " .

(Clayton et al, 1997) These islands of sparing present as patches of normal

skin interspersed between areas of yellow to orange colored scales. It often

begins on the scalp and then progresses downward to the feet. Peak incidence

occurs in the first and fifth decades of life.

Classification

The presentation below is based on information published in the various

journal articles cited, especially the one by Clayton et al (1997) and the

one by Cohen and Prystowsky (1989).

Type I

Type I PRP is the most common type. Its mean onset is at age 55. According

to Vanderhooft et al (1995), this disease has an incidence of 1:3500 to

1:5000 among patients presenting to a dermatologist for the first time. The

erythrodermatous lesions tend to spread from " cephalad to caudad " (Cohen &

Prystowsky, 1989) with patches of normal-looking skin interspersed. The

palms and soles tend to be hyperkeratotic as the stratum corneum is much

thicker than normal. This hyperkeratosis often has an orange hue. The knees

and elbows tend to be covered with follicular-based papules. (Clayton et al,

1997) According to Cohen and Prystowsky (1989), the finger and toe nails

tend to have a distal yellow-brown discoloration, subungual hyperkeratosis,

nail plate thickening, and splinter hemorrhages. Some patients also showed

" accentuation of their seborrheic keratoses " (ibid) as would present if the

patient had a malignancy. Remission rates tend to be highest for this type

of pityriasis rubra pilaris.

Common histological presentations tend to be nonspecific. There tends to be

a hyperkeratosis and a " psoriasiform hyperplasia, alternating with

orthokeratosis and parakeratosis in vertical and horizontal directions " .

(Clayton et al, 1997) " Acanthosis, a confluent granular layer, and a

perivascular lymphocytic dermal infiltrate " may also be present. (Cohen &

Prystowsky, 1989) Pruritis may also be caused.

Type II

Type II PRP is a rare form. It is characterized by its long duration, which

is often greater than 20 years. Its presentations, in addition to those of

Type I, include alopecia and ichthyosiform lesions. According to Cohen and

Prystowsky (1989), there also may be eczematous areas. Therefore,

Dicken (1994) believes that this type of pityriasis rubra pilaris may

represent a disorder of keratinization.

Type III

Type III PRP is the classic juvenile type. It tends to present in the same

way as Type I, but its age of onset is frequently between the ages of 1 and

2. According to Darmstadt & Tunnessen (1995), the " eruption starts with a

follicular erythema and scaling of the scalp and face " . He says that this

may occur in a single patch or with thickening of stratum corneum of the

palms and feet. The plaques formed tend to be well-defined, but irregularly

bordered, occurring most commonly on the palms, soles, knees, elbows,

ankles, face, and dorsal surfaces of the hands and feet. The scaling tends

to be finer on the face and thicker on the palms and soles. Darmstadt and

Tunnessen say that the yellowish-orange plaques may progress to generalized

erythroderma with islands of normal skin. (1995) The hair and teeth tend to

be normal. There may be longitudinal ridging, a distal yellow-brown

discoloration, splinter hemorrhages, or subungual hyperkeratosis found with

the nails (Darmstadt & Tunnessen, 1995). Itching is prevalent when the

disease is widespread.

Type IV

Type IV PRP tends to occur in prepubertal children. According to Dicken

(1994), the disease " consists of localized plaques, usually on the elbows

and knees, that are sharply demarcated areas of follicular hyperkeratosis

and erythema " . It rarely progresses to a more serious form.

Type V

Type V PRP is the rarest form. Its onset occurs in the first few years of

life and runs a chronic course. According to Dicken (1994), it may

also be a disorder of keratinization. The main feature of this type is

follicular hyperkeratosis. (Clayton et al, 1997) Erythema may be present,

but is manifested somewhat infrequently. There also may be " scleroderma-like

changes on the palms and soles " . (Cohen & Prystowsky, 1989) There is little

tendency for this to remit spontaneously, in contrast to Type I. According

to Castanet et al (1994), " type V PRP probably overlaps with poorly defined

disorders such as follicular ichthyosis " .

Type VI

Type VI is only beginning to be included as a type of pityriasis rubra

pilaris. According to Miralles et all (1995), type VI is " characterized by

the presence of HIV infection, usually without immunosuppression " . The onset

seems to occur around the same time as the patient tests positive for HIV

infection. Misery, Faure, and Claudy (1008) suggest that it is actually part

of the HIV-associated follicular syndrome, which also includes acne

conglobata, hidradenitis suppurativa, and lichen spinulosus. Most often

these patients are not immunosuppressed and had a helper T-cell count of

greater than 300/mm3. According to Miralles et al, " PRP in HIV-infected

patients often has a simultaneous onset of a severe nodulocystic or

follicular pustular eruption resembling acne or furuncles " . (1995) The

lesions, however, do not contain any pathogenic organisms. The diagnosis is

then made if the other typical features of pityriasis rubra pilaris are

present.

Etiology

The cause of PRP is currently not known. There have been some hypotheses,

however. Darmstadt and Tunnessen cite that some cases of type III may result

from an upper respiratory tract infection. (1995) -Regana et al

(1995) writes that " HIV may precipitate PRP in a genetically predisposed

individual " . Since there are beneficial effects observed after treatment

with vitamin A, some physicians have postulated that a deficiency in vitamin

A or a deficiency in retinol-binding protein are the culprits. These two

postulates, however, have been refuted by more recent studies. From these,

it has been rationalized that the improvements seen upon treatment with

vitamin A may actually be due to the pharmacologic actions of the vitamin

rather than correcting a deficiency. Other postulates correlate to the

presence of other disease states, such as a neoplasm, which may allow for

the expression of the disease. Genetics also seems to play a role as there

is a familial type. Although these observations have been made, there is not

yet an answer to the question of what causes this disease.

Diagnosis

Pityriasis rubra pilaris must initially be differentiated from psoriasis.

The follicular accentuation in PRP is more prominent than in psoriasis

according to Darmstadt & Tunnessen (1995) The plaques in PRP tend to have an

orangish-red hue, whereas in psoriasis the scales tend to be silvery in

appearance. The margins of the plaques in PRP are more irregular. Darmstadt

and Tunnessen (1995) say that with PRP there is no Auspitz's sign, which is

a point of bleeding upon removal of a scale.

Biopsy specimens are taken from the patient to make a correct diagnosis.

Light and electron microscopy will show the thickness of the stratum corneum

along with any infiltrates or other irregularities. Vanderhooft et al (1995)

write that staining of the biopsies of a Type V patient shows " staining of

the basal, spinous, and granular layers with AE1 " whereas the basal layer

was only stained in the control. Another nonspecific finding was the

detection of keratins 6 and 16 by immunoblot. There two keratins are

associated with abnormal differentiation of the epidermis. Another finding

was the presence of " an additional acidic 45-kd keratin, which might

represent k17 " . (Vanderhooft et al, 1995) This keratin (17) is normally

found in basal and myoepithelial cells, but not in normal stratified

squamous epithelia. (ibid)

Treatment

Pityriasis rubra pilaris does not have a universal treatment. The regimen is

variable and often multiple therapies must be tried before a feasible one is

found. Therefore, each patient's disease tends to be treated according to

what the dermatologist finds to be working.

According to Dicken (1994), most patients are initially hospitalized

for 3 to 7 days and receive topical triamcinolone cream 0.05% and tap water

wet dressings to relieve the itching, burning, and exfoliation. After this,

they are given a drug regimen for several months until a remission is seen.

With many of the drugs, a response is seen after 4-6 months of treatment.

The following table lists the drugs and corresponding dosage regimens

utilized for the treatment of pityriasis rubra pilaris.

Darmstadt & Tunnessen (1995) explain the typical treatments for juvenile

PRP. The dermatologist often starts with emollients containing a keratolytic

agent, such as salicylic acid, lactic acid, and/or urea. This tends to

decrease the degree of scaling. Topical corticosteroids are tried next.

These tend to work best under occlusion. If the disease is severe,

widespread, or refractive to the above therapies, systemic retinoid therapy

may be tried. " Juvenile response is better than adults to oral retinoids "

(van Dorren-Greebe & van de Kerkhof, 1995) However, due to the long term

side effects, their use is limited in this population. These long term side

effects include " DISH (diffuse idiopathic skeletal hyperostosis),

extraskeletal ossification, premature epiphyseal closure, and osteoporosis " .

(ibid)

There tends to be a consensus that the UVB or PUVA light therapies are not

beneficial and may aggravate the condition in some instances. There also

seems to be a correlation with exacerbation caused by a previous sunburn or

from sunlight itself.

Isotretinoin and etretinate tend to be the most widely used and tolerated

drugs for the treatment of pityriasis rubra pilaris. If they don't work,

methotrexate is often considered as the next line of therapy. Many patients

taking methotrexate, however, tend to relapse when treatment is stopped. The

condition can then be improved once again after restarting the medication,

but thus indicates chronic treatment.

HIV positive patients show some response to AZT. However, topical

corticosteroids or either isotretinoin or etretinate are used in conjunction

with AZT to obtain a better response. Methotrexate could also be used with

AZT is no response is seen with the other treatments. This type of PRP is

very difficult to treat and often requires a combination of drugs.

Stanozolol, an anabolic steroid, has also been tried. This drug tends to

increase the levels of retinol-binding protein in the body. It can cause

masculinization, and, therefore, is often not used by women. It has met with

variable success.

References

B.D. Clayton, et al, Adult pityriasis rubra rilaris: A 10-year case series,

Journal of the American Academy of Dermatology, 36,959 (1997).

C.H. Dicken, Treatment of classic pityriasis rubra pilaris, Journal of the

American Academy of Dermatology, 31, 997 (1994).

E.S. Miralles, et al, Pityriasis rubra pilaris and human immunodeficiency

virus infection, British Journal of Dermatology, 133, 990 (1995).

G.L. Darmstadt and W.W. Tunnessen, Picture of the Month - Juvenile

Pityriasis Rubra Pilaris, Archives of pediatrics and Adolescent Medicine,

149, 923 (1995).

J Castanet, J.P.H. LaCour, C Perrin, P Brun, and J.P. Ortonne, Juvenile

pityriasis rubra pilaris associated with hypogammaglobulinaemia and

furunculosis, British Journal of Dermatology, 131, 717 (1994).

K.D. Duncan, S Imaeda, and L.M. Milstone, Pneumocystic carinii pneumonia

complicating methotrexate treatment of pityriasis rubra pilaris, Journal of

the American Academy of Dermatology, 39, 276 (1998).

L Misery, M Faure, A Claudy, Pityriasis rubra pilaris and human

immunodeficiency virus infection -Type 6 pityriasis rubra pilaris?, British

Journal of Dermatology, 135,1008 (1996).

M -Regana, C.G. Fuentes, L Creus, M Salleras, and P Umbert,

Pityriasis rubra pilaris and HIV infection: a part of the spectrum of

HIV-associated follicular syndrome, British Journal of Dermatology, 133, 818

(1995).

P.R. Cohen and J.H. Prystowsky, Pityriasis rubra pilaris: a review of

diagnosis and treatment, Journal of the American Academy of Dermatology, 20,

801 (1989).

R Yaniv, A Barzilai, and H Trau, Pityriasis rubra pilaris Exacerbated by

Ultraviolet B Phototherapy, Dermatology, 189, 313 (1994).

R.J. van Dooren-Greebe and P.C.M. van de Kerkhof, Extensive extraspinal

hyperstoses after long-term oral retinoid treatment in a patient with

pityriasis rubra pilaris, Journal of the American Academy of Dermatology,

32, 322 (1995).

S.L. Vanderhooft, J.S. Francis, K.A. Holbrook, B.A. Dale, and P. Fleckman,

Familial Pityriasis Rubra Pilaris, Archives of Dermatology, 131, 448 (1995).

Links

Picture of PRP on dorsal side of the hands

Information on PRP and a support group for patients

Another online support group

Article reviews on pityriasis rubra pilaris

This page by Jody A. , December 1998

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January 17, 2003