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Atrophoderma vermiculatum in Adult
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Atrophoderma vermiculatum in Adult

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Contributors: Saagar Jadeja, Susan Burgin MD
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Synopsis

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.

Codes

ICD10CM:
L66.4 – Folliculitis ulerythematosa reticulata

SNOMEDCT:
2736005 – Atrophoderma vermiculatum

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Diagnostic Pearls

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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

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Management Pearls

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Therapy

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References

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Last Reviewed: 05/24/2017
Last Updated: 07/26/2017
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Atrophoderma vermiculatum in Adult
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Atrophoderma vermiculatum : Erythema, Pits, Cheeks
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