Pityriasis Rubra Pilaris (Devergie's Disease) ICD-10: L44.0

Rare inflammatory skin disease

Often searched as: orange red rash all over body, rash that looks like psoriasis but isn't, rough bumpy rash on skin, red skin with islands of normal skin, skin turning orange red, thick hard skin on palms feet, full body red rash rare, skin rash that spreads everywhere…

Clinical urgency level

lowmediumhigh

Systems Affected

skinnailspalms and soles

Severity Levels

mild

Localized or limited follicular papules and plaques, stable, not affecting daily function — manageable with topical retinoids or emollients.

moderate

Widespread orange-red plaques on trunk and limbs with palmoplantar keratoderma, significant cosmetic and functional impact — requires systemic treatment.

severe

Erythroderma (near-total skin redness and scaling covering 90%+ of body surface), with pain, skin tightness, nail changes, and major quality-of-life impairment — requires urgent specialist care.

Red Flags

Rapid spread of reddish-orange skin over most of the body (possible erythroderma)
Skin becoming very tight, painful, or cracking — especially on hands and feet
High fever or chills accompanying widespread skin redness (infection risk in erythroderma)
Difficulty walking due to thickened, painful soles
Nail changes (thickening, ridging, crumbling) alongside skin rash
Skin rash not responding to any treatment after several weeks

Clinical Presentation

Pityriasis rubra pilaris — characteristic orange-red plaques with islands of sparing on the trunk

Source: Wikimedia Commons (CC BY-SA 3.0 )

PRP palmoplantar keratoderma — thickened, orange-tinted palms

View gallery on DermNet NZ (image gallery reference)

When to See a Doctor

See a dermatologist if you develop a spreading orange-red rash with follicular bumps, particularly if your palms and soles are also thickening. PRP can mimic psoriasis and requires specialist evaluation for accurate diagnosis and treatment planning. Seek urgent care if the rash covers most of your body or is accompanied by fever.

Differential Diagnosis

Psoriasis — the most common mimicker, especially plaque and erythrodermic variants
Seborrheic dermatitis
Ichthyosis (genetic scaling disorders)
Erythrodermic drug reaction
Cutaneous T-cell lymphoma (Sézary syndrome)
Dermatomyositis
Reactive arthritis (formerly Reiter syndrome)

Comorbidities

Nail dystrophy (common in PRP)
HIV infection (associated with adult-onset PRP in some cases)
Internal malignancy (rare association with paraneoplastic PRP)
Depression and anxiety (chronic visible skin disease)
Secondary skin infections in erythrodermic cases

Prognosis

PRP follows an unpredictable course. The classic adult form (Type I) often remits spontaneously within 1–3 years in about 80% of patients, but this can be a slow and difficult period. Juvenile-onset forms (Type III) also tend to resolve, while circumscribed juvenile forms (Type IV) may persist. Treatment options include oral retinoids (acitretin, isotretinoin), methotrexate, and increasingly biologics (TNF inhibitors, IL-17/23 inhibitors). Response to treatment is variable and unpredictable, making management challenging.

Detailed Overview

Causes, Symptoms, and When to Take Action Pityriasis rubra pilaris (PRP), also called Devergie's disease, is a rare skin disorder characterized by reddish-orange plaques and follicular papules, often with distinct patterns. It may affect both children and adults, with variable severity. How It Looks Texture: Follicular papules that coalesce into well-demarcated plaques; non-adherent flaking or fine scale Color: Reddish-orange on lighter skin tones; may appear hyperpigmented or violaceous on more pigmented skin Shape: Plaques are often sharply demarcated, may include "islands of sparing" (areas of unaffected skin surrounded by lesions) Location: Frequently involves palms and soles (palmoplantar keratoderma), follicles, and generalized skin areas; may progress to widespread involvement in severe cases Why Does It Appear? The cause is unknown; thought to involve both genetic and environmental factors Subtypes vary among individuals; some forms begin in early childhood, others in adulthood Some cases are associated with immune system differences or triggering events Should You Be Concerned? PRP is not malignant, but in severe forms it can significantly impact quality of life: There may be intense itching, burning, skin tightness, pain, or discomfort In widespread disease, skin reddening and scale may cover large body areas Nail changes (thickening, ridging) and complications like fissures in palms/soles may occur Consult a healthcare provider if symptoms are extensive, painful, or interfering with daily activities. Can You Prevent It? There is no known prevention. However: Avoiding skin trauma and maintaining skin care may help reduce worsening Monitoring for changes, especially after early signs, to manage symptoms promptly 👉 How Piel AI Can Help Piel AI allows you to track the appearance of lesions over time via regular imaging. While not diagnostic, it helps you notice progression, evaluate severity, and prepare information to share with a healthcare professional. Summary Pityriasis rubra pilaris (Devergie) is a rare inflammatory skin condition with reddish-orange plaques and distinct follicular bumps. It can be mild or severe; severe forms may affect large skin areas and cause discomfort. The cause is not fully understood; genetic and environmental factors play a role. Early observation, symptom tracking, and supportive care are key to managing impact.

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