Late-onset focal dermal elastosis

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Overview

Late-onset focal dermal elastosis is a rare acquired skin disorder characterized by the abnormal accumulation of elastic fibers in the dermis, typically presenting in middle-aged to elderly adults. The condition manifests as multiple small, yellowish or skin-colored papules that are often distributed symmetrically on the trunk, particularly the back, and sometimes on the extremities. These papules may coalesce into plaques and can have a cobblestone-like or peau d'orange (orange peel) appearance. The condition primarily affects the skin and is generally considered a benign dermatosis, though it can be cosmetically concerning for patients. The exact cause of late-onset focal dermal elastosis remains unclear. It is thought to be an acquired condition rather than an inherited one, and it is not associated with systemic connective tissue disorders. Histopathological examination reveals increased and fragmented elastic fibers in the mid and upper dermis, which distinguishes it from other elastotic conditions such as solar elastosis or pseudoxanthoma elasticum. The condition must be differentiated from other papular dermatoses and disorders of elastic tissue through clinical and histological evaluation. There is currently no established curative treatment for late-onset focal dermal elastosis. Management is primarily supportive and cosmetic. Since the condition is benign and asymptomatic in most cases, reassurance and observation may be sufficient. Some reports have described attempts at treatment with topical retinoids or other modalities, but no consistently effective therapy has been identified. The rarity of the condition limits the availability of large-scale clinical studies to guide treatment decisions.

Also known as:

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Late onset

Begins later in life, typically after age 50

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Late-onset focal dermal elastosis.

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No actively recruiting trials found for Late-onset focal dermal elastosis at this time.

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No specialists are currently listed for Late-onset focal dermal elastosis.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

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Common questions about Late-onset focal dermal elastosis

What is Late-onset focal dermal elastosis?

Late-onset focal dermal elastosis is a rare acquired skin disorder characterized by the abnormal accumulation of elastic fibers in the dermis, typically presenting in middle-aged to elderly adults. The condition manifests as multiple small, yellowish or skin-colored papules that are often distributed symmetrically on the trunk, particularly the back, and sometimes on the extremities. These papules may coalesce into plaques and can have a cobblestone-like or peau d'orange (orange peel) appearance. The condition primarily affects the skin and is generally considered a benign dermatosis, though i

How is Late-onset focal dermal elastosis inherited?

Late-onset focal dermal elastosis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Late-onset focal dermal elastosis typically begin?

Typical onset of Late-onset focal dermal elastosis is late onset. Age of onset can vary across affected individuals.