A new article published in the International Journal of Dermatology discussed that Dowling-Degos disease – rare autosomal dominant genodermatosis, is characterized by acquired reticulate hyperpigmentation over the flexures, comedone-like follicular papules and pitted perioral scars that usually develop during adulthood.

The authors stated that mutations in genes affecting melanosome transfer, and melanocyte and keratinocyte differentiation have been implicated in the pathogenesis of this disease. These genes include KRT5, POFUT1, POGLUT1 and, most recently, PSENEN.

This article elaborated that Dowling-Degos disease can be found in isolation or with other associated findings, most notably hidradenitis suppurativa. This condition belongs to a spectrum of conditions that all result in reticulate hyperpigmentation that at times are hard to distinguish from each other.

The most closely linked entity is Galli-Galli, which is clinically indistinguishable from Dowling-Degos disease and can only be distinguished by the presence of acantholysis on microscopy. However, Dowling-Degos disease is generally progressive and recalcitrant to treatment.

Source: International Journal of Dermatology. 2020 Dec 23. doi: 10.1111/ijd.15385.