Necrobiosis Lipoidica

Arun Chakrabarty, MD, Tania J. Phillips, MD, FRCPC

Introduction
Presentation A 51-year-old Caucasian woman presented to the clinic complaining of bilateral lowerextremity swelling and recurrent cutaneous ulcers of the right leg. A previous biopsy of the affected area was consistent with necrobiosis lipoidica (NL). She had been applying aluminum acetate soaks and high-potency topical steroid creams to the affected area. There was a history of cellulitis in the past treated with oral antibiotics. Her past medical history was significant for asthma and tonsillectomy. She denied a personal history of diabetes or any other endocrinological disorder. The patient quit smoking nearly five years previously. The patient's medications included albuterol inhaler, budesonide inhaler, hydrochlorothiazide, sulfamethoxazole, and azithromycin. The patient reported an allergic reaction to amoxicillin-clavulanate. Her family history was positive for breast cancer, diabetes mellitus type 2, and coronary artery disease. Physical Examination Physical examination revealed a morbidly obese woman, approximately 310 pounds and fivefeet two-inches tall. Head and neck examination were normal. There was no evidence of thyroid enlargement. She had numerous skin tags and seborrheic keratoses on the chest. There was a surgical scar from a benign mole removal. She had bilateral pitting edema on examination of the lower extremities. There was lipodermatosclerosis, erythema, atrophie blanche, and scaling affecting the bilateral ankles and pretibial regions. There were also a few superficial open lesions on the right shin, which were slightly tender. Peripheral pulses and capillary refill time were normal. There were no signs of cellulitis nor evidence of peripheral neuropathy.

This figure illustrates a typical necrobiosis lipoidica diabeticorum lesion. Notice the discrete erythematous plaque with the waxy, telangiectatic center. Investigations A biopsy illustrated a granulomatous inflammatory reaction with deterioration of the collagenous bundle pattern and preliminary stages of sclerosis of the deep dermis. A basic chemistry panel, urinalysis, and complete blood count except for a diminished platelet count (113; Norm: 130-400) were normal. The ankle brachial index was greater than 1.0, and duplex ultrasound depicted evidence of mild venous insufficiency. Diagnosis The clinical distribution of the lesions combined with the histologic diagnosis were consistent with the diagnosis of NL, since there was no documented history of diabetes. Necrobiosis lipoidica diabeticorum (NLD) is the same condition occurring in diabetic patients. Discussion NLD appears as well-circumscribed, erythematous plaques with waxy, telangiectatic centers. Histologically, there is a degeneration of collagen in the dermis and subcutaneous fat with concomitant granulomatous infiltration of the lower dermis. NLD was first described in diabetic patients and has been associated with the disease. NLD can precede the diagnosis of diabetes, thereby alerting the physician to screen for the diagnosis of diabetes. A few studies reported that around 0.3 to 0.7 percent of people with diabetes develop the lesions of NLD. When the condition occurs in nondiabetic patients, the preferred term has been necrobiosis lipoidica. Women have been more commonly affected than men by approximately 3:14. The vast majority of lesions occur bilaterally on the shins. When NLD lesions have occurred in other areas of the body, such as the face or trunk, there has been less of a relationship with diabetes. One-third of the lesions can ulcerate, often precipitated by trauma. The rest of the lesions have been documented to undergo spontaneous remission with residual cutaneous atrophy and scarring. The pathogenesis of NLD has not been clearly elucidated. A few studies have reported a connection between NLD and the microvascular effects of diabetic nephropathy and retinopathy. Most common theories have described a neuropathy with microvascular changes leading to the degeneration of collagen. Treatment There has been no reported standard therapy for the management of NLD. Most of the literature has focused on anecdotal reports. Common therapies include nonsteroidal inflammatory agents and topical and intralesional corticosteroids. A randomized, doubleblinded Swedish trial of combination aspirin and dipyridamole versus placebo did not reveal any benefit. The successful treatment of ulcerated NLD with pentoxifylline has been documented. One case report mentioned the significant improvement of NLD with pulsed dye laser with decrease in erythema and telangiectasia. Retinoids, such as tretinoin, have been shown to have some benefit in the treatment of NLD. Much still remains to be discovered about the pathogenesis and treatment options of NLD.

Patient Management In this case, the ulcerated areas of the patient's lower extremities were treated with hydrocolloid dressings and compression bandages. The lesions were treated with topical corticosteroid creams and improved over several months. The patient will continue to be monitored for diabetes.