NECROBIOSIS LIPOIDICA

Necrobiosis lipoidica


Necrobiosis lipoidica (NL) (formerly necrobiosis lipidica diabeticorum) is an idiopathic granulomatous skin disorder typically associated with diabetes. The etiology is probably multifactorial with microangiopathy, immune complex formation, abnormal collagen synthesis and breakdown, and altered hemostasis all thought to play a part.

Clinical

Well-demarcated, yellow-red plaques with epidermal atrophy on the shins is characteristic. Initially a red plaque forms. As it spreads, the center becomes depressed and yellow with telangiectasias. Severe ulceration is not uncommon. Lesions occurring in sites other than the legs occur in about 15% of patients. Partial alopecia, hypohidrosis and anesthesia have been reported within the plaques. Occasionally, overlap cases with granuloma annulare have been reported. In one review of 100 cases [Eur J Dermatol. 2015 Nov 12], the average age was 50 years (15-95 range), 77% of patients were women, ulceration occurred in 33% and thyroid disorders were found in 15% of cases.

Treatment

Therapy may be difficult. Control of diabetes does not help. An occluded high potency topical steroid may be tried. Alternatively, IL triamcinolone 3 mg/cc, and prednisone have been used successfully. Topical tacrolimus cleared on woman's facial NL over a year [JEADV 2016;30;381]. Both pentoxifylline [Australas J Dermatol 2015 Nov 12], and cyclosporine [Hautarzt 2007;58:684-8] boast multiple reports of their efficacy. Other therapies include ASA, dipyridamole, ticlopidine, clofazamine, fibrinolytic agents, hyperbaric oxygen, infliximab, phototherapy (PUVA and UVA1), topical heparin gel and nicotinamide. Surgical excision or grafting has been done. One refractory case was treated successfully with topically applied bovine collagen. One case of ulcerative NLD responded well to clofazamine. Another responded to topical becaplermin (Regranex) [CED 2013;38;745-7]. Hydroxychloroquine has been reported helpful. One case of widespread ulcerative NL was treated with 6 sessions of topical ALA-PDT at two weeks intervals. At the end of the treatment a dramatic improvement of the clinical features was observed, with complete healing of cutaneous ulcers and marked reduction of erythema in all the treated areas [Photodiagnosis Photodyn Ther. 2014 Dec;11(4):516-8]. Finally, thalidomide has been used.

Additional Pictures

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