The genetics of hyperkeratosis lenticularis perstans

Bean, S. F.

doi:10.1001/archderm.106.1.72

Cited by 11 publications

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“…2 Removal of the spiny scale above each papule causes slight bleeding. [2][3][4][5][6][7][8][9][10][11][12][13] Although HLP has been considered an autosomal dominant condition, [14][15][16] most cases seem to occur sporadically and usually start in late adult life. 2,4,5,8,[10][11][16][17][18][19] HLP can be clearly distinguished from clinically similar disorders such as Kyrle's disease, porokeratosis and stucco keratosis by its characteristic histologic features.…”

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confidence: 99%

Histopathological Differences Between Early and Old Lesions of Hyperkeratosis Lenticularis Perstans (Flegel's Disease)

Andō

Hattori

Yamauchi

2006

The American Journal of Dermatopathology

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A case of hyperkeratosis lenticularis perstans (Flegel's disease) was studied histopathologically comparing early and old lesions. The age of the lesions were judged by both the patient's history and direct observation. The histopathologic and immunophenotypic features of a new lesion were essentially in accordance with previous findings. However, the old lesion had obviously different pathologic features. These included the absence of epidermal atrophy and infiltrate in the upper dermis under the lesion. Ultrastructural studies revealed that the presence of many normal-appearing membrane-coating granules in the keratinocytes of the old lesion, whereas the organelles were not found in the keratinocytes of the early lesion.

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confidence: 99%

Histopathological Differences Between Early and Old Lesions of Hyperkeratosis Lenticularis Perstans (Flegel's Disease)

Andō

Hattori

Yamauchi

2006

The American Journal of Dermatopathology

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“…Since his report, there has been no agreement as to whether this condition is a discrete entity or a KD variant [ 7 , 8 ]. Flegel disease (FD), as it is now known, is considered a genodermatosis with autosomal dominant inheritance, manifesting in the third to fourth decade of life, although it has been reported in patients as young as 13 years of age [ 9 ].…”

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A Case of Infantile Kyrle-Flegel Disease in a 6-Year-Old Yemeni Girl

Alshami

Mohana

2016

Case Rep Dermatol

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Kyrle disease (KD) and Flegel disease (FD) are rare variants of primary perforating dermatoses, characterized by transepidermal elimination of abnormal endogenous materials. We describe a 6-year-old Yemeni girl with a 2-year history of generalized asymptomatic, small, reddish-brown keratotic papules with a lenticular central keratotic plug. Although these features are synonymous with FD, the histology of a punch biopsy was consistent with KD. The patient was otherwise healthy, and no family members had a history of similar diseases. The patient was diagnosed with KD-FD, owing to the manifestation of features associated with both diseases.

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“…In familial cases, autosomal dominant transmission is shown. [3] Almost all reported cases are from white population. No sex predilection is seen.…”

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Hyperkeratosis lenticularis perstans: Case report of a rare entity

Patel

2013

Indian J Dermatol

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The genetics of hyperkeratosis lenticularis perstans

Cited by 11 publications

References 0 publications

Histopathological Differences Between Early and Old Lesions of Hyperkeratosis Lenticularis Perstans (Flegel's Disease)

Histopathological Differences Between Early and Old Lesions of Hyperkeratosis Lenticularis Perstans (Flegel's Disease)

A Case of Infantile Kyrle-Flegel Disease in a 6-Year-Old Yemeni Girl

Hyperkeratosis lenticularis perstans: Case report of a rare entity

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