Livedo Reticularis With Perineural and Intraneural Mucin Deposition in a Child With AT-III Deficiency: A Case Suggestive of Neuropathia Mucinosa Cutanea?

This case expands the spectrum of intraneural mucin deposition by associating it with severe AT-III deficiency. Although fulfilling the morphologic description of NMC, the findings could also reflect a reactive change to ischemic nerve injury rather than a primary mucinous neuropathy. Further reports integrating clinical and histopathologic data are needed to clarify whether NMC represents a unique neurocutaneous disorder or a nonspecific reactive phenomenon.

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Publication Details

Journal
American Journal of Dermatopathology
Published
2025-12-04
DOI
https://doi.org/10.1097/dad.0000000000003185
Primary Topic
Skin and Cellular Biology Research
Type
article
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article

Livedo Reticularis With Perineural and Intraneural Mucin Deposition in a Child With AT-III Deficiency: A Case Suggestive of Neuropathia Mucinosa Cutanea?

Franco Rongioletti, Clemens Reifeltshammer
American Journal of Dermatopathology
Skin and Cellular Biology Research
article

Livedo Reticularis With Perineural and Intraneural Mucin Deposition in a Child With AT-III Deficiency: A Case Suggestive of Neuropathia Mucinosa Cutanea?

Franco Rongioletti, Clemens Reifeltshammer
article en

Abstract

This case expands the spectrum of intraneural mucin deposition by associating it with severe AT-III deficiency. Although fulfilling the morphologic description of NMC, the findings could also reflect a reactive change to ischemic nerve injury rather than a primary mucinous neuropathy. Further reports integrating clinical and histopathologic data are needed to clarify whether NMC represents a unique neurocutaneous disorder or a nonspecific reactive phenomenon.

American Journal of DermatopathologyVol. 48(4)
Vita-Salute San Raffaele University (IT), Klinik Hietzing (AT)
Good health and well-being
Openalex Percentile: Top 29%
Skin and Cellular Biology Research
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