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Pityriasis Rubra Pilaris

Pityriasis rubra pilaris, sometimes also referred to as pityriasis rubra, Besnier's or Devergie's disease, is a rare, chronic skin disease of mainly unknown etiology. Small papules and scaly plaques are characteristic for pityriasis rubra pilaris and the disease may progress to erythroderma.


Presentation

While sporadic PRP is characterized by an acute onset, familial PRP develops gradually. Patients usually note first symptoms on their scalp and in their face. Papules are not present at the very beginning of the disease, but skin irritation, redness and scales may be observed. These symptoms usually spread from the head to thorax, abdomen and limbs. In the course of the disease, scaly plaques of orange-reddish or salmon color form. They are typically delimited by sharp borders. Redness and thickening of palms and soles indicates palmoplantar keratoderma. Hyperkeratotic areas may have an orange hue and tend to fissure, what causes pain in the affected individual. The Elbows, wrists and dorsal surfaces of proximal phalanges often show signs of follicular hyperkeratosis. Due to their overall aspect, these regions of the skin are sometimes compared to a nutmeg grater. Eventually, the whole body may be affected, but the aforementioned sparing islands are also common. Of note, pruritus is not characteristic for PRP but may be observed in the initial stages of the disease.

Eyes, mucous membranes and nails may also be affected by PRP. With regards to the eyes, the lower lid may turn outward and form an ectropion. Excess dryness of the eye and blurred vision have also been related with PRP. Lacy whitish or grayish papules and plaques affecting the buccal mucosa may cause mucous membrane irritation and pain. Also, generalized erythema and possible mucosal erosions may be detected in the oral cavity [7]. Nail color frequently changes towards a yellow-brownish shade. Longitudinal ridges indicate nail formation problems and may be accompanied by subungual hyperkeratosis and thickened nail plates.

As has been mentioned before, five types of PRP are distinguished according to their clinical picture. They present as follows:

  • Classic adult PRP accounts for approximately half of all PRP cases. Presentation is as described above with follicular papules, scaling orange-reddish plaques and palmoplantar hyperkeratosis. Islands of sparing are frequently observed.
  • Atypical adult PRP skin lesions are rather ichthyosiform or eczematous. Alopecia is common.
  • Classic juvenile PRP resembles classic adult PRP but is characterized by an early onset, usually within the first two years of life.
  • Circumscribed juvenile PRP is the second most frequent form of PRP and is diagnosed approximately half as often as classic adult PRP. Onset occurs in pre-puberty. Skin lesions are typically limited to sharply demarcated areas where follicular hyperkeratosis is the dominating symptom. Elbows and knees display erythema.
  • Atypical juvenile PRP is often shown by those patients suffering from familial PRP. An early onset and a chronic course are typical. Symptoms comprise follicular and palmoplantar hyperkeratosis.

The HIV-associated form of PRP is usually characterized by nodulocystic and pustular acneiform lesions.

High Fever
  • We report here the case of a 38-year-old female with onset of classical PRP after a high fever. Virological studies indicated that the patient had primary systemic cytomegalovirus (CMV) infection and not HIV infection.[ncbi.nlm.nih.gov]
Pertussis
  • Pityriasis rubra pilaris occurring after vaccination with diphtheria-pertussis-tetanus and oral poliovirus vaccines. Indian J Dermatol Venereol Leprol. 2015 Nov-Dec. 81 (6):618-20. [Medline].[emedicine.com]
Hoarseness
  • Microlaryngoscopy was performed because of the patient's hoarseness, and this revealed leucoplakia on the left vocal cord. Histopathological examination led to the diagnosis of squamous cell carcinoma in situ.[ncbi.nlm.nih.gov]
Blurred Vision
  • Excess dryness of the eye and blurred vision have also been related with PRP. Lacy whitish or grayish papules and plaques affecting the buccal mucosa may cause mucous membrane irritation and pain.[symptoma.com]
Papule
  • The papules are about the size of a pin and topped by a horny plug A rare cutaneous disorder of unknown etiology characterized by the presence of reddish-orange scaly plaques, palmoplantar keratoderma, keratotic follicular papules, and confluent erythematous[icd9data.com]
  • PRP is a papulosquamous, hyperkeratotic disorder and patients typically present follicular papules, scaling plaques and palmoplantar hyperkeratosis.[symptoma.com]
  • Pityriasis rubra pilaris is a rare inflammatory disorder of the skin characterized by follicular keratotic papules coalescing into plaques, along with palmoplantar keratoderma.[ncbi.nlm.nih.gov]
  • Abstract Pityriasis rubra pilaris (PRP) is a rare skin condition which typically presents in adults as red-orange plaques with islands of sparing, perifollicular keratotic papules, waxy palmoplantar keratoderma, and erythema with fine, diffuse scale.[ncbi.nlm.nih.gov]
  • Pityriasis rubra pilaris (PRP) or Devergie's disease is a chronic and rare papulosquamous disorder of unknown etiology characterized by reddish orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules.[ncbi.nlm.nih.gov]
Follicular Hyperkeratosis
  • Pityriasis rubra pilaris (PRP) is a chronic papulosquamous eruption of the skin characterized by follicular hyperkeratosis, salmon pink scaly plaques with islands of unaffected skin, and palmoplantar keratoderma.[ncbi.nlm.nih.gov]
  • All patients promptly showed a decrease in erythema, follicular hyperkeratosis and scaling.[ncbi.nlm.nih.gov]
  • The typical features of pityriasis rubra pilaris include follicular hyperkeratosis and a reddish orange, scaling dermatitis with islands of normal skin.[accessmedicine.mhmedical.com]
  • Cutis . 2012 November;90(5):244-247 Pityriasis rubra pilaris (PRP) is a chronic papulosquamous eruption of the skin characterized by follicular hyperkeratosis, salmon pink scaly plaques with islands of unaffected skin, and palmoplantar keratoderma.[mdedge.com]
Eczema
  • Initial missed diagnoses include psoriasis, contact dermatitis, eczema/spongiotic dermatitis, seborrheic de... No published randomized controlled trials exist due to the rarity of this disorder.[5minuteconsult.com]
  • This is usually accompanied by eczema of some type. Classical juvenile onset PRP typically develops between 5 and 10, and follows an infection in most cases. This type of PRP usually resolves within 1 year.[ozarkderm.com]
  • National Eczema Association. Eczema Facts. Accessed August 7, 2017. 11. National Psoriasis Foundation. About the National Psoriasis Foundation. Accessed August 7, 2017. 12, Genetic and Rare Diseases Information Center. Pityriasis rubra pilaris.[rarediseases.org]
  • Very often PRP is mistaken for similar skin conditions such as psoriasis or eczema, and is only diagnosed when it doesn’t respond to standard treatment for those conditions.[skinsite.com]
Dry Skin
  • Emollients recommended by your doctor or specialist are a very important part of treatment to help dry skin and restore the barrier function of the skin. The emollient should be applied liberally.[britishskinfoundation.org.uk]
  • Terrasil Itch, Rash & Pain Relief's powerful all-natural formulation rapidly moisturizes dry skin to provides long-lasting relief and protect skin cracks. Customer Reviews* *Results may vary.[aidanceproducts.com]
Splinter Hemorrhage
  • Distal yellow-brown discoloration, subungual hyperkeratosis, nail plate thickening, and splinter hemorrhages indicate a diagnosis of type 1 pityriasis rubra pilaris rather than psoriasis, while onycholysis (particularly marginal), salmon patches, small[ncbi.nlm.nih.gov]
  • The patient then progressed to clinically resemble an acute adult onset PRP with erythroderma, (Figure 1) palmar plantar keratoderma and nails with thick, distal splinter hemorrhages.[austinpublishinggroup.com]
  • Nail changes are frequent and include nail plate thickening, yellow-brown discoloration, and splinter hemorrhages (Figure 6).[clinicaladvisor.com]
Scaly Scalp
  • She also had a scaly scalp, yellow thickening of the palms and soles, and subungual hyperkeratosis.[omim.org]
Facial Scar
  • Nine years after ART initiation, she remains well, with viral suppression and immune recovery, without PRP recurrence but with sparse hair regrowth and facial scarring.[ncbi.nlm.nih.gov]

Workup

There are no specific tests for PRP, thus diagnosis is based on the clinical picture and histological results [8]. Laboratory tests may, however, be applied to rule out potential differential diagnoses and to assess the overall condition of the patient in severe cases of PRP.

Skin biopsies should be analyzed for pathological alterations but will not reveal any pathognomonic findings. Similar to what has been said about laboratory tests, histopathological evaluation of tissue samples mainly aims at ruling out other pathologies. In PRP, alternating hyperkeratosis with ortho- and parakeratosis dominate the stratum corneum of the skin. Parakeratotic features may also be detected in the perifollicular region, the hair follicle is usually plugged. Hypergranulosis is often seen. Dermal papillae are abnormally narrow. Perivascular lymphocytic infiltration is limited. In some samples, acantholysis can be detected [9].

Staphylococcus Aureus
  • Cultures were positive in both eyes for Herpes simplex and in the left eye for Pseudomonas fluorescens and Staphylococcus aureus A microbiological cure was obtained with a combination of tectonic keratoplasty, fortified topical antibiotics, and systemic[ncbi.nlm.nih.gov]
  • Clinical course had been complicated by episodes of Staphylococcus aureus bacteremia, mucocutaneous candidiasis, and development of localized squamous cell carcinoma of the skin.[thedoctorsdoctor.com]
Pseudomonas
  • Cultures were positive in both eyes for Herpes simplex and in the left eye for Pseudomonas fluorescens and Staphylococcus aureus A microbiological cure was obtained with a combination of tectonic keratoplasty, fortified topical antibiotics, and systemic[ncbi.nlm.nih.gov]

Treatment

Possibly due to the low prevalence of the disease, no controlled trails have been carried out regarding PRP treatment.  Neither the European Medicines Agency nor the US Food and Drug Administration approved any drug for use in PRP. Thus, any given recommendations are based on empirical analyses. Effectivity and safety are unclear.

In order to treat PRP, topical and systemic medication is often combined. While maximal drug concentrations and thus effectivity may be higher if the skin is treated topically, this approach may be neither suitable nor sufficient for patients suffering from generalized PRP. It is, however, highly recommended for those with circumscribed and mild PRP. In this context, corticosteroids, keratolytics, calcipotriol as well as topical retinoids tretinoin and tazarotene may be utilized [10].

Systemic retinoids, i.e., synthetic derivatives of retinol, are the medication of choice for treating PRP [11]. If the response to such treatment is not satisfactory, methotrexate may be administered. Methotrexate is also first choice for PRP therapy in patients with contraindications for systemic retinoids [12]. Further therapeutic options may consist in immunomodulatory treatment with of TNF-α inhibitors, azathioprine or cyclosporine.

Some patients may benefit from phototherapy.

These treatments are usually not effective in HIV-associated PRP. Here, anti-retroviral treatment may yield an improvement of skin condition.

Prognosis

Prognosis of familial and sporadic PRP differ widely. While the former usually persists for life, that latter may be self-limiting in between a few years. It is not yet known which event triggers the spontaneous cure of PRP.

Also, there are differences regarding adult and juvenile, classical, circumscribed and atypical forms of PRP. Classic adult PRP has the best prognosis and 80% of these cases resolve spontaneously within three years. However, the disease may also persist for much longer periods of time [6]. In some cases, scarring alopecia remains after healing. As for atypical adult PRP, no rapid spontaneous cure should be expected since the disease often persists for decades. Classic juvenile PRP is generally self-limiting and resolves within a year, atypical juvenile PRP does not. Little is known about the long-term prognosis of circumscribed juvenile PRP.

Etiology

In general, hereditary and acquired PRP have to be distinguished.

With regards to the former, familial PRP is apparently inherited with an autosomal dominant trait, i.e. males and females may carry the affected chromosome with equal likelihood and will develop the disease even in heterozygous state. Recently, the CARD14 gene that encodes for caspase recruitment domain-containing protein 14 has been related with psoriasis and PRP [3]. CARD14 expression differs between distinct epidermal cell populations and is presumably altered in PRP patients. Other mutations may also trigger PRP, but have not yet been identified. A functional relation between CARD14 and possibly other genes and PRP has yet to be established.

The vast majority of PRP cases occurs sporadically and no specific causes can be identified. It is also not known whether sporadic PRP results from the same pathogenetic mechanisms that are triggered in patients suffering from familial PRP.

Epidemiology

PRP is considered a rare skin disease but precise data regarding incidence and prevalence cannot be presented. A study conducted in Great Britain revealed that about 1 in 5000 patients looking for dermatological advice present with PRP [1].

No preferences regarding distinct races have been determined. The male-to-female ration is presumably one to one [4].

Sex distribution
Age distribution

Pathophysiology

Little is known about PRP pathogenesis. More light may be shed on this topic if an association of familial PRP with specific gene mutations can be confirmed. Then, assessment of gene functions may help to understand which metabolic pathways are interrupted in PRP patients.

It has been speculated that vitamin metabolism is altered in PRP patients, particularly vitamin A metabolism. The fact that systemic retinoids alleviate symptoms associated with PRP may support this hypothesis. However, reliable data is not available and further research is required to confirm this theory.

Another hypothesis is about PRP being associated with an abnormal immune response that may be triggered by certain pathogens. Isolated case reports related PRP with previous streptococcal infections [5].

Prevention

There are no specific recommendations to prevent sporadic PRP.

Patients suffering from chronic, familial PRP may benefit from genetic counseling and  prenatal diagnosis. Also, affected parents may have genetic tests run on their newborn in order to better assess their risk for developing the disease. Similarly, family members may be tested to determine whether they are carrying the mutated gene.

Summary

Pityriasis rubra pilaris (PRP) is a rare, inflammatory dermatosis that may also be referred to as pityriasis rubra, Besnier's disease or Devergie's disease, whereby the latter names have been chosen in honor of two physicians who described this illness in the nineteenth century.

While some cases of PRP result from an unspecified genetic disorder and are thus deemed familial PRP, no direct causes can be detected for the majority of PRP cases. PRP is a papulosquamous, hyperkeratotic disorder and patients typically present follicular papules, scaling plaques and palmoplantar hyperkeratosis. Although these skin alterations may be found all over the body, patients frequently show apparently healthy skin areas which are known as islands of sparing. Transition from PRP to erythroderma is not clearly defined. Of note, the clinical picture presented by PRP patients may vary widely. Therefore, five different types of PRP are distinguished [1]:

  • Classic adult PRP
  • Atypical adult PRP
  • Classic juvenile PRP
  • Circumscribed juvenile PRP
  • Atypical juvenile PRP

Recently, another type of PRP has been described that has been associated to HIV infections [2].

Because little is known regarding etiology and pathogenesis of the disease, treatment is rather difficult. Different drugs have been used to treat PRP, but due to the low incidence of the disease reliable data is sparse. Most commonly, systemic retinoids as well as the antifolate drug methotrexate are administered. Also, TNF-α inhibitors have been proposed as potential therapeutic options.

Patient Information

Pityriasis rubra pilaris (PRP) is a rare, chronic skin disease.

Causes

While a small share of PRP cases results from genetic disorders, no precise cause could be identified for the sporadic form. More than 90% of all PRP cases are sporadic.

It has been speculated that alterations in vitamin A metabolism or previous streptococcal infections may predispose individuals for PRP, but these hypotheses could not yet be confirmed.

Symptoms

The most common symptoms of PRP are follicular papules, scaling orange-reddish plaques and palmoplantar hyperkeratosis, i.e., a thickening of the skin of hands and feet. Often, scalp and face are affected first, but the disease may spread to thorax, abdomen and limbs. Some people present generalized PRP but do show islands of sparing, areas of the skin that remain unaffected.

PRP may also provoke pathological alterations in eyes, mucous membranes and nails. In this context, the lower lid may turn outwards, dry eyes and blurred vision have been described. With regards to mucous membranes, the oral cavity is usually most severely affected. Papules, plaques and mucosal erosions may cause pain. Nails change color and become yellow-brownish. The nail plate thickens but presents longitudinal ridges.

There are different forms of PRP that may affect children, adolescents and adults and that manifest differently.

Diagnosis

There are no specific tests for PRP and the physician will base their diagnosis on symptoms observed during clinical examination and on histopathological analysis of tissue samples. In order to do so, skin biopsies have to be obtained.

The histopathological examination of skin biopsies requires a microscope and is necessary to distinguish PRP from other skin diseases, e.g., from psoriasis or pityriasis rosea. Treatment for each of these diseases is different.

Treatment

Treatment is symptomatic and consists in topical and systemic administration of vitamin A derivatives, methotrexate or immunomodulatory agents. Data regarding the effectivity of these therapeutic options is sparse and while there are certain standard treatments, therapy will need to be adjusted to the response of the individual patient.

Some forms of PRP, among them the most common, classical juvenile and adult forms, tend to resolve spontaneously within a few years.

References

Article

  1. Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol. 1980; 5(1):105-112.
  2. Auffret N, Quint L, Domart P, Dubertret L, Lecam JY, Binet O. Pityriasis rubra pilaris in a patient with human immunodeficiency virus infection. J Am Acad Dermatol. 1992; 27(2 Pt 1):260-261.
  3. Fuchs-Telem D, Sarig O, van Steensel MA, et al. Familial pityriasis rubra pilaris is caused by mutations in CARD14. Am J Hum Genet. 2012; 91(1):163-170.
  4. Allison DS, El-Azhary RA, Calobrisi SD, Dicken CH. Pityriasis rubra pilaris in children. J Am Acad Dermatol. 2002; 47(3):386-389.
  5. Mohrenschlager M, Abeck D. Further clinical evidence for involvement of bacterial superantigens in juvenile pityriasis rubra pilaris (PRP): report of two new cases. Pediatr Dermatol. 2002; 19(6):569.
  6. Abbott RA, Griffiths WA. Pityriasis rubra pilaris type 1 spontaneously resolving after 20 years. Clin Exp Dermatol. 2009; 34(3):378-379.
  7. Martinez Calixto LE, Suresh L, Matsumura E, Aguirre A, Radfar L. Oral pityriasis rubra pilaris. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006; 101(5):604-607.
  8. Cohen PR, Prystowsky JH. Pityriasis rubra pilaris: a review of diagnosis and treatment. J Am Acad Dermatol. 1989; 20(5 Pt 1):801-807.
  9. Magro CM, Crowson AN. The clinical and histomorphological features of pityriasis rubra pilaris. A comparative analysis with psoriasis. J Cutan Pathol. 1997; 24(7):416-424.
  10. Karimian-Teherani D, Parissa M, Tanew A. Response of juvenile circumscribed pityriasis rubra pilaris to topical tazarotene treatment. Pediatr Dermatol. 2008; 25(1):125-126.
  11. van Dooren-Greebe RJ, van de Kerkhof PC. Extensive extraspinal hyperostoses after long-term oral retinoid treatment in a patient with pityriasis rubra pilaris. J Am Acad Dermatol. 1995; 32(2 Pt 2):322-325.
  12. Durairaj VD, Horsley MB. Resolution of pityriasis rubra pilaris-induced cicatricial ectropion with systemic low-dose methotrexate. Am J Ophthalmol. 2007; 143(4):709-710.

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Last updated: 2019-07-11 20:38