|Year : 2019 | Volume
| Issue : 11 | Page : 1626-1628
Necrobiosis lipoidica in a Nigerian woman – Report of a case
Dermatology Unit, Department of Medicine, Kaduna State University/Barau Dikko Teaching Hospital, Kaduna, Nigeria
|Date of Submission||21-Jan-2019|
|Date of Acceptance||15-Jun-2019|
|Date of Web Publication||13-Nov-2019|
Dr. H Yahya
Dermatology Unit, Department of Medicine, Barau Dikko Teaching Hospital, PO Box 9727, Kaduna
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Necrobiosis lipoidica (NL), one of a group of “necrobiotic” granulomatous cutaneous disorders, is characterized histologically by layers of poorly defined, horizontally oriented, altered “necrobiotic” collagen surrounded by, and alternating with, layers of chronic inflammatory cells, histiocytes, and giant cells throughout the full thickness of the dermis. It is a rare disease associated mostly with diabetes mellitus in whom it may affect 0.3%–1.2% although it has also been associated with other cutaneous and systemic disorders and may occur in otherwise normal people. NL has been reported from all over the world, but there appears to be very few reports of NL in Black Africans. We report the case of a 55-year-old diabetic Nigerian woman who presented with typical NL lesions on the lower legs and who responded very well to topical betamethasone dipropionate 0.05% cream. We also briefly review the probable underlying mechanisms leading to the condition and the various treatments that have been found useful.
Keywords: Black Africans, diabetes, necrobiosis lipoidica, necrobiosis lipoidica diabeticorum, Nigerian woman
|How to cite this article:|
Yahya H. Necrobiosis lipoidica in a Nigerian woman – Report of a case. Niger J Clin Pract 2019;22:1626-8
| Introduction|| |
Necrobiosis lipoidica (NL), one of a group of cutaneous disorders termed “necrobiotic granulomas,” is a rare disease characterized histologically by full-thickness dermal areas of poorly defined, horizontally oriented altered “necrobiotic” collagen alternating with broad areas of intervening perivascular inflammatory reaction, made predominantly of lymphocytes, plasma cells, and occasional eosinophils, giving a sandwich appearance. The necrobiotic areas are often surrounded by a variable histiocytic infiltrate with multinucleated giant cells and there may be variable fibrosis. NL has traditionally been associated with diabetes, hence the old name “necrobiosis lipoidica diabeticorum” but is now recognized that only 0.3%–1.2% of diabetics develop the condition and the disease may occur without diabetes or in association with other diseases. Although NL is a well-known disease and has been reported from all over the world, there have been few reports in Black people and especially Black Africans.,, We report a case of the disease in a 55-year-old Nigerian woman who presented with NL and briefly discuss the suggested underlying mechanisms and treatment modalities. We hope this adds to the literature on the subject.
| Case Report|| |
A 55-year-old Nigerian woman presented to the skin clinic with a 1 year history of asymptomatic skin lesions on her lower legs which started as small lesions but increased in size and number. She was diagnosed with diabetes mellitus 6 years earlier and placed on glucophage and glibenclamide. Her latest fasting blood sugar was 6.4 mmol/L (normal range: 4.0–5.9 mmol/L) and glycosylated hemoglobin was 8.3% (normal range: 4.6%–6.5%). She was otherwise very well. She did not have a history suggestive of any autoimmune disease. There was no family history of diabetes. On examination, she weighed 74 kg and her height was 1.65 m (body mass index 27.2); she had multiple, dark brown flat papules and plaques as well as multiple single to confluent, well-defined annular plaques of various sizes on all sides of both her lower legs. Large plaques had slightly raised irregular hyperpigmented edges and hypopigmented, atrophic, yellowish center with prominent telangiectasia in some lesions [Figure 1]. There was no ulceration in any of the lesions. Examination of the rest of the skin and other systems was unremarkable. She did not have retinopathy. Histological examination of a 4-mm punch skin biopsy from a lesion edge confirmed the clinical suspicion of NL [Figure 2]. Chest radiograph, serum chemistries, and lipid profile and thyroid function tests were all normal. There was no proteinuria. She was treated with topical betamethasone dipropionate 0.05% cream applied overnight to the edges of the lesions under polythene occlusion. Two months later, lesions had flattened, became less pigmented, and the hypopigmented centers had improved markedly [Figure 3]. She was instructed to avoid trauma to the lesions using support stockings.
|Figure 1: Clinical photograph of necrobiosis lipoidica on the left lower leg showing darkly pigmented papules, plaques, and a large annular lesion with hypopigmented atrophic center and prominent telangiectasia|
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|Figure 2: Photomicrograph of necrobiosis lipoidica showing horizontally oriented necrobiotic collagen bundles alternating with layers of chronic inflammatory infiltrate in the dermis (H and E, ×100)|
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|Figure 3: Clinical photograph showing marked improvement of necrobiosis lipoidica lesions on the left lower leg after treatment with topical betamethasone dipropionate 0.05% cream|
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| Discussion|| |
Our case is a typical presentation of NL. NL affects three times as many women as men. Although lesions occur on the lower legs in most patients, there have also been reports of lesions on the scalp, face, back of hands, and forearms. Granuloma annulare, necrobiotic xanthogranuloma, and sarcoidosis may sometimes produce lesions on the legs that are indistinguishable clinically from NL, but histological examination is often sufficient to differentiate the various conditions. Ulceration may occur in up to 35% of cases, is often induced by trauma, and can be difficult to treat. Squamous cell carcinoma has been reported to occur in some long-standing cases.
NL has typically been associated with diabetes. Muller and Wilkelmann, in a large study involving 171 patients, found 62% of cases had diabetes for a variable period at presentation; in 24%, the two conditions were diagnosed at the same time; and in 14%, diagnosis of NL preceded diabetes. In addition, there was a family history of diabetes in 43% with NL compared with 26% in those without diabetes. In diabetes-related NL, those with type 1 disease tended to present in the third decade of life, while those with type 2 present in the fifth decade or later as were those who did not have diabetes at presentation. Although the precise cause of NL is still not clear, its strong association with diabetes suggests that the two conditions may share identical pathogenesis which is believed to be due to microangiopathy as a result of glycogen deposition in dermal blood vessels leading to thickening of the vessels ; microangiopathic complications of diabetes such as retinopathy and proteinuria were more common in diabetic patients with NL in one study than those without. Other abnormalities that may play a role in some cases include disorders of collagen, immune complex deposition at the dermoepidermal junction, and defective neutrophil migration. NL has rarely been associated with granulomatous conditions such as sarcoidosis, and histologically confirmed cases of sarcoidosis have presented with annular lesions on the legs that were indistinguishable clinically from NL. Furthermore, it may be difficult to distinguish sarcoidosis and NL histologically in this instance as the two conditions may have elements of the other; Lee et al. suggest that the two conditions may represent the spectrum of the same condition. NL has also occurred in association with other granulomatous conditions such as Crohn's disease and granuloma annulare. Other rare associations include autoimmune thyroid disease and ulcerative colitis.
There are many treatment options with variable successes that have been used in NL. This has recently been well reviewed by Sibbald et al. Avoiding trauma to lesions on the lower legs, including using support stockings, is of utmost importance. Ulcers are treated with standard wound care practices including controlling edema and infections. Topical or intralesional steroids applied to the active edges have been the main stay of treatment although a short course of systemic corticosteroids may be required. Topical tacrolimus has also been used with good effect, while topical tretinoin has shown promise in improving discoloration and atrophy of lesions., Phototherapy and photodynamic therapy, and aspirin alone or in combination with dipyridamole are other modalities of treatment which have been used. In recalcitrant cases, systemic treatments such as hydroxychloroquine, methotrexate, cyclosporine, pentoxyphylline, mycophenolate mofetil, clofazimine, thalidomide, biologics, and fumaric esters have been used alone or in combination with topical agents., Excision of lesions is best avoided because of Kobnerization. Appropriate management of underlying diabetes, if present, is desirable although it is still not clear whether this influences the healing of lesions of NL.,,
| Conclusion|| |
Although NL is a well-known condition, it is rare worldwide and has been reported even more infrequently in Black Africans. However, as our case shows, the clinical presentation, settings of its occurrence, and response to treatment are similar.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]