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.
Necrobiosis lipoidica diabeticorum is a rare idiopathic dermatological condition, commonly seen in women and frequently associates with diabetes. The perforating variant of disease in which necrotic collagen eliminates via transfollicular perforations is very seldom.
We present a case of type II diabetes patient with disseminated perforating necrobiosis lipoidica (PNL). According to our knowledge this is one of very few cases published in the literature.
A 42-year-old Caucasian female patient suffering from long-term diabetes mellitus type II, controlled with rapid and retard acting insulin, was referred to our clinic. She complained of multiple asymptomatic firm plaques on the upper and lower extremities which enlarged peripherally and formed dark-brown centrally depressed plaques that appeared about 3 years ago. There was no history of trauma or operative interventions on the involved areas. One year later multiple reddish papules appeared on the upper extremities and slowly enlarged formatting indurated plaques.
The clinical examination revealed infiltrated plaques disseminated on the extensor surfaces of the upper and lower extremities. The lesions on the lower extremity were presented by brown-yellow plaques with different sizes, irregular shape, sharp slightly elevated border, and atrophic center focally studded with comedo-like papules (Figures
Infiltrated circinate plaques, erythematous border, and comedo-like papules in the center on the lower extremities of a 42-year-old diabetic patient.
Close view of an irregularly shaped brown-yellow plaque with comedo-like papules.
Systemic examination showed no diabetic retinopathy or neuropathy; arterial hypertension was controlled with propranolol with only single measurement of RR 150/90 mm/Hg at admission.
Laboratory investigations however revealed moderate anemia of 85 g/L and ketone bodies in urine. Blood sugar levels were fasting 4.2 mmol/L and postprandial 16.2 mmol/L. The patient was consulted with an endocrinologist, and insulin treatment was corrected.
The histological examination demonstrated degenerated collagen and fibrosis with peripheral lymphohistiocytic infiltrate (Figure
Histological examination demonstrated degenerated collagen and fibrosis with a peripheral lymphohistiocytic infiltrate in dermis (haematoxylin and eosin, original magnification ×200).
Histology demonstrates transepidermal elimination of necrotic material (haematoxylin and eosin, original magnification ×100).
The differential diagnosis included granuloma annulare, sarcoidosis, necrobiotic xanthogranuloma, lichen sclerosus et atrophicus, Darier-Roussy sarcoid, and erythema induratum of Bazin; however the clinical and histological findings were compatible with disseminated perforating necrobiosis lipoidica diabeticorum.
Initiated treatment with topical steroids was ineffective. Intralesional corticosteroid therapy was discussed but not started as the patient was lost to followup.
Necrobiosis lipoidica diabeticorum was initially described by Oppenheim in 1929 [
PNL belongs to the group of perforating disorders with transfollicular and transepidermal elimination of degenerated collagen or elastin as
PNL associates in about 90% of cases with diabetes mellitus; however it is unrelated to the glucose control [
There are two histopathological types of necrobiosis lipoidica—a necrobiotic and a granulomatous one. The diabetic variation expresses necrobiotic changes, whereas in the nondiabetic form the granulomatous changes are present. In the necrobiotic type a necrobiosis of the collagen is present in the deep dermis with deposit of mucin and mixed inflammatory infiltrate consisting of lymphocytes, plasmocytes, histiocytes, fibroblasts, and epithelioid cells [
The local and systemic treatment often gives unsatisfactory results. In some cases the surgical therapy is useful, however in other it could provoke the Köbner phenomenon. The local therapy consists of the application of local corticosteroid [
In conclusion, we present a rare case of a 42-year-old patient with disseminated perforating necrobiosis lipoidica diabeticorum. Although the disease has benign course, the therapeutic resistance and uncontrolled diabetes may cause serious outcome.