Pityriasis rubra pilaris (adult type) L44.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Barbed lichen; Besnier braid; Besnier's disease; Devergie's disease; PRP

History
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Tarral, 1835; Devergie, 1856; Besnier, 1889

Definition
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Rare, predominantly acquired, chronic inflammatory cornification disorder with seasonally variable (often improved in winter), large-area keratotic plaques, follicular hyperkeratosis and extensive palmo-plantar hyperkeratosis associated with skin disease of unknown etiology. Although the skin lesions phenotypically show a psoriasiform pattern, there is no relationship.

Classification
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According to Griffith, there are 5 types to which an HIV-associated form is added:

  1. Classic adult type (approx. 50%): 80% healing after 3 years.
  2. Atypical adult type (approx. 5%): Eminently chronic course.
  3. Classic juvenile type (approx. 10%): Healing mostly after 1 year.
  4. Circumscribed juvenile type (approx. 25%): Different healing rates (1/3 in 3 years).
  5. Atypical juvenile type (about 5%): Chronic course.
  6. HIV-associated form (< 5%).

Occurrence/Epidemiology
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Incidence: 0.2/100,000 inhabitants.

Etiopathogenesis
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Unknown. It's a hyperproliferative keratinization disorder with increased turnover rates. Mostly sporadically occurring. Type 5 (hereditary, atypical juvenile form) is inherited autosomal dominant. This form is probably caused by a gain-of-function mutation on CARD14 (Lwin et al 2018)

Furthermore, a lack of retinol-binding protein and disturbances in vitamin A metabolism have been described.

Manifestation
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Initial manifestation is possible at any age. Disease peaks are in children in the 1st decade of life and in adults in the 5th decade of life. More frequent after serious illness or accidents and in patients with HIV infection. S.u. Immune reconstruction syndrome.

Localization
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  • Types 1-3: Mainly localized on the back of the fingers and hands as well as on the sides of the extremities (e.g. back of the upper arm, sides of the fingers), on the capillitium, in the face (especially eyebrows, nasolabial folds), more rarely also on the trunk. Bending-sided (axillary) localization is also detectable.
  • Type 4: Located mainly on the knees and elbows.
  • Type 5: Located mainly on the hands and feet.

Clinical features
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Initial symptoms: Densely packed, follicular, keratotic, red to reddish-brownish (orange-red), pinhead-sized, pointed papules with central, conical or crater-shaped dropping keratosis. Pronounced especially on the back of the fingers and hands as well as the extensor sides of the extremities.

Confluence leads to the formation of large, reddish-brownish, velvety erythema and plaques that cover entire areas of the body, in which the initially existing follicular character is lost. Included islands of normal skin ( nappes claires) are typical. Red, diffuse hyperkeratoses on palmae and plantae as well as on the back of the hand and extensor sides of the fingers. Formation of painful rhagades. The nails may be longitudinally furrowed and also thickened. Hair loss in the later stages and usually very strong, plaster-like dandruff. Ectropion. The classic forms often progress to erythroderma. Often worsening in summer (important DD to psoriasis vulgaris) and marked improvement (until healing) in the cold seasons.

Pityriasis rubra pilaris type 2 and 3: Types 2 and 3 are morphologically not different from the classic adult type.

Histology
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Irregular acanthosis without thinning of the epidermis above the papillae. Severe hyperkeratosis with alternating islands of parakeratosis(checkerboard pattern). The follicles are often enlarged like ampoules and filled with horn masses. In the dermis there is a slight to moderate lympho-histiocytic infiltrate. Focal exocytosis with spongiosis is possible.

Differential diagnosis
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  • Psoriasis vulgaris: Important differential diagnosis. Follicular hyperkeratosis ("grater skin") and nappes claires are unusual for psoriasis. The uniform palmo-plantar hyperkeratoses are not encountered in psoriasis.
  • Seborrhoeic eczema: Identical statement to psoriasis vulgaris.
  • Erythrokeratodermia figurata variabilis: Migrating (variable), bizarrely configured, annular or polycyclic, scaly papules and plaques with alternating clinical expressivity and acuteity (RPR relatively constant in location). Follicular hyperkeratoses are absent. Urticarial skin changes are also possible (always absent in pityriasis rubra pilaris). Older lesions present as large red or reddish-brown plaques, mostly borderline.
  • Lichen planus follicularis: Histology and possibly immunohistology are conclusive.

General therapy
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The therapy of pityriasis rubra pilaris is laborious and must be carried out with "long breath". Spontaneous remissions can be observed in individual cases, but are by no means the rule. All therapeutic measures should be aimed at improving the quality of life of those affected.

External therapy
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  • Nurturing ointment therapy: In the case of eczematised or irritated skin, mild glucocorticoids such as 0.5-1.0% hydrocortisone cream or lotio (e.g. hydrogalene, R123 ) have a short-term positive effect. Otherwise, nurturing, keratolytic or anti-inflammatory ointments should be applied on the still moist skin immediately after bath therapy and other washing procedures according to the clinical picture. This allows a lasting storage of moisture. Good effects are achieved with saline or propylene glycol containing ointments R105. The correct treatment has to be tested in each individual case.
  • Skin care and cleansing: Basic principle: As little degreasing of the skin as possible! As little irritating cleansing agents as possible, i.e. sparing use of soaps or syndets. No use of liquid soaps. Mild cleansing of the body skin with hydrophilic (water-miscible) body oils. Hereby a sufficient cleansing effect and at the same time a refatting care is achieved. Instead of hydrophilic oils also O/W emulsions may be used (e.g. Abitima Lotion, Excipial U Hydrolotio, Sebamed Lotion). Briefly shower the skin, apply and distribute the emulsion on the moist skin, shower again briefly. The remaining emulsion film is not perceived as unpleasant.
  • Baths: are important and recommended for skin care. Different bath additives depending on the skin condition (see table 1). The patient should be familiarized with the therapy modalities and be able to prepare the baths himself, e.g. oil baths as"Cleopatra bath" or bran baths.
  • In phases of clinical improvement only external skin care measures are necessary, e.g. Ungt. emulsif. aq., Linola milk, ash base cream.
  • Clothing: Wear non-irritating body wash. Smoothly woven fabrics of cotton or linen are most suitable. Unsuitable are fabrics of animal wool. Plastic fabrics are equally unsuitable, but materials made of viscose are generally tolerated.

Radiation therapy
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UV irradiation proves to be beneficial in individual cases. UVB treatment or PUVA therapy are recommended for adults.

Internal therapy
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  • Retinoids (first-line therapy): Isotretinoin (e.g. isotretinoin-ratiopharm; acne normin) 0.5-1.0 mg/kg bw p.o.
  • Alternatively: Acitretin (neotigason), initially 0.5-0.7 mg/kg bw/day; as maintenance dose aim for 10 mg/day.
  • Alternatively, in case of resistance to therapy and severe forms of the disease: success is described with methotrexate 10-25 mg/week p.o. Effects can be expected after 4-6 months. Maintenance therapy: 2.5-5.0 mg/week p.o. In very severe cases a combination of acitretin 0.5-0.7 mg/kg bw/day p.o. with methotrexate 15.0-30.0 mg/week p.o. can be tried.
  • Alternatively, Ciclosporin was used in adults and children (3.0 mg/kg bw/day). The results are controversial. Other immunosuppressive drugs such as azathioprine (e.g. Imurek) were not very successful.
  • Alternative: Own positive experiences with fumarates ( off-label use), which have been confirmed by other working groups, exist.
  • Alternative: Sporadic success has been described with extracorporeal photopheresis.
  • Alternative: In individual cases, therapy success could be achieved with Infliximab (5 mg/kg bw i.v.), Adalimumab and Etanercept and Ustekinumab (in usual dosage) ( Off-Label-Use). A combination with Low-dose-MTX is possible in severe cases.

Progression/forecast
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Unpredictable, mostly eminently chronic course. Spontaneous remissions are possible.

Tables
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Balneotherapy for pityriasis rubra pilaris

skin condition

Bathroom

Example preparations

Frequency

Dry

3% salt bath

300 mg table salt to 1 full bath

2-4 times a week 10-20 minutes

oil baths

Balneum Hermal F, oil bath Cordes, linola grease N oil bath

Cleopatra Bath

1(-2) cups of milk + 1(-2) tbs olive oil to 1 full bath

Eczematous, inflammatory

Bran Bath

Pottery bran bath

Valerian Bath

Silvapin Valerian oil bath

Polidocanol oil baths

Balneum Hermal plus

Melissa Bath

Kneipp good night bath

Note(s)
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Good results can be achieved with thalassotherapeutic healing methods. Dermatological clinics at the North and Baltic Sea are recommended.

Literature
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  1. Albert MR (1999) Pityriasis rubra pilaris. Int J Dermatol 38: 1-11
  2. Auffret N et al (1992) Pityriasis rubra pilaris in a patient with human immunodeficiency virus infection. J Am Acad Dermatol 27: 260-261
  3. Behr FD et al (2002) Atypical pityriasis rubra pilaris associated with arthropathy and osteoporosis: a case report with 15-year follow-up. Pediatric dermatol 19: 46-51
  4. Besnier E (1889) Observations pour servis a l'histoire clinique de pityriasis rubra pilaris. Annales de Dermatologie et de Syphilographie (Paris) 11: 253-287, 398-427, 485-544
  5. Coras B et al (2005) Fumaric acid esters therapy: a new reatment modality in pityriasis rubra pilaris? Br J Dermatol 152: 386-403
  6. Devergie MGA (1856) Pityriasis pilaris, maladie de la peau non décrite par les dermatologistes Gazette hebdomadaire de médecine et de surgery (Paris) 3: 197-201
  7. Gemmeke A et al (2010) Pityriasis rubra pilaris - a retrospective monocentric analysis over 8 years. JDDG 8: 439-445
  8. Haenssle H et al (2004) Extracorporeal photochemotherapy for the treatment of exanthematic pityriasis rubra pilaris. Clin Exper Dermatol 29: 244-246
  9. Hashimoto K et al (1999) Pityriasis rubra pilaris with acantholysis and lichenoid histology. At J Dermatopathol 21: 491-493
  10. Kaskel P et al (2001) Phototesting and phototherapy in pityriasis rubra pilaris. Br J Dermatol 144: 430
  11. Kaposi M (1895) Lichen ruber acuminatus and Lichen ruber planus. Archive for Dermatology and Syphilis (Prague) 31: 1-32
  12. Lwin SM et al2018) Benefi. (cial effect of ustekinumab in familial pityriasis rubra pilaris with a newmissense
    mutation in CARD14. Br J Dermatol 178:969-972.

  13. Mohrenschlager M et al (2002) Further clinical evidence for involvement of bacterial superantigens in juvenile pityriasis rubra pilaris (PRP): report of two new cases. Pediatric dermatol 19: 569

  14. Nakafusa J et al (2002) Pityriasis rubra pilaris in association with polyarthritis. Dermatology 205: 298-300
  15. Tarral C (1835) General Psoriasis. Desquamation from the part covered with hair. In: Rayer P (editor) A theoretical and practical treatise on the diseases of the skin. Bailliere (London): 648-649
  16. Terheyden P et al (2001) Erythroderma after corticosteroid withdrawal in papulosquamous dermatosis in children. Diagnosis: pityriasis rubra pilaris, classic juvenile type. Dermatologist 52: 1058-1061
  17. Wetzig T et al (2003) Juvenile pityriasis rubra pilaris: successfull treatment with cyclosporine. Br J Dermatol 149: 202-203

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Last updated on: 29.10.2020