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Introduction And Importance: Darier-Ferrand dermatofibrosarcoma (DFS) is a rare mesenchymal tumor with an aggressive local behavior, high local recurrence frequency and low metastatic potential. It commonly presents as a raised slowly growing mass. It usually occurs on trunk and proximal extremities but rarely touches distal extremities such as hands, fingers, or foot below knees.

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An Update on the Cutaneous Manifestations of Darier Disease.

J Cutan Med Surg

September 2021

26747 Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel.

Article Synopsis
  • The study investigates the clinical features of Darier disease, a rare genetic disorder, involving 76 patients through structured interviews, physical exams, and medical record reviews.
  • Most lesions were found on the hands and fingernails, with a unique "wet hand sign" observed, indicating increased visibility after water exposure; 43% of patients also presented with oral lesions.
  • Severe cases showed a significantly higher risk of genital lesions, emphasizing the importance of documenting specific areas like the hands and mucosal regions for accurate diagnosis.
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Darier disease is an autosomal-dominant inherited condition caused by mutation of a gene, which produces a protein involved in calcium channel regulation. The disease has a variety of manifestations and lacks consistent genotype-phenotype correlations. Acral hemorrhagic Darier disease causes macules, papules, vesicles and/or hemorrhagic blisters on the extremities.

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Dermoscopy: a useful auxiliary tool in the diagnosis of type 1 segmental Darier's disease.

Dermatol Pract Concept

April 2016

Department of Experimental and Clinical Medicine, Institute of Dermatology, University of Udine, Italy.

Type 1 segmental Darier's disease is a blaschkolinear variant of Darier's disease resulting from a postzygotic mosaicism. Since it usually lacks diagnostic clues typical of the generalized form, including positive family history of the disease, nail and mucosal abnormalities, and/or acral involvement, its distinction from other acquired inflammatory blaschkolinear dermatoses may often be quite challenging, thus requiring histopathological examination to reach a definitive diagnosis. We report a case of type 1 segmental Darier's disease with its dermoscopic findings in order to show the usefulness of dermoscopy in assisting the noninvasive identification of this condition.

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