Hidradenitis suppurativa, Dowling-Degos and multiple epidermal cysts: a new follicular occlusion triad

Abstract

This case illustrates the rare association between hidradenitis suppurativa (HS) and Dowling-Degos disease (DDD). Furthermore the association of HS, DDD and multiple epidermal cysts has not to our knowledge been described before, but their coexistence in the same patient is likely to reflect the same follicular anomaly. It is possible that a single underlying defect of follicular proliferation may account for the coexistence of these conditions.

Novel Mutations in PSENEN Gene in Two Chinese Acne Inversa Families Manifested as Familial Multiple Comedones and Dowling-Degos Disease

“…[14] The histopathological change of DDD is very characteristic, showing dilated follicular, fingerlike projections called rete ridges, with thinning of the suprapapillary plates, resulting in an “antler-like” pattern and increased pigmentation of the basal layer. [15] The histological change of the family 1 in the present study resembled DDD or suggested a coexistence of AI and DDD. AI has been reported to be associated with DDD, and it is possible that the defect of follicular proliferation may account for the coexistence of these conditions.…”

Dowling-Degos Disease Associated With Hidradenitis Suppurativa: A Case Report

“…One etiologic and pathogenic factor that has been observed is a defect of epithelial proliferation in the external sheath, leading to follicular occlusion, described as a possible common origin for all these disorders. 5,7,9 DDD must be differentiated from other genodermatoses with reticular pigmentation 2,3 : Galli-Galli disease, 10 reticulate acropigmentation of Kitamura, Haber syndrome, and reticulate acropigmentation of Dohi. It has recently been agreed that all these reticular pigmentary diseases are variants of a single entity.…”

“…DDD has been associated with certain skin diseases, in particular multiple keratoacanthomas, 5 epidermoid cysts, 5 and squamous cell carcinoma. 6 An important association is with HS, as found in our patient, though few cases have been reported in the literature.…”

“…It has recently been agreed that all these reticular pigmentary diseases are variants of a single entity. 5 Other important diseases in the differential diagnosis include acanthosis nigricans, Naegeli-Franceschetti-Jadassohn syndrome, and confluent and reticulate papillomatosis (Gougerot-Carteaud syndrome).…”

Dowling-Degos disease and hidradenitis suppurativa: Co occurrence or association?

“…[2] It is characterized by a reticulate pigmentation of the flexures with prominent comedone like lesions and pitted scars. [345] The disease runs in families and has an autosomal-dominant mode of transmission with female preponderance.…”