Dowling-Degos disease associated with hidradenitis suppurativa: a case report.
نویسندگان
چکیده
Dowling-Degos disease (DDD) or reticular pigmented anomaly of the flexures is a rare autosomal dominant genodermatosis with variable penetrance. It mainly affects young women and is characterized by acquired reticular hyperpigmentation of the large skin folds. We present a case of DDD associated with hidradenitis suppurativa (HS) in a 43-year-old Spanish woman. Physical examination revealed brownish macules of 5-10mm diameter, with an irregular morphology, homogeneous pigmentation, and a reticular pattern, situated predominantly in the posterior cervical region (Fig. 1A) and inguinal and intergluteal folds (Fig. 1B). In addition, she presented active HS lesions in both axillas (Fig. 2), as well as depressed pinpoint perioral scars suggestive of acne. The patient stated that her mother, 2 maternal uncles, and 1 of her sisters had similar lesions. The dermatopathology study of a perianal skin lesion revealed basal hyperpigmentation, thin elongated digiform rete ridges, and suprapapillary thinning, as well as dermal melanophages and amild perivascular lymphohistiocytic infiltrate (Fig. 3). Based on these data, we made a diagnosis of DDD with associated HS. We offered topical therapy as the first option, but this was rejected by the patient. The disease has shown no significant change after more than 5 years of follow-up. DDD is a rare autosomal dominant genodermatosis that was first described by Behçet in 1932 as a variant of acanthosis nigricans of the axillas. Since then a number of cases of DDD have been reported, and there have been further revelations about its etiology, pathogenesis, and treatment.
منابع مشابه
Hidradenitis suppurativa, Dowling-Degos and multiple epidermal cysts: a new follicular occlusion triad.
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 ...
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Dowling –Degos disease is a rare condition. It is inherited by an autosomal dominant gene. It usually presents in adult life as small, pigmented, asymptomatic macules in flexural regions. We report a 35-year-old woman with Dowling- Degos disease, in whom the reticular pigmentation confined to the genital area.
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Dowling-Degos disease is a rare sporadic or autosomal dominant pigmentary entity, in which clusters of papules and reticulate macules slowly develop with predominance in flexural regions. This entity is due to mutations in the keratin 5 gene, and is related with other cutaneous disorders. We report the sporadic form of Dowling-Degos disease in an elderly man with multiple seborrheickeratosis ...
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ورودعنوان ژورنال:
- Actas dermo-sifiliograficas
دوره 106 4 شماره
صفحات -
تاریخ انتشار 2015