CASE REPORTAn otherwise healthy 49-year-old woman presented with a 15-year history of a slowly extending rash and pigment changes particularly on the thighs and between the breasts. She described episodic pruritus, worse with heat, friction and perspiration. Her family history was unremarkable.Clinical examination revealed numerous generalized 2-4 mm brown and hypopigmented macules. Excoriated papules were seen over the thighs and intermammary skin (Fig. 1). There were pitted acneiform scars with hypo-and hyperpigmentation in the perioral area.Dermatoscopy of the papules consistently revealed an irregular star-shaped brown outline on a red-brown background (Fig. 2). Follicular plugging and inclusion cysts were seen centrally.Skin biopsies from papules on the thighs and chest revealed flattened epidermis centrally and on the periphery, adenoid proliferations of the rete ridges in a filiform pattern with basal hyperpigmentation, inclusion cysts and follicular plugging (Fig. 3). Parakeratosis was seen in some lesions, possibly due to excoriation. There were scattered melanophages in the upper papillary dermis. Acantholysis was not seen despite examination of multiple sections of multiple papules. S100, Melan A and HMB45 stains showed increased pigment especially at the periphery.The diagnosis of generalized Dowling-Degos disease was made on the clinical and histological findings.
DISCUSSIONDowling-Degos disease is an autosomal dominant genodermatosis with variable penetrance, but is often sporadic, as in our patient.1 Localized and generalized forms have been recognised. 2 It typically presents as an acquired hyperpigmentation, particularly of the flexures, beginning in adult life.To our knowledge, the dermatoscopic features of Dowling-Degos disease have been reported in only one case of localized Dowling-Degos disease of the vulva.
A 56-year-old man presented with tingling ulcers on the feet. On further skin examination, papules on the knees were observed. Biopsies revealed extravascular eosinophilic deposits of IgM, and Waldenström macroglobulinaemia was diagnosed. The skin manifestations have resolved with chemotherapy. Peripheral neuropathy and storage papules are rare manifestations of Waldenström’s macroglobulinaemia.
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