Perforating Disseminated Necrobiosis Lipoidica Diabeticorum

Abstract

Perforating necrobiosis lipoidica is a very rare clinical variant which consists of degeneration and transepidermal elimination of the collagen with few cases reported in the literature. In two-thirds of the patients it associates with diabetes, with no relation with the glucose control. We present a 42-year-old female patient with a 7-year history of diabetes on insulin therapy, referred to our clinic with a 3-year history of multiple asymptomatic firm plaques disseminated on the upper and lower extremities. The clinical and histological findings proved the diagnosis of perforating necrobiosis lipoidica.

A case of disseminated perforating necrobiosis lipoidica

“…Approximately 90% of cases of PNL described in literature are associated with diabetes mellitus, although unrelated to glucose control . Lesions are usually located on the lower extremities, less frequently on the upper extremities and trunk.…”

Disseminated perforating necrobiosis lipoidica: a case report and literature review

“…Of the 16 cases of PNL described in the literature (2-4, 11-15), only 3 have presented with multiple lesions and can be classified as disseminated (2,12,14). Our patient is the fourth such case to be reported.…”

“…There is frequent extension into the superficial subcutis in NL. Plasma cells accompany the lymphocytic perivascular infiltrate in NL and are not a feature of GA. Eosinophils maybe seen in GA. Mucin deposition is absent in the zones of necrobiosis in NL but typically present in GA (2,3,5,6,11,14). There is discordance between Authors whether to accept transfollicular perforation within the spectrum of PGA (3,18,19), however it is widely accepted in PNL.…”

“…It was originally termed 'dermatitis atrophicans lipoidica' by Oppenheim (2), and historically also known as necrobiosis lipoidica diabeticorum. It occurs in 0.3% of diabetic adults and 0.06% of diabetic children (3), but also in patients without diabetes mellitus (4).…”

“…The perforating variant of necrobiosis lipoidica was first reported in 1977 by Parra (3,11). As with NL, this form is usually related to diabetes, and not necessarily to poor glycaemic control (2,4). To our knowledge, there have been sixteen cases of perforating necrobiosis lipoidica (PNL) published to date (2,4,(11)(12)(13)(14)(15), including two paediatric cases of patients with type1 diabetes mellitus (3,4).…”