Co‐Occurrence of Agminated Lentigines, Café‐Au‐Lait Macules, and Vitiligo: A Case Report and Literature Review

Lentigines, café-au-lait macules (CALMs), and vitiligo are pigmentary disorders that seldom occur together in a single individual. Their co-occurrence may indicate underlying genetic syndromes requiring differential diagnosis. We report an 18-year-old male who developed CALMs at age 11, agminated lentigines at age 13, and vitiligo on the right chest six months thereafter. Comprehensive examinations excluded Peutz-Jeghers syndrome, Leopard syndrome, Carney complex, and Neurofibromatosis type 1. Eight-year follow-up-the longest reported for this combination-demonstrated a benign natural history: Stable vitiligo throughout and lentigines reaching a plateau by early adulthood. Negative autoimmune screening suggests localized immune dysregulation, supporting the Immunocompromised Cutaneous District hypothesis. A staged therapeutic approach prioritizing vitiligo stabilization is recommended.

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Publication Details

Journal
Clinical Case Reports
Published
2026-05-01
DOI
https://doi.org/10.1002/ccr3.72660
Primary Topic
melanin and skin pigmentation
Type
article
Field-Weighted Citation Impact
0.00

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article

Co‐Occurrence of Agminated Lentigines, Café‐Au‐Lait Macules, and Vitiligo: A Case Report and Literature Review

XinXin Lei, Bo Xie
Clinical Case Reports
melanin and skin pigmentation
article

Co‐Occurrence of Agminated Lentigines, Café‐Au‐Lait Macules, and Vitiligo: A Case Report and Literature Review

XinXin Lei, Bo Xie
article en

Abstract

Lentigines, café-au-lait macules (CALMs), and vitiligo are pigmentary disorders that seldom occur together in a single individual. Their co-occurrence may indicate underlying genetic syndromes requiring differential diagnosis. We report an 18-year-old male who developed CALMs at age 11, agminated lentigines at age 13, and vitiligo on the right chest six months thereafter. Comprehensive examinations excluded Peutz-Jeghers syndrome, Leopard syndrome, Carney complex, and Neurofibromatosis type 1. Eight-year follow-up-the longest reported for this combination-demonstrated a benign natural history: Stable vitiligo throughout and lentigines reaching a plateau by early adulthood. Negative autoimmune screening suggests localized immune dysregulation, supporting the Immunocompromised Cutaneous District hypothesis. A staged therapeutic approach prioritizing vitiligo stabilization is recommended.

Clinical Case ReportsVol. 14(5)
Zhejiang Chinese Medical University (CN), Third People's Hospital of Hangzhou (CN)
National Natural Science Foundation of China
Openalex Percentile: Top 11%
melanin and skin pigmentation
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