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Porokeratotic eccrine ostial and dermal duct nevus - Skin in Adult
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Porokeratotic eccrine ostial and dermal duct nevus - Skin in Adult

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Contributors: Amy Fox MD, David Dasher MD, Jeffrey D. Bernhard MD, Belinda Tan MD, PhD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare congenital nevus of eccrine origin. Although considered a sporadic disorder, familial cases of PEODDN have recently been reported. These eccrine hamartomas are most often found on the extremities; palms and soles are the most commonly reported sites.

PEODDN typically appears within the first few years of life and is asymptomatic, although pruritus has been reported. Classically, it is described as linearly distributed keratotic papules and plaques with a comedo-like appearance. Verrucous lesions may also be observed. Histology is characteristic, which can help distinguish PEODDN from similarly appearing porokeratotic disorders. Well-formed cornoid lamellae are observed overlying dilated and hyperplastic eccrine acrosyringia. A cornoid lamella is a narrow vertical column of parakeratotic cells pointing into the stratum corneum. It is characteristic of a number of forms of porokeratosis and gives rise to the clinical appearance of a thread-like border, often with a Great Wall of China character.

Although extracutaneous disease is uncommon, squamous cell carcinoma arising within PEODDN has been reported, and thus clinical follow up is advisable.

Codes

ICD10CM:
D23.9 – Other benign neoplasm of skin, unspecified

SNOMEDCT:
239118007 – Porokeratotic eccrine ostial and dermal duct nevus

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Nevus comedonicus – Can have similar linear presentation with comedone-like structures, but histology will not show coronoid lamella.
  • Porokeratotic dermatoses (punctate porokeratosis, linear porokeratosis, and porokeratosis of Mibelli) – All of these conditions demonstrate coronoid lamella on histology; however, only PEODDN will have this isolated to acrosyringium.
  • Epidermal nevus (including verrucous) – Can have linear or verrucous appearance, as has been reported with PEODDN, but again histology will lack coronoid lamella.
  • Inflammatory linear verrucous epidermal nevus (ILVEN) – Appearance can be linear, similar to PEODDN, but ILVEN is frequently symptomatic and clinically appears more inflammatory. Histology is confirmatory.

Best Tests

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Therapy

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References

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Last Updated: 11/21/2013
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Porokeratotic eccrine ostial and dermal duct nevus - Skin in Adult
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Porokeratotic eccrine ostial and dermal duct nevus : Plantar foot, Tiny papules, Scaly papules
Clinical image of Porokeratotic eccrine ostial and dermal duct nevus
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