Dowling-Degos Disease and Hidradenitis Suppurativa. Epidemiological and Clinical Study of 15 Patients and Review of the Literature

Abstract

Fig. 1. Clinical images. a) Patient #13. Symmetrically distributed hyperpigmented macules with a reticular disposition affecting the perineal area. Note the 2 nodules and the non-draining fistula. b) Patient #13. Comedon-like infundibular cysts and follicular plugging. c) Patient #14. Typical DDD hyperpigmented macules in major skinfolds. Note the inflamed abscess in the perineal area. d) Patient #14. Note the non-inflammatory nodules in the right axillae, hyperpigmentation and multiple doublecomedon openings.

Novel nicastrin mutation in hidradenitis suppurativa–Dowling–Degos disease clinical phenotype: more than just clinical overlap?

“…The clinical and genetic overlap of HS-DDD may also have a clinical relevance, translating into a personalized therapeutic management, such as the combination of retinoids and sulfones. 8 S. Garcovich iD , 1.2 P.M. Tricarico, 3 C. Nait-Meddour, 4,5 G. Giovanardi, 1,2 K. Peris, 1,2 S. Crovella 3,6 and M. Boniotto iD…”

“…The affected daughter did not yet present any clinical signs of DDD, which might develop at a later age, as observed in her father. The clinical and genetic overlap of HS‐DDD may also have a clinical relevance, translating into a personalized therapeutic management, such as the combination of retinoids and sulfones …”

Follicular Dowling-Degos Disease with Hidradenitis Suppurativa: A Case Report and Review of the Literature

“…HS is a chronic inflammatory disorder of the hair and follicles typically involving axillae, buttocks, groin, perineal, and inframammary regions [ 8 ]. According to case reports and clinical studies, concurrent DDD with HS has been reported in 53 cases (including our case) as shown in Table 2 [ 10 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 ]: 2 cases of which are follicular DDD variant and 1 case is associated with Galli-Galli disease, a variant of DDD [ 10 , 33 ]. Most cases reported were of female predominance and more than half presented with at least Hurley stage 2.…”

The pathogenesis of hidradenitis suppurativa: Evolving paradigms in a complex disease

“…These results may explain the diffuse scattering of comedones seen in HS prone areas, the presence of comedones in extra-flexural sites and the presence of comedones in previously inflamed ("burnt out") tissue or sites distant from a follicular unit. It also raises the additional question of what differentiates conditions in which subclinical inflammation and diffuse flexural comedone formation (such as Dowling-Degos disease 52,53 ) exist, from highly inflammatory HS lesions. Direct molecular comparisons of these conditions may further inform the differences in subclinical inflammation that leads to the development of one condition over another (or indeed the coexistence of both conditions at different timepoints).…”