Generalized Dowling-Degos Disease: Case Reports
The proband was an 82-year-old man who was presented with progressively generalized skin lesions since adolescence. He began to develop areas of hyperpigmentation, initially on the face, but then soon affecting the neck, axillae, groin, trunk and limbs (). The eruption has continued to become more extensive throughout adulthood leading to generalized pruritus. The patient was born of a non-consanguineous marriage. All of his offspring were affected with the same disease. His general physical examination was normal. Skin examination revealed a symmetrical reticulate hyperpigmentation predominantly affecting the lower face, skin folds, major flexures, back and legs. Symmetrically distributed reticulate hypopigmentation was also seen in the legs. Multiple pits varying in size from 1~3 mm and open comedone-like lesions were distributed over the cheeks, perioral area, neck and back. No breaks in the epidermal ridge pattern on the palms and soles were seen. The mucosa, nails, teeth and hair were normal. On investigation, complete blood count, blood chemistry, liver and renal function tests were all within normal limits.\nA biopsy was conducted from a hyperpigmented lesion over the shin, and histopathological examination revealed hyperkeratosis, acanthosis and irregular elongated thin branching rete ridges growing down into the dermis and increased melanin pigment in the lower part of the rete pegs (). Another skin biopsy from comedo-like lesions on the back shows keratin-filled cysts resembling comedones (). These features were entirely consistent with the clinical diagnosis of DDD,.