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一例梅勒达病：掌跖角化病的罕见表现。

A Case of Mal De Meleda: The Rare Presentation of Palmoplantar Keratoderma Disease.

作者信息

Kalyan Vamsi, Suvvari Tarun K, Kandula Venkata Dinesh Kumar, Shanker Aparajeya, Matiashova Lolita

机构信息

Medicine and Surgery, Rangaraya Medical College, Kakinada, IND.

Medicine and Surgery, Dr. N.T.R University of Health Sciences, Vijayawada, IND.

出版信息

Cureus. 2021 Sep 17;13(9):e18061. doi: 10.7759/cureus.18061. eCollection 2021 Sep.

DOI:10.7759/cureus.18061
PMID:34692287
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8523366/
Abstract

Mal de Meleda (MDM) is a rare sub-type of palmoplantar keratoderma (PPK) disease. The primary symptoms of PPK are scleroatrophy, transient keratoderma, scleroatrophic erythema, pseudoainhum around the digits, and perioral erythema. MDM is a pathology with a difficult clinical course. This case study presents two cases of MDM in siblings born out of second-degree consanguinity. The presenting complaint was the peeling of the palmar skin since birth. Both patients were treated with acitretin orally (dose: 10 mg) for three months and tretinoin (topical) for two months. The prognosis was good after three months of treatment.

摘要

梅勒达病（MDM）是掌跖角化病（PPK）的一种罕见亚型。PPK的主要症状包括硬化萎缩、一过性角化病、硬化萎缩性红斑、手指周围假阿洪病以及口周红斑。MDM是一种临床病程复杂的疾病。本病例研究呈现了两例二级近亲结婚所生的患有MDM的兄弟姐妹。主要症状为自出生起手掌皮肤脱皮。两名患者均口服阿维A（剂量：10毫克）三个月，外用维甲酸两个月。治疗三个月后预后良好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eac6/8523366/c50c95cb5dfe/cureus-0013-00000018061-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eac6/8523366/5044bcf03c36/cureus-0013-00000018061-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eac6/8523366/d0274b6c9cb7/cureus-0013-00000018061-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eac6/8523366/c50c95cb5dfe/cureus-0013-00000018061-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eac6/8523366/5044bcf03c36/cureus-0013-00000018061-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eac6/8523366/d0274b6c9cb7/cureus-0013-00000018061-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eac6/8523366/c50c95cb5dfe/cureus-0013-00000018061-i03.jpg

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引用本文的文献

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J Neurosci. 2024 Jul 10;44(28):e0260232024. doi: 10.1523/JNEUROSCI.0260-23.2024.

本文引用的文献

1
Mal de Meleda: A Focused Review.遗传性少毛症：聚焦综述。
Am J Clin Dermatol. 2016 Feb;17(1):63-70. doi: 10.1007/s40257-015-0157-1.
2
Particular Mal de Meleda phenotypes in Tunisia and mutations founder effect in the Mediterranean region.突尼斯的特定 Meleda 病表型和地中海地区的突变创始效应。
Biomed Res Int. 2013;2013:206803. doi: 10.1155/2013/206803. Epub 2013 Sep 4.
3
Mal de meleda with lip involvement: a report of two cases.伴有唇部受累的梅勒达病：两例报告
Indian J Dermatol. 2012 Sep;57(5):390-3. doi: 10.4103/0019-5154.100497.
4
Keratoderma hereditaria mutilans (vohwinkel's syndrome).遗传性残毁性角化病（沃温克尔综合征）。
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Huriez syndrome.于里埃综合征
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Greither's disease.格赖特氏病
Indian J Dermatol Venereol Leprol. 2003 Jul-Aug;69(4):292-3.
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What syndrome is this? Greither syndrome.这是什么综合征？格赖特综合征。
Pediatr Dermatol. 2003 May-Jun;20(3):272-5. doi: 10.1046/j.1525-1470.2003.20319.x.