Inframammary Dermatitis: A Case of Localized Late-Onset Darier’s Disease

Linder, Dennis; Marinello, Elena; Donisi, Pietro Maria; Salmaso, Roberto; Zattra, Edoardo; Zampetti, Anna

doi:10.1159/000447482

Cited by 6 publications

(8 citation statements)

References 12 publications

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“…4 Tal fato, por sua vez, leva à alteração na sinalização de cálcio intracelular com perda da adesão suprabasal dos queratinócitos e à ativação das vias apoptóticas celulares, culminando nos achados histopatológicos de acantólise e disceratose, respectivamente. [3][4][5][6][7] As manifestações clínicas da doença de Darier-White ocorrem na forma de pápulas foliculares e, em alguns casos, interfoliculares. São de tonalidade amarelada-acinzentada, com tendência a coalescerem-se, resultando em placas grossas e untuosas.…”

Section: Introductionunclassified

“…Ademais, as palmas, as solas, as unhas e as mucosas podem também ser atingidas. 3,7,8 Paralelamente a isso, a disceratose folicular apresenta, ainda, diferentes classificações clínicas descritas, tais como as variações vesículobolhosa, comedônica, acral hemorrágica, localizada linear, leucodérmica macular e a cornificada. Nesse ponto de vista, a DD localizada pode ser dividida em dois subtipos: Tipos 1 e 2.…”

Section: Introductionunclassified

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Prevalência da doença de Darier no estado do Ceará

Barros

Lima

et al. 2019

Rev. Med. UFC

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RESUMOObjetivo: averiguar a situação epidemiológica da doença de Darier no estado do Ceará no ano de 2015. Metodologia: trata-se de um estudo retrospectivo quantitativo, no qual foram analisados todos os registros das consultas médicas realizadas no Centro de Referência em Dermatologia Dona Libânia (CDERM), Fortaleza, Ceará, em 2015, através das variáveis: nome, sexo, idade, data da consulta, número do prontuário, diagnóstico presuntivo ou confirmado e a frequência do paciente no CDERM. Resultados: dos 19.576 pacientes analisados, 33 pacientes foram diagnosticados com doença de Darier, representando uma incidência de 0,1685% no serviço, enquanto que na população cearense uma incidência de 1:270.000, aproximadamente. Dentre estes pacientes, 18 eram do sexo feminino e 15 do sexo masculino. A idade média dos pacientes foi de 40,66 anos. Conclusão: a menor prevalência da doença de Darier no estado do Ceará, em discordância com a literatura, no ano de 2015, poderia ser justificada por um subdiagnóstico da doença. O atraso na idade de diagnóstico corrobora para esta hipótese. Entretanto, estudos multicêntricos e epidemiológicos maiores, tanto no Ceará como no Brasil, serão necessários para uma adequada avaliação da prevalência desta doença.Palavras-chave: Doença de Darier. Epidemiologia. Dermatologia. ABSTRACTObjective: Determine the epidemiological situation of Darier disease in the state of Ceará, Brazil, in the year of 2015. Methods: It´s a retrospective quantitative study in which all the records of the medical consultations performed at the Centro de Referência em Dermatologia Dona Libânia (CDERM), at Fortaleza, Ceará, Brazil, in 2015, were analyzed, using the following variables: name, gender, age, date of the consultation, medical record number, presumptive or confirmed diagnosis and the patient's frequency in CDERM. Results: Of the 19,576 patients analyzed, 33 patients were diagnosed with Darier disease, representing an incidence of 0.1685% in the service, while in the population of Ceará, the prevalence is approximately 1:270,000. Among these patients, 18 were female and 15 were male. The mean age of the patients was 40.66 years. Conclusion: The lower prevalence of Darier disease in the state of Ceará, in disagreement with the literature, in the year 2015, could be justified by an underdiagnosis of the disease. The delay in the age of diagnosis corroborates this hypothesis. However, larger multicenter and epidemiological studies, both in Ceará and Brazil, will be necessary for an adequate evaluation of the prevalence of this disease.

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Section: Introductionunclassified

Prevalência da doença de Darier no estado do Ceará

Barros

Lima

et al. 2019

Rev. Med. UFC

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“…Darier disease, also known as 'Darier-White disease' or 'dyskeratosis follicularis', is an uncommon autosomal dominant genodermatosis with complete penetrance and variable expressivity. 1,2 It mainly manifest as hyperkeratotic, firm papules predominating in seborrheic areas and flexures with associated nail abnormalities. 3 However, It is often under-diagnosed because of the paucity of lesions.…”

Section: Introductionmentioning

confidence: 99%

“…3 However, It is often under-diagnosed because of the paucity of lesions. 2 Skin signs may be associated with itch and a distinct odor, which could lead to discomfort and social handicap. 1 We present a rare case of Darier's disease with late onset in a 61year old female with no familial history of identical disease.…”

Section: Introductionmentioning

confidence: 99%

Late onset Darier’s disease: clinical and dermoscopic features

Senhaji¹,

Jouari²,

Gallouj³

et al. 2018

JDC

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“…The gene is responsible for proper functioning of a calcium pump in endoplasmic reticulum (ATPase 2, a so-called SERCA 2) involved in an adhesion and epithelial cell differentiation process. The mutation in the gene ATP2A2 weakens connections between keratinocytes, causes acantholysis and abnormal keratinization of epidermis, what is of key importance in a pathomechanism of lesion development in Darier's disease [2]. The disease occurs in all ethnical groups with the frequency of 1/100,000 population, equally frequently in both sexes.…”

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confidence: 99%

Severe Darier’s disease in a 39-year-old patient

Kozłowska¹,

Reduta²,

Bacharewicz-Szczerbicka³

et al. 2018

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Introduction. Darier's disease is a rare genodermatosis inherited in an autosomal dominant manner. Clinically, it manifests with hyperkeratotic papules in seborrhoeic and intertriginous areas as well as involvement of nail plates and mucous membranes. Patients may present with neuropsychiatric comorbidities. Objective. To present a case of a patient with severe Darier's disease that was successfully treated with acitretin. Case report. A 39-year-old man with a 24 year history of Darier's disease was admitted to the hospital because of an exacerbation of skin lesions. A year earlier he underwent an episode of severe depression. Because of signficant severity of skin lecions, acitretin was introduced with improvement. Conclusions. Oral retinoids are the first line treatment against severe exacerbations of Darier's disease. Because of possible development of neuropsychiatric disorders, patients with Darier's disease should receive psychological and/or psychiatric consultations. streszczenie Wprowadzenie. Choroba Dariera jest rzadką genodermatozą dziedziczoną autosomalnie dominująco. Klinicznie objawia się występowaniem hiperkeratotycznych grudek w obrębie okolic łojotokowych i wyprzeniowych oraz zajęciem płytek paznokciowych i błon śluzowych. U chorych mogą występować zaburzenia neuropsychiatryczne. Cel pracy. Przedstawienie przypadku pacjenta z chorobą Dariera o ciężkim przebiegu skutecznie leczonego acytretyną. Opis przypadku. Trzydziestodziewięcioletni pacjent z wywiadem choroby Dariera od 15. roku życia został przyjęty do Kliniki z powodu zaostrzenia zmian skórnych. Przed rokiem przebył epizod ciężkiej depresji. Ze względu na bardzo duże zaawansowanie schorzenia do terapii włączono acytretynę z dobrym efektem. Wnioski. Retinoidy doustne stanowią leczenie pierwszego wyboru ciężkich zaostrzeń choroby Dariera. Ze względu na możliwy rozwój zaburzeń neuropsychiatrycznych pacjenci z chorobą Dariera powinni być objęci stałą opieką psychologiczną i/lub psychiatryczną.

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Inframammary Dermatitis: A Case of Localized Late-Onset Darier’s Disease

Cited by 6 publications

References 12 publications

Prevalência da doença de Darier no estado do Ceará

Prevalência da doença de Darier no estado do Ceará

Late onset Darier’s disease: clinical and dermoscopic features

Severe Darier’s disease in a 39-year-old patient

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