Necrobiosis lipoidica is a rare disorder that usually appears in the lower extremities and it is often related to diabetes mellitus. There are few reported cases of necrobiosis lipoidica in children. We present an interesting case in that the patient developed lesions on the abdomen, which is an unusual location.
A 16-years-old girl, with type 1 diabetes diagnosed elsewhere in 2000, was brought to our Department for a first routine visit in 2006, during which two yellow-brown, atrophic plaques surrounded by raised, violaceus rims on the right side of the lower abdomen (
Yellow-brown, atrophic plaques surrounded by raised, violaceus rims on the right side of the lower abdomen (a); particular of the skin lesions (b).
Skin lesions typically appear on the legs (a); particular of the skin lesions (b).
Necrobiosis lipoidica (NL) is an idiopathic dermatological condition that is strongly associated with diabetes mellitus, so much so that some authors name it “diabeticorum.”
NL is characterized by a rash that occurs on the lower legs and only rarely on hands, fingers, face, and scalp [
At the beginning, the lesions appeared as erythematous circle papules that then evolve to well-demarcated, atrophic, shiny, yellow-brown telangiectasic multiple and bilateral plaques. Except as they are ulcerated, the lesions are generally asymptomatic [
The pathogenesis of NL is largely enigmatic. It has been suggested that NL is one of the possible manifestations of microangiopathy, due to its clear association with diabetes. Some authors [
Few data exist in pediatric population. A recent paper by Pavlović et al. [
Some authors [
NL diagnosis is commonly a clinical one; only in those few cases, usually in the starting stages of the disease, when the diagnosis may be awkward, a biopsy of the lesion can be useful.
Treatment of NL is often difficult. First-line therapies include smoking cessation and diabetes control improvement. In addition, topical and intralesional corticosteroid may be effective [
Recently, a new therapeutic attempt has been reported, including topical PUVA photochemotherapy [
On the other side, systemic therapies using corticosteroids and azathioprine could facilitate malignant transformation [
Differential diagnosis includes granuloma annulare, sarcoidosis, and amyloidosis. Granuloma annulare has typical lesions with epidermis not thinned. Besides the histopathologic findings that may rule out the differences between the diseases, it shows resolution with near restoration of structure, normal and without sclerosis. On the other hand, necrobiosis lipoidica shows a progression to collagen bundles in the reticular dermis in septa in the subcutaneous fat becoming crowded and thickened. Sarcoidosis is a multisystem disease that may involve almost any organ system, and its dermatological findings can assume a vast array of morphologies. Correctly diagnosing sarcoidosis may be a challenge. Patients are diagnosed with sarcoidosis when a compatible clinical or radiologic picture is present, along with histologic evidence of noncaseating granulomas, and when other potential causes, such as infections, are excluded. Amyloidosis is a systemic disease too, a plasma-cell dyscrasia of unknown cause, with well-recognized dermatological signs that may be the presenting features. Skin and soft-tissue lesions may, indeed, be the only manifestations of the disease prior to later-stage organ involvement. Although the skin manifestations of systemic amyloid are common to many conditions, their presentation in certain clinical settings should help to indicate this disorder for inclusion in the differential diagnosis.
None of the authors have any financial interest related to the study to disclose.