Linear atrophoderma of Moulin

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Overview

Linear atrophoderma of Moulin (LAM) is an extremely rare dermatological condition first described by Moulin et al. in 1992. It is characterized by the development of band-like or linear areas of skin atrophy (atrophoderma) that follow the lines of Blaschko, which are developmental lines on the skin surface that reflect patterns of embryonic cell migration. The affected skin appears as slightly depressed, hyperpigmented bands, typically on the trunk or limbs. The condition primarily affects the skin and does not usually involve other organ systems. Linear atrophoderma of Moulin typically presents during childhood or adolescence. The atrophic bands are usually unilateral and may be brownish or violaceous in color. The lesions tend to remain stable over time and are generally asymptomatic, though some patients may report mild cosmetic concern. Histopathological examination may show dermal atrophy with reduced collagen and elastic fibers, along with basal layer hyperpigmentation. The condition is considered a form of cutaneous mosaicism, meaning it likely results from a postzygotic somatic mutation affecting a subset of skin cells during embryonic development. There is no established curative treatment for linear atrophoderma of Moulin. Management is primarily supportive and cosmetic. The condition is benign and does not progress to systemic disease. Because of its rarity, evidence for therapeutic interventions is limited to individual case reports. Patients are generally reassured about the benign nature of the condition, and follow-up is recommended to monitor for any changes in the lesions.

Clinical phenotype terms— hover any for plain English:

Linear hyperpigmentationHP:0007546Stiff skinHP:0030053SclerodermaHP:0100324
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Linear atrophoderma of Moulin.

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No actively recruiting trials found for Linear atrophoderma of Moulin at this time.

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No specialists are currently listed for Linear atrophoderma of Moulin.

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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

No travel grants are currently matched to Linear atrophoderma of Moulin.

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Community

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Common questions about Linear atrophoderma of Moulin

What is Linear atrophoderma of Moulin?

Linear atrophoderma of Moulin (LAM) is an extremely rare dermatological condition first described by Moulin et al. in 1992. It is characterized by the development of band-like or linear areas of skin atrophy (atrophoderma) that follow the lines of Blaschko, which are developmental lines on the skin surface that reflect patterns of embryonic cell migration. The affected skin appears as slightly depressed, hyperpigmented bands, typically on the trunk or limbs. The condition primarily affects the skin and does not usually involve other organ systems. Linear atrophoderma of Moulin typically prese

How is Linear atrophoderma of Moulin inherited?

Linear atrophoderma of Moulin follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Linear atrophoderma of Moulin typically begin?

Typical onset of Linear atrophoderma of Moulin is childhood. Age of onset can vary across affected individuals.