A Case of Acrogeria Associated with Late-Onset Focal Dermal Elastosis

Tajima, S.; Inazumi, Toyoko; Kobayashi, Teruki

doi:10.1159/000246381

Cited by 14 publications

(5 citation statements)

References 5 publications

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“…Classically, it has been described that the increased elastic fibers of the lesions have a normal appearance. [2][3][4][5][6] Unlike other cases, the increased elastic dermal tissue of our patients was composed of clearly abnormal elastic fibers, which appeared thick and interlacing. In our opinion, a critical review of the histopathologic illustrations of previously reported examples of late-onset focal dermal elastosis also reveals that the increased elastic dermal tissue is composed of structurally abnormal fibers.…”

Section: Discussionmentioning

confidence: 93%

“…This disorder affects preferentially elderly patients, with all reported cases ranging in age from 65 to 87 years, 2-5 with the exception of a 18-year old woman who suffered a premature aging syndrome. 6 Clínically, it is characterized by 2-4 mm diameter yellowish flat-topped multiple papules that may be asymptomatic or slightly pruritic. Characteristically, the lesions are arranged in groups, and in some cases, these may coalesce, resulting in confluent plaques with a cobblestone pattern.…”

Section: Discussionmentioning

confidence: 99%

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Familial Acral Localized Late-Onset Focal Dermal Elastosis

Camacho

Machan²,

Pielasinski³

et al. 2012

The American Journal of Dermatopathology

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Late-onset focal dermal elastosis is a rare disorder of elastic tissue, characterized by a local accumulation of elastic fibers in the mid and deep reticular dermis. This disorder occurs mainly in elderly people and consists of multiple yellow papules that are typically distributed on the sides of the neck and flexural areas, closely resembling pseudoxanthoma elasticum from the clinical point of view. We report 2 sisters who have presented with a 20-year history of multiple yellow papules, mainly located on the dorsum of their hands. They also had few lesions of similar morphology scattered on the anterior aspects of their wrists, thighs, and lower abdomen. Histopathologic study demonstrated a focal increase in thick, interlacing elastic fibers in the mid and deep reticular dermis. The most striking features of our cases were the early age of clinical presentation, the familial involvement, and the predominantly acral distribution of the lesions.

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Section: Discussionmentioning

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Familial Acral Localized Late-Onset Focal Dermal Elastosis

Camacho

Machan²,

Pielasinski³

et al. 2012

The American Journal of Dermatopathology

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“… 9 However, the cases in younger patients differ also in other clinical features from classic presentation, e.g. association with acrogeria, 6 familiarity, 7 or acral distribution. 5 , 7 From a review of the cases reported in the literature ( Table 1 ) it appears that under the term of LOFDE have been grouped heterogeneous clinical presentations, solely on the basis of the histologic trait of increased dermal elastic tissue.…”

Section: Discussionmentioning

confidence: 97%

“…LOFDE affects preferentially patients after the sixth decade, but a few reports relate to younger patients (18 years in the case of Tajima et al, 1996; around 30 years in the two familiar cases of Camacho et al, 2012; 39 years in the case 2 of Wang et al, 2012). [6][7][8] The term "focal dermal elastosis" has been proposed as an update of the nomenclature to include also these earlier presentations. 9 However, the cases in younger patients differ also in other clinical features from classic presentation, e.g.…”

Section: N O N -C O M M E R C I a L U S E O N L Ymentioning

confidence: 99%

Late-onset focal dermal elastosis: Report of a case and review of the literature

Fantini

Dainese

Recalcati³

2022

Dermatol Reports

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Late-onset focal dermal elastosis is a rare cutaneous condition classified within the disorders of increased dermal elastic tissue. It is characterized clinically by multiple papules with predilection for the neck and other flexures, without systemic alterations, and histologically by focally increased elastic fibers in the reticular dermis. Several elastic tissue disorders share a similar clinical presentation in the skin. Distinction of late-onset focal dermal elastosis and other pseudoxanthoma elasticum mimickers from pseudoxanthoma elasticum is critical, as they are not associated to systemic lesions. We report here on a case of late-onset focal dermal elastosis and review the scientific literature on this unusual condition.

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“…The first possibility seems likely because 1) degenerative skin changes were found on the nuchae, neck, axillae, and groin, where other types of elastosis are commonly seen (18)(19)(20), and 2) collagen-aggregated tissue does not contain any vessels nor sweat glands suggesting that an acute reactive aggregation had occurred in the scar-like tissue. However, it still is possible that penicillamine has some direct effect on collagen fibers.…”

Section: Discussionmentioning

confidence: 99%

Penicillamine‐Induced Degenerative Dermatoses: Report of a Case and Brief Review of Such Dermatoses

Iozumi

Nakagawa

Tamaki

1997

The Journal of Dermatology

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We describe a case of elastosis perforans serpiginosa with additional findings of degenerative skin changes. A 20-year-old man with hepatolenticular degeneration, under prolonged treatment with D-penicillamine, presented with a circular or serpiginous arrangement of nuchal papules. Histopathologically, transepidermal channels were accompanied by granulomatous reactions, with several giant cells engulfing elastic fibers. In addition to these findings of a typical elastosis perforans serpiginosa, we observed scar-like skin changes inside the circular arrangement of the papules. At the scar-like tissue, we found electron-microscopical evidence of randomly aggregated thin collagen fibers with no tendency toward systemic combined bundle formation, which is a characteristic feature of normal collagen fiber formation. Pseudoxanthoma-elasticum-like changes were observed on his neck. On his axillae and groin, slight skin thickening and wrinkling were detected. The diagnosis of elastosis perforans serpiginosa does not represent all of the manifestations or the pathological background described above. The skin manifestations described here represent not only an elastosis but also a total degenerative dermatosis with overhealed collagenosis. Thus, those dermatoses should be summarized as one entity, penicillamine-induced degenerative dermatosis. After considering the pathogenic background and clinical similarities, we further propose to simplify the penicillamine-induced skin manifestations to three categories: acute sensitivity reactions, bullous dermatoses, and degenerative dermatoses.

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A Case of Acrogeria Associated with Late-Onset Focal Dermal Elastosis

Cited by 14 publications

References 5 publications

Familial Acral Localized Late-Onset Focal Dermal Elastosis

Familial Acral Localized Late-Onset Focal Dermal Elastosis

Late-onset focal dermal elastosis: Report of a case and review of the literature

Penicillamine‐Induced Degenerative Dermatoses: Report of a Case and Brief Review of Such Dermatoses

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