Dowling-Degos disease – characteristic symptoms. How does the disease develop?

Dowling-Degos disease is a reticulated pigmented abnormality of the flexural surfaces. It is a rare disease that begins in old age.

How does Dowling-Degos disease progress?

An autosomal dominant inheritance is suggested. Disturbances in the K5 keratin gene responsible for the distribution of melanosomes inside keratinocytes have been demonstrated. In some cases, the disease is referred to as familial.

Dowling-Degos disease and characteristic symptoms

In Dowling-Degos disease, small brown spots appear that gradually merge into a mesh-like pattern. They occupy the armpits, groins, and sometimes other bends symmetrically. Less commonly, hyperkeratotic lumps on the hands, pockmarked depressions, and cysts and blackhead-like lesions around the mouth can be seen. Vulva involvement has been described in some patients.

Diagnostics of Dowling-Degos disease

During diagnosis, an increased amount of melanin is revealed in the basal layer. In addition, there is an epidermal growth similar to the image of seborrheic warts, usually with glandular hypertrophy, and perivascular monocyte infiltrates. Galli-Galli disease is an acantholytic variant.

In the diagnosis of the disease, it is important to differentiate with other ailments, e.g .:

  1. choroba Galli-Galli,
  2. choroba Kitamury.

What’s the prognosis?

Dowling-Degos disease is progressive. Sometimes basal cell carcinoma develops in the macula.

Lit.: [1] Arda F., Fetil E., Ilknur T. i wsp.: Dowling-Degos and Kitamura: a diff erent features of the same entity? J Dermatol 2008, 35; 311-3. [2] Massone C., Hofmann—Wellenhof R.: Dermoscopy of Dowling-Degos disease of the vulva. Arch Dermatol 2008, 144; 417-8. [3] Planko L., Böhse K., Höhfeld J. i wsp.: Identifi cation of a keratin-associated protein with a putative role in vesicle transport. Eur J Cell Biol 2007, 86; 827-9. [4] Wu Y.H., Lin Y.C.: Generalized Dowling-Degos disease. J Am Acad Dermatol 2007, 57; 327-34.

Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House

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