DowlingDegos disease is a rare autosomal dominant condition. It is caused by loss of function mutations in keratin 5 gene (kRT5) situated in keratin gene cluster on 12q13. We are reporting three cases of Dowling Degos disease out of which two belong to same family. The clinical manifestations and histopathology were analyzed. All the three patients of which two were mother and son had symmetric, reticulate hyperpigmentation, pitted scars and acneiform eruption over face (predominantly perioral area, nasolabial folds), pinnae, upper trunk, thighs were seen. Palms, soles and scalp were spared. Buccal mucosa also showed reticulate hyperpigmentation. In addition the female patient had vulval reticulate hyperpigmentation and hydradenitis suppurativa affecting axillae, groins and inframammary folds.
Key words: Dowling Degos disease, Hidradenitis suppurativa, Reticulate pigmentary disorders
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