Generalized Dowling-Degos disease

Wu, Yu‐Hung; Lin, Yen-Ting

doi:10.1016/j.jaad.2006.12.023

Cited by 61 publications

(46 citation statements)

References 30 publications

(52 reference statements)

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“…These may be variants of the same entity with similar clinicopathological features and they overlap in some cases 1 . The genetic defect of DDD has not yet been well defined 9 . A recently reported series described the loss of function mutations in the KRT5 gene encoding keratin K5 in two German pedigrees 10 .…”

Section: Discussionmentioning

confidence: 99%

“…A recently reported series described the loss of function mutations in the KRT5 gene encoding keratin K5 in two German pedigrees 10 . KRT5 gene mutations have previously been recognized as being involved in the pathogenesis of epidermolysis bullosa simplex 9,10 . However, no association between DDD and epidermolysis bullosa simplex has been reported 9 .…”

Section: Discussionmentioning

confidence: 99%

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A Case of Dowling-Degos Disease on the Vulva

et al. 2011

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Dowling-Degos disease (DDD) is an autosomal dominant genodermatosis and this disease is a genetically determined disturbance of epidermal proliferation. It is characterized by acquired, slowly progressive pigmented lesions that primarily involve the great skin folds and flexural areas such as the axilla, neck, limb flexures, the inframammary area and the inguinal folds. The vulva is an unusual location for DDD. A 41-year-old woman presented with a 10-year history of multiple, small, reticulated and brownish macules distributed symmetrically on the bilateral external genital regions. We found no other similarly pigmented skin lesions on her body, including the flexural areas. There was no known family history of similar eruptions or pigmentary changes. The histologic examination showed irregular rete ridge elongation with a filiform or antler-like pattern and basilar hyperpigmentation on the tips. Fontana-Masson staining showed increased pigmentation of the rete ridges and the S100 protein staining did not reveal an increased number of melanocytes in the epidermis. From these findings, we diagnosed this lesion as DDD.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A Case of Dowling-Degos Disease on the Vulva

et al. 2011

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“…1 Localized and generalized forms have been recognised. 2 It typically presents as an acquired hyperpigmentation, particularly of the flexures, beginning in adult life.…”

Section: Discussionmentioning

confidence: 99%

Unique brown star shape on dermatoscopy of generalized Dowling-Degos disease

Geissler

Dyall‐Smith

Coras

et al. 2011

Australasian Journal of Dermatology

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CASE REPORTAn otherwise healthy 49-year-old woman presented with a 15-year history of a slowly extending rash and pigment changes particularly on the thighs and between the breasts. She described episodic pruritus, worse with heat, friction and perspiration. Her family history was unremarkable.Clinical examination revealed numerous generalized 2-4 mm brown and hypopigmented macules. Excoriated papules were seen over the thighs and intermammary skin (Fig. 1). There were pitted acneiform scars with hypo-and hyperpigmentation in the perioral area.Dermatoscopy of the papules consistently revealed an irregular star-shaped brown outline on a red-brown background (Fig. 2). Follicular plugging and inclusion cysts were seen centrally.Skin biopsies from papules on the thighs and chest revealed flattened epidermis centrally and on the periphery, adenoid proliferations of the rete ridges in a filiform pattern with basal hyperpigmentation, inclusion cysts and follicular plugging (Fig. 3). Parakeratosis was seen in some lesions, possibly due to excoriation. There were scattered melanophages in the upper papillary dermis. Acantholysis was not seen despite examination of multiple sections of multiple papules. S100, Melan A and HMB45 stains showed increased pigment especially at the periphery.The diagnosis of generalized Dowling-Degos disease was made on the clinical and histological findings. DISCUSSIONDowling-Degos disease is an autosomal dominant genodermatosis with variable penetrance, but is often sporadic, as in our patient.1 Localized and generalized forms have been recognised. 2 It typically presents as an acquired hyperpigmentation, particularly of the flexures, beginning in adult life.To our knowledge, the dermatoscopic features of Dowling-Degos disease have been reported in only one case of localized Dowling-Degos disease of the vulva.

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“…3,4 Despite the lack of any consensus regarding the classification of hereditary pigmentary dermatoses, it was recently agreed that Dowling-Degos disease is a clinicopathological entity that has several variants including the acantholytic form referred to as GalliGalli disease and the acral form referred to as reticulate acropigmentation of Kitamura. 1,2,5 The present paper reports the case of a patient with clinical and histopathological characteristics of Dowling-Degos disease. In addition, the appearance of a verrucous lesion on one of the patient's feet years after onset of the disease was particularly noteworthy.…”

Section: Commentsmentioning

confidence: 95%

“…1,2 It is characterized clinically by reticular hyperpigmentation in the flexures, such as in the neck, armpits, antecubital fossae, the area under the breasts and in the groin. Additional findings include pitted perioral scars in patients with no previous history of acne and hyperkeratotic comedone-like follicular papules in the neck and axillary regions.…”

Section: Commentsmentioning

confidence: 99%

Caso para diagnóstico

Hohmann¹,

Köche²,

Bonamigo³

et al. 2010

An. Bras. Dermatol.

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A doença de Dowling-Degos é uma genodermatose rara, caracterizada principalmente por hiperpigmentação reticular progressiva de áreas flexurais. Apesar de apresentar evolução benigna, pode estar associada a neoplasias da pele. Além disso, as alterações cutâneas características potencialmente ocasionam prejuízo psicossocial, devido aos danos estéticos significativos. Os autores descrevem um caso dessa doença associado a ceratoacantoma e sintetizam os conceitos atuais sobre ela.

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Generalized Dowling-Degos disease

Cited by 61 publications

References 30 publications

A Case of Dowling-Degos Disease on the Vulva

A Case of Dowling-Degos Disease on the Vulva

Unique brown star shape on dermatoscopy of generalized Dowling-Degos disease

Caso para diagnóstico

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