Ann Dermatol.  2013 Aug;25(3):360-364. 10.5021/ad.2013.25.3.360.

Generalized Dowling-Degos Disease: Case Reports

• Wititsuwannakul J
• Noppakun N

• KMID: 2265879
• DOI: http://doi.org/10.5021/ad.2013.25.3.360

Abstract

Dowling-Degos disease (DDD) is a rare autosomal dominant trait characterized by numerous, symmetrical, progressive and pigmented macules over the axillae, groins, face, neck, arms and trunk as well as scattered comedo-like lesions (dark dot, follicles) and pitted acneiform scars. Histopathology is diagnostic testing using a distinctive form of acanthosis, characterized by an irregular elongation of thin branching rete ridges, with a concentration of melanin at the tips. We report cases of generalized DDD in a single family with autosomal dominant penetrance. DDD can be presented in a generalized form with hypopigmented lesions instead of reticulate hyperpigmentation confined to the flexor areas. This form can be differentiated from DUH by histopathology.

Keyword

Dowling-Degos disease; Pigmentary disorder; Reticulate

MeSH Terms

Arm
Axilla
Cicatrix
Diagnostic Tests, Routine
Dichlorodiphenyldichloroethane
Groin
Humans
Hyperpigmentation
Melanins
Neck
Penetrance
Skin Diseases, Genetic
Skin Diseases, Papulosquamous
Dichlorodiphenyldichloroethane
Hyperpigmentation
Melanins
Skin Diseases, Genetic
Skin Diseases, Papulosquamous

Figure

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  Fig. 1 Pedigree of family with generalized Dowling-Degos disease (DDD). Proband (P), his first son (case 2), and his second daughter (case 3) had generalized DDD.

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  Fig. 2 Skin lesion of proband, 82-year-old man. (A, B) Reticulate hyperpigmentation, multiple pits and comedo-like lesions distributed over the face, perioral area and neck. (C) Reticulate hyperpigmentation with symmetrically distributed hypopigmented macules on shins. (D) Comedo-like lesions distributed over the back.

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  Fig. 3 Son of proband, 58-year-old man. (A) Reticulate pigmented macules, multiple pits and comedo-like lesions distributed over the face, perioral area and neck. (B) Reticulate hyperpigmentation on axilla.

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  Fig. 4 Daughter of proband, 50-year-old woman. (A) Reticulate pigmented macules, multiple pits and comedo-like lesions distributed over the face, perioral area and neck. (B, C) Reticulate hyperpigmentation on forearms.

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  Fig. 5 Skin biopsy specimens from proband of hyperigmented macule (A) and comedo-like lesion (B). (A) Epidermal hyperkeratosis, acanthosis, irregular elongated thin branching rete ridges growing down into the dermis and increased melanin pigment in the lower part of rete pegs (H&E, ×200). (B) Keratin-filled cysts resembling comedones with irregular elongated thin branching rete ridges growing down into the dermis and increased melanin pigment in the lower part of rete pegs (H&E, ×40).

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