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Association of Dowling-Degos disease and multiple seborrheic keratosis in a "Christmas tree pattern".

Dos Santos VM, Pereira NL, Silva RF, Silva FH, Garcia CJ, Sousa MA - Med J Islam Repub Iran (2014)

Bottom Line: This entity is due to mutations in the keratin 5 gene, and is related with other cutaneous disorders.Worthy of note in this case study is the lesions evolved for over than 30 years.The aim is to describe the association of these keratoses with Dowling-Degos disease in a healthy man.

View Article: PubMed Central - HTML - PubMed

Affiliation: 1. MD, PhD. Internal Medicine Department of Armed Forces Hospital (HFA) and Catholic University (UCB), Brasília- DF, Brazil. vitorinomodesto@gmail.com.

ABSTRACT
Dowling-Degos disease is a rare sporadic or autosomal dominant pigmentary entity, in which clusters of papules and reticulate macules slowly develop with predominance in flexural regions. This entity is due to mutations in the keratin 5 gene, and is related with other cutaneous disorders. We report the sporadic form of Dowling-Degos disease in an elderly man with multiple seborrheic keratosis in a "Christmas tree" pattern. Worthy of note in this case study is the lesions evolved for over than 30 years. The aim is to describe the association of these keratoses with Dowling-Degos disease in a healthy man.

No MeSH data available.


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Mentions: A 75-year-old afro descendent man was hospitalized with transient ischemic attack (TIA). Because of diabetes mellitus, arterial hypertension, and antecedent ischemic cerebrovascular events, he was taking captopril, metformin, indapamide, and simvastatin. Physical examination, showed perioral acneiform pits and comedones, and symmetric bilateral pigmented papules in the flexures (neck, axilla, and groin) and around the ears. There were thoracodorsalmacules with a “Christmas tree” pattern, with no remarkable skin changes in extremities (Fig. 1). He was 45-years-old at the onset of lesions, which slowly appeared in the groins and axillae and after affected ears and neck. Over three decades, the pigmented changes had progressed and evolved asymptomatic, except for rare pruritus. He wondered that the skin papules were associated with his natural ageing; and the pigmented macules over the trunk as a consequence of his afro descendent origin. Otherwise, he observed that the main development of hyperkeratotic papules occurred in areas of friction. He denied family history of similar lesions. During the last two years, he had been under clinical outpatient surveillance. Laboratory tests and imaging studies were done to discard the hypothesis of eventual unsuspected malignancy. Routine controls, tumor markers, gastrointestinal endoscopy, and chest images were normal. The histopathology study of flexural lesions showed keratin plugs within thedilated follicularostium of pseudocysts from seborrheic papules, increased melanin in the basal membrane, melanophages in the superficial dermis, melanocytes in the granular layer, and thin branches of epidermal downgrowths in the upper dermis (Fig. 2). The clinical features and microscopic findings were consistent with the diagnosis of Dowling-Degos disease coexistent with seborrheic keratosis. Taking in account the absence of any consistent finding of internal malignancy or HIV infection, he was discharged to home after improvement of the neurological manifestations related to TIA.


Association of Dowling-Degos disease and multiple seborrheic keratosis in a "Christmas tree pattern".

Dos Santos VM, Pereira NL, Silva RF, Silva FH, Garcia CJ, Sousa MA - Med J Islam Repub Iran (2014)

© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4219898&req=5

Mentions: A 75-year-old afro descendent man was hospitalized with transient ischemic attack (TIA). Because of diabetes mellitus, arterial hypertension, and antecedent ischemic cerebrovascular events, he was taking captopril, metformin, indapamide, and simvastatin. Physical examination, showed perioral acneiform pits and comedones, and symmetric bilateral pigmented papules in the flexures (neck, axilla, and groin) and around the ears. There were thoracodorsalmacules with a “Christmas tree” pattern, with no remarkable skin changes in extremities (Fig. 1). He was 45-years-old at the onset of lesions, which slowly appeared in the groins and axillae and after affected ears and neck. Over three decades, the pigmented changes had progressed and evolved asymptomatic, except for rare pruritus. He wondered that the skin papules were associated with his natural ageing; and the pigmented macules over the trunk as a consequence of his afro descendent origin. Otherwise, he observed that the main development of hyperkeratotic papules occurred in areas of friction. He denied family history of similar lesions. During the last two years, he had been under clinical outpatient surveillance. Laboratory tests and imaging studies were done to discard the hypothesis of eventual unsuspected malignancy. Routine controls, tumor markers, gastrointestinal endoscopy, and chest images were normal. The histopathology study of flexural lesions showed keratin plugs within thedilated follicularostium of pseudocysts from seborrheic papules, increased melanin in the basal membrane, melanophages in the superficial dermis, melanocytes in the granular layer, and thin branches of epidermal downgrowths in the upper dermis (Fig. 2). The clinical features and microscopic findings were consistent with the diagnosis of Dowling-Degos disease coexistent with seborrheic keratosis. Taking in account the absence of any consistent finding of internal malignancy or HIV infection, he was discharged to home after improvement of the neurological manifestations related to TIA.

Bottom Line: This entity is due to mutations in the keratin 5 gene, and is related with other cutaneous disorders.Worthy of note in this case study is the lesions evolved for over than 30 years.The aim is to describe the association of these keratoses with Dowling-Degos disease in a healthy man.

View Article: PubMed Central - HTML - PubMed

Affiliation: 1. MD, PhD. Internal Medicine Department of Armed Forces Hospital (HFA) and Catholic University (UCB), Brasília- DF, Brazil. vitorinomodesto@gmail.com.

ABSTRACT
Dowling-Degos disease is a rare sporadic or autosomal dominant pigmentary entity, in which clusters of papules and reticulate macules slowly develop with predominance in flexural regions. This entity is due to mutations in the keratin 5 gene, and is related with other cutaneous disorders. We report the sporadic form of Dowling-Degos disease in an elderly man with multiple seborrheic keratosis in a "Christmas tree" pattern. Worthy of note in this case study is the lesions evolved for over than 30 years. The aim is to describe the association of these keratoses with Dowling-Degos disease in a healthy man.

No MeSH data available.


Related in: MedlinePlus