Atrophoderma vermiculata

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ORPHA:79100OMIM:209700L66.4
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Overview

Atrophoderma vermiculata (also known as folliculitis ulerythematosa reticulata, or honeycomb atrophy) is a rare skin condition classified among the keratosis pilaris atrophicans group of disorders. It primarily affects the cheeks, presenting as small, worm-like (vermiculate) pits and depressions in the skin that create a characteristic honeycomb or reticulated pattern of atrophy. The condition typically begins in childhood, often between ages 5 and 12, and results from inflammation and subsequent atrophy of hair follicles in the affected areas. The skin may initially show small keratotic follicular papules with surrounding erythema before progressing to the distinctive pitted scarring. The condition predominantly affects the facial skin, particularly the cheeks, though it can occasionally extend to the preauricular areas, forehead, and ears. The pitted scars are usually symmetric and permanent. Atrophoderma vermiculata can occur as an isolated finding or may be associated with other conditions, including keratosis pilaris on the limbs and trunk. It has been described in association with certain syndromes, and some familial cases suggest a genetic component. There is no definitive cure for atrophoderma vermiculata. Treatment is largely symptomatic and cosmetic. Topical retinoids, keratolytic agents, and emollients have been used with limited success to manage the keratotic component. Procedural interventions such as dermabrasion, laser resurfacing (including fractional CO2 laser and erbium YAG laser), and chemical peels have been attempted to improve the appearance of established atrophic scars, with variable results. Early treatment aimed at reducing follicular inflammation may help limit progression of scarring.

Also known as:

Clinical phenotype terms— hover any for plain English:

Abnormal cheek morphologyHP:0004426Hypoplastic pilosebaceous unitsHP:0007515Abnormal epidermal morphologyHP:0011124Skin pitHP:0100276Abnormality of the chinHP:0000306Follicular hyperkeratosisHP:0007502Hyperkeratotic papuleHP:0045059Periauricular skin pitsHP:0100277Abnormal forehead morphologyHP:0000290NeurofibromaHP:0001067Heart blockHP:0012722
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Atrophoderma vermiculata.

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No actively recruiting trials found for Atrophoderma vermiculata at this time.

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No specialists are currently listed for Atrophoderma vermiculata.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 Atrophoderma vermiculata.

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Community

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Common questions about Atrophoderma vermiculata

What is Atrophoderma vermiculata?

Atrophoderma vermiculata (also known as folliculitis ulerythematosa reticulata, or honeycomb atrophy) is a rare skin condition classified among the keratosis pilaris atrophicans group of disorders. It primarily affects the cheeks, presenting as small, worm-like (vermiculate) pits and depressions in the skin that create a characteristic honeycomb or reticulated pattern of atrophy. The condition typically begins in childhood, often between ages 5 and 12, and results from inflammation and subsequent atrophy of hair follicles in the affected areas. The skin may initially show small keratotic folli

At what age does Atrophoderma vermiculata typically begin?

Typical onset of Atrophoderma vermiculata is childhood. Age of onset can vary across affected individuals.