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

Dowling-Degos disease (DDD) is an autosomal dominant disorder with variable phenotypic expression. Classically, DDD is characterized by progressive reticulate hyperpigmentation on flexures with perioral pitted scars and comedone-like hyperkeratotic papules. Follicular DDD is a rare variant which was introduced by Singh et al. [<i>Indian J Dermatol Venereol Leprol</i>. 2013 Nov–Dec;79(6):802–4]. Follicular DDD differs from other variants because of its notable comedone-like hyperkeratotic hyperpigmented papules and a distinct histopathology which demonstrates pigmented filiform and branching rete pegs originating at the follicular infundibulum with many epidermal horn cysts while the interfollicular epidermis is essentially normal. Hereby, we present a case of follicular DDD with hidradenitis suppurativa (HS). A 37-year-old Thai man presented with slowly progressive hyperpigmented comedone-like papules on the face, neck, axillae, upper trunk, and buttocks with perioral pitted scars. Punch biopsy from a comedonal lesion on his back was consistent with follicular DDD. He also had recurrent painful nodules and abscess on the back, groin, and buttock which matched the clinical criteria for the diagnosis of HS. To date, a paucity of concurrent DDD with HS has been reported. Recent genetic studies speculate a shared pathophysiologic mechanism of DDD and HS.

A Case of Degos Disease Complicated By Constrictive Pericarditis In Remote Phase

Background: Degos disease, also known as malignant atrophic papulosis, is characterized by cutaneous manifestations due to chronic thrombo-obliterative vasculopathy. There have been reports of rare late-onset Degos disease complicated by constrictive pericarditis (CP). We report a case of CP caused by Degos disease that developed 20 years after diagnosis.Case presentation: A 62-year-old woman who has been taking aspirin for 20 years for Degos disease was hospitalized for worsening heart failure. The patient was diagnosed with CP and underwent pericardiectomy. Pathological findings suggested the involvement of Degos disease. The postoperative course was uneventful, and her heart failure and Degos disease did not worsen.Conclusions: This report suggests that Degos disease can cause long-term CP. Aspirin effectively inhibited the progression of Degos disease, and surgical treatment is necessary when heart failure due to CP is refractory to treatment.