Pathology of Dowling-Degos disease

Introduction

Dowling-Degos disease is a autosomal dominant skin disorder characterized by progressive pigmentation of the skin within the folds of the body. Association with loss of function mutations in KRT5 (encoding curb 5) is documented.

Histology of Dowling-Degos disease

Epidermal changes in Dowling-Degos disease include hyperkeratosis, often with small horn cysts and thinning of the suprapapillary epidermis. Slim epithelial the threads extend towards the surface dermis of the epidermis and hair follicles resulting in a “horn” pattern (Figures 1, 2). Epidermal hyperpigmentation It is common although rarepigmented Variants are reported. Hair follicle infundibulum may be dilated Dermal perivascular lymphohistiocytic infiltrate (figures 1,2,3) or a lichenoid A tissue reaction pattern can be observed.

Differential diagnosis of Dowling-Degos disease

Galli-Galli disease is a subtype of Dowling-Degos disease and is distinguished by the additional finding ofdyskeratotic acantholysis (Figure 3, arrow). Other clinics and histological The characteristics are identical to Dowling-Degos disease.

Seborrheic keratosis - in Dowling-Degos disease, epithelial strands extend from the epidermis and hair follicles. The clinical presentation is very useful to differentiate these conditions.

To have syndrome – histological characteristics shared with Dowling-Degos disease; however, the clinical presentation is different with facial rosacea-like lesions, pitted scars, and seborrheic keratosis-like lesions on the trunk and push-ups. Haber syndrome is not associated with keratin 5 mutations.