Atrophodermia vermiculata

Overview

Atrophoderma Vermiculata (AV) is a rare, inherited skin condition characterized by follicular atrophy that primarily affects the cheeks during childhood. It results in small, pit-like depressions in a reticulated or honeycomb-like pattern, giving the skin a worm-eaten or "vermiculated" appearance. The condition is generally benign but may cause cosmetic concern due to its facial involvement.

AV is often considered part of a group of disorders called keratosis pilaris atrophicans, which also includes keratosis pilaris atrophicans faciei and keratosis follicularis spinulosa decalvans.

Causes

Atrophoderma Vermiculata is typically inherited in an autosomal dominant pattern, though sporadic cases can occur. The exact genetic defect is not fully understood, but it is believed to involve abnormal keratinization of hair follicles leading to inflammation, follicular plugging, and eventual atrophy of surrounding skin.

There is no known environmental trigger, and the condition usually manifests in early childhood regardless of external factors.

Symptoms

Symptoms of AV usually begin in the first or second decade of life and primarily involve the facial skin:

• Symmetric, small, pitted scars on the cheeks, temples, and sometimes forehead

• Reticulate (net-like) or honeycomb pattern of atrophy

• Keratotic follicular papules may precede atrophy

• Smooth, non-inflammatory skin – lesions are not typically itchy or painful

• Occasional progression: In some cases, the condition may extend to other areas, such as the scalp, resulting in sparse hair growth or scarring alopecia (in severe variants)

The rest of the body is typically unaffected, and systemic symptoms are absent.

Diagnosis

Diagnosis of Atrophoderma Vermiculata is clinical and based on history and skin appearance. Key diagnostic steps include:

• Physical examination: Reveals characteristic pitted scarring in a reticulated pattern, usually on the cheeks

• Dermatoscopy: May assist in visualizing follicular plugging and skin atrophy

• Skin biopsy (optional): Shows follicular hyperkeratosis, epidermal atrophy, and perifollicular fibrosis

• Family history: Helpful to establish inherited nature in familial cases

AV must be distinguished from other conditions such as acne scarring, folliculitis, and other forms of keratosis pilaris.

Treatment

There is no definitive cure for AV. Treatment aims to improve cosmetic appearance and prevent further progression. Options include:

Topical Therapies:

• Topical retinoids (e.g., tretinoin): Promote cell turnover and reduce follicular plugging

• Keratolytic agents (e.g., salicylic acid, lactic acid, urea): Help smoothen the skin

• Moisturizers with emollients: Maintain skin hydration and barrier function

Procedural Treatments:

• Laser therapy (e.g., fractional CO₂ laser): May improve the texture and appearance of scarring

• Dermabrasion or microneedling: Occasionally used for cosmetic improvement

Other Supportive Measures:

• Sun protection: Essential to reduce pigmentation and photoaging of affected areas

• Psychological support: May be helpful for adolescents concerned about facial appearance

Prognosis

Atrophoderma Vermiculata is a non-progressive and non-life-threatening condition. The skin changes typically stabilize after adolescence. Key prognostic points include:

• Permanent cosmetic changes: Atrophy and scarring do not usually resolve completely

• Stable course: The condition often stops progressing in early adulthood

• Normal life expectancy and health: No systemic involvement or internal organ effect

With consistent skincare and possible cosmetic interventions, individuals can manage the visible effects of the condition and maintain good self-esteem and quality of life.