Isolated acral Darier's disease with haemorrhagic lesions in a kindred

Regazzini, R.; Zambruno, Giovanna; Defilippi, Claudio; Rosso, Renato; Donadini, A.

doi:10.1111/j.1365-2133.1996.tb01531.x

Cited by 13 publications

(17 citation statements)

References 6 publications

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“…The family tree of the patient and the family history were negative for Darier’s disease. In the cases discussed by Regazzini et al [4], consanguineous marriage and an alteration of chromosome 12 were found. Our case, in which chromosome mapping was not possible, can be classified as sporadic.…”

Section: Discussionmentioning

confidence: 99%

“…Hur et al [6]reported an acral case in a 16-year-old Korean boy complicated by Kaposi’s varicelliform eruption. Three other cases were described by Regazzini et al [4]: a 42-year-old woman with keratotic papules and pits of the hands and feet and haemorrhagic macules on the fingers, her 38-year-old sister with palmoplantar pits associated with haemorrhagic macules on the hands and the former’s 12-year-old daughter with hyperkeratotic papules on the extensor surface of the arms and haemorrhagic macules on the hands and fingers. Hafner and Vakilzadeh [7]reported 3 patients with verruciform papules on the dorsum of the hands and feet.…”

Section: Discussionmentioning

confidence: 99%

“…Acral involvement occurs in almost all cases [4], manifesting with keratotic papules on the backs of the hands and feet, palmoplantar keratosis and dystrophic nails. The exclusively acral form of Darier’s disease is rare and questioned in its nosology.…”

Section: Discussionmentioning

confidence: 99%

“…Sometimes the disease begins at the extremities and speads to the typical areas. Rarely it is limited to the acral regions; only few such cases have been described [3, 4, 5, 6, 7]. The disease has a chronic course, manifesting itself between the ages of 5 and 10 years, with a peak at puberty.…”

Section: Introductionmentioning

confidence: 99%

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A Case of Acral Darier’s Disease

Romano

Massai²,

Alessandrini³

et al. 1999

Dermatology

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

A Case of Acral Darier’s Disease

Romano

Massai²,

Alessandrini³

et al. 1999

Dermatology

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“…An erster Stelle stehen Nageldystrophien, gefolgt von "palmar pits" und der Akrokeratosis verruciformis. Diese können initiale Manifestationen vor Auftreten anderer DfD Läsionen darstellen [11,13]. Auch abortive Fälle von M. Darier, die lediglich Leistenunterbrechungen im Papillarrelief, evtl.…”

Section: Introductionunclassified

Eine hämorrhagische akrale Verlaufsformen der Dyskeratosis follicularis Darier

Jörg

Erhard

Rutten³

2000

Der Hautarzt

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A 76-year-old woman presented with a venous leg ulcer 7. Incidentally hemorrhagic macules on hands and fingers and dystrophy of toe and fingernails were noticed. Except for submammary erythema, no further dermatologic signs were shown. Histology of palmar lesions fit with a hemorrhagic lesion of dyskeratosis follicularis in acral skin. During following months actinic induced reddish-brown hyperkeratotic papules appeared on the forehead and in seborrheic and intertriginous areas of the trunk. Papules were most prominent during summer and almost completely resolved in winter. The hemorrhagic variant of dyskeratosis follicularis represents a rare clinical variant which has to be separated from other purpuric macules on acral sites.

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Inherited Acantholytic Disorders

Zamiri

Munro

2016

Rook's Textbook of Dermatology, Ninth Edition

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Darier disease (DD) and Hailey–Hailey disease (HHD) are autosomal dominant disorders characterized by epidermal acantholysis. They have a similar molecular pathogenesis, with mutations in the intracellular calcium pumps of the endoplasmic reticulum (SERCA2) and Golgi (SPCA1), respectively. DD presents with persistent, hyperkeratotic papules in a seborrhoeic distribution, associated with nail changes and palmar pitting. HHD presents with painful, mainly flexural, erosions. In both, secondary infection is common and hypertrophic flexural disease can be disabling. Histologically, both are characterized by loss of epidermal intercellular adhesion (acantholysis). In DD, acantholysis is predominantly suprabasal and there is overlying dyskeratosis. In HHD, acantholysis is more diffuse. First line topical treatment is with emollients, antimicrobials and corticosteroids. Oral antibiotics are often necessary for exacerbations; other systemic options include retinoids and ciclosporin. Physical measures such as laser resurfacing, photodynamic therapy, botulinum toxin and surgical interventions can be considered in refractory cases.

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Isolated acral Darier's disease with haemorrhagic lesions in a kindred

Cited by 13 publications

References 6 publications

A Case of Acral Darier’s Disease

A Case of Acral Darier’s Disease

Eine hämorrhagische akrale Verlaufsformen der Dyskeratosis follicularis Darier

Inherited Acantholytic Disorders

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