ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (4)
Atrophoderma vermiculatum in Child
Other Resources UpToDate PubMed

Atrophoderma vermiculatum in Child

Print Images (4)
Contributors: Saagar Jadeja, Susan Burgin MD
Other Resources UpToDate PubMed


Atrophoderma vermiculatum (AV), also known as folliculitis ulerythematosa reticulata, is a rare variant of keratosis pilaris atrophicans. Patients with this follicular dermatosis typically present in childhood with a reticular pattern of atrophic pits on the cheeks, preauricular region, and forehead. The condition progresses gradually. AV is sporadic in most cases, but there have been reports of autosomal dominant inheritance.

In a review of 25 cases of AV, the mean age of onset was 10 years, with ages ranging from 3 months to 25 years. One case of congenital AV was noted. Males represented 63% of all cases that were reviewed.

The exact etiology of AV is unknown. One hypothesis is that keratinocytes mediate release of inflammatory cytokines such as transforming growth factor beta (TGF-β) in response to follicular plug formation. This process can result in fibrosis and the characteristic "honeycomb" atrophic lesions seen in AV.

AV is known to occur both as an isolated skin condition and in association with certain genetic disorders. AV has been reported in association with Down syndrome, Ehlers-Danlos syndrome, Marfan syndrome, Melkersson-Rosenthal syndrome, Rombo syndrome, neurofibromatosis, TGFBR2-related Loeys-Dietz syndrome, congenital heart block and atrial septal defect with Eisenmenger complex, and unilateral AV with ipsilateral congenital cataract.

For more information, see OMIM.


L66.4 – Folliculitis ulerythematosa reticulata

2736005 – Atrophoderma vermiculatum

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

  • Post-acne scarring – History of acne in affected area. Generally develops during puberty. 
  • Depressed scars after resolution of chickenpox.
  • Nevus comedonicus – Characterized by presence of large comedones.
  • Lipoid proteinosis – Patients often have mucosal involvement. Atrophic lesions are the result of vesicles, bullae, and pustules that crust and scar over. 
  • Erythropoietic protoporphyria – Edema and possible erythema of sun-exposed skin with associated small, discrete scars.

Best Tests

Subscription Required

Management Pearls

Subscription Required


Subscription Required


Subscription Required

Last Reviewed: 05/24/2017
Last Updated: 07/26/2017
Copyright © 2020 VisualDx®. All rights reserved.
Atrophoderma vermiculatum in Child
Print 4 Images
View all Images (4)
(with subscription)
Atrophoderma vermiculatum : Erythema, Pits, Cheeks
Copyright © 2020 VisualDx®. All rights reserved.