Inherited Localized Disorder
There is no recognized disorder in this category.
Acquired Localized Disorders
Primary localized lipoatrophies correspond to a heterogenic group of disorders whose denomination mainly depicts their clinical appearance. Insulin and centrifugal lipoatrophy can present in childhood. Annular lipoatrophy, semi-circular lipoatrophy and lipoatrophy of the ankles are clinically distinct entities mainly seen in adult women and, therefore, will only be mentioned briefly.
Centrifugal Lipoatrophy
Syn.
lipodystrophia centrifugalis abdominalis infantilis, centrifugal lipoatrophy
Definition.
Described in 1971 by Imamura and colleagues [1] under the name ‘lipodystrophia centrifugalis abdominalis infantilis’, this form of lipoatrophy is characterized by (a) a localized loss of subcutaneous fat involving the greater part of the abdomen, (b) an onset before 3 years of age, (c) a centrifugal enlargement of the depressed area, (d) slightly reddish and scaly changes in the surrounding area, and (e) no other significant abnormalities. More than 100 cases have been reported to date, mainly among Japanese [2]. Occurrence in Caucasian children appears exceptional [3].
Aetiology.
The origin of centrifugal lipoatrophy is uncertain. Although small inflammatory findings such as lymph node enlargement and peripheral inflammatory cellular infiltrate are initially present in about two-thirds of cases, systemic signs of inflammation are usually absent [2]. These findings and the fact that corticosteroids do not stop the progressive enlargement of centrifugal lipoatrophy argue against a primary inflammatory process as in other types of panniculitis. Speculation has covered several possible mechanisms: (a) a primary loss of subcutaneous fat with reactive inflammatory infiltrate and lymphadenopathy, (b) localized trauma such as friction, contusion, inguinal hernia or congenital dislocation of the hip, which have all been reported as possible triggers in some patients, and (c) intercurrent infections. The higher expression of the disorder in Japanese children, together with the description of affected dizygotic twins and siblings [4], may suggest a genetic predisposition.
Histology.
Lesions are characterized by a decrease or loss of subcutaneous fat, with the presence of inflammatory cells that are more prominent in the surrounding area. The inflammatory cell infiltrate may involve the dermis as well as the subcutaneous tissue and consists of lymphocytes, histiocytes and few plasma cells in most cases [2,3,5]. Multinucleated giant cells and foamy cells have been reported [5]. Mild vascular changes (endothelial swelling) can occur, but not apparent vasculitis.
Clinical Features.
With a 2 : 1 female : male ratio, 90% of cases are characterized by an onset before 8 years of age and an abdominal location, most often the groin or surrounding area. The initial presentation includes erythematous, bluish macules or ecchymoses with regional lymph node enlargement in about one-half of the cases. In the other half, the parents first notice the lesion only by a well-defined depression or atrophy of the skin. The lesion then spreads centrifugally to leave a central part of lipoatrophy, where subcutaneous veins become easily visible. A few patients have developed two or three lesions. In a follow-up review of cases, it was found that cessation of enlargement occurs within 3 years in 50% of patients and within 8 years in 90%, followed by spontaneous resolution or marked improvement in a majority of cases [2,5–7].
The clinical course appears rather uniform in most cases. However, a few variations were recently reported. These include extra-abdominal locations, such as the head [5,8], neck [7] and lumbar region [3,6] and non-regressing cases [9]. Adult cases are extremely rare [10–12], and whether these should be classified as large unusual semi-circular lipoatrophies is disputable.
Treatment and Prognosis.
Topical and oral corticosteroids have been used with little benefit. They are usually effective at decreasing the peripheral inflammation/erythema but do not halt the progressive centrifugal extension [2]. Although most cases spontaneously stop progressing before the age of 13 years and then regress, persistence into adulthood of a lesion further complicated by angioblastoma has been reported [9].
Annular Lipoatrophy
This entity is characterized by a circular depressed band, 1 cm wide and 0.5–2 cm deep, that encircles an upper limb, usually in women aged 40–70 years. The atrophic lesion is preceded by tenderness and swelling of the entire limb. Unexplained neuralgia and arthralgic pain with muscle weakness or myopathy are frequent. Annular lipoatrophy does not spontaneously regress and may last up to 20 years. Histological findings may be minimal or show polyarteritis and strands of connective tissue replacing the subcutaneous fat. The prevalence of ‘rheumatic’ pain and associated findings suggests an underlying connective tissue disease [13–15].
Atrophy of the Ankles
This is an extremely rare disorder, mainly characterized by its peculiar location. Less than 10 cases have been described with bilateral circumferential, asymptomatic, lipoatrophic bands, 9–11 cm wide, on the ankles. Local symptomatology and muscle involvement are absent. The disorder should be differentiated from acral lipoatrophy, which may develop as an autoimmune process (Fig. 141.2) [16–19].
Fig. 141.2 Diffuse, predominantly acral lipoatrophy in a girl with autoimmune hepatitis, alopecia and positive anti-liver and anti-kidney microsome antibodies.
Semi-Circular Lipoatrophy
Semi-circular lipoatrophy occurs more frequently than annular lipoatrophy and atrophy of the ankles, and mainly in adults. Patients present with semi-annular cutaneous depressions symmetrically distributed on the anterolateral aspects of both thighs. The lesions are asymptomatic and flesh coloured. Spontaneous resolution usually occurs within 3 years of onset. Although the aetiology of the disorder may be heterogeneous, most authors believe that the lipoatrophy follows a panniculitis of traumatic origin. Histological changes include fat atrophy replaced by collagen and mild perivascular cellular infiltrate in the dermis [20–31].
Naevoid Disorders
Localized lipoatrophy can occasionally be associated with naevoid disorders such as Becker’s naevus [32,33] or naevoid hypertrichosis [34].
Insulin Lipoatrophy
Aetiology.
Lipoatrophy following subcutaneous insulin injections was probably one of the most common causes of localized fat atrophy when diabetic patients used conventional bovine–porcine insulin preparations. As lipoatrophy was more commonly seen with longer acting insulins rather than soluble ones, and as its occurrence was greatly reduced with the availability in the early 1980s of highly purified porcine insulins [35,36], it is considered to be an immunological reaction to impurities in the insulin preparations and/or to the xenogenic insulin [37–39].
These immunological reactions should be differentiated from allergic reactions to the content of long-acting insulins, which result in generalized urticaria and not lipoatrophy [40,41].
Histopathology.
Skin biopsies show a loss of fat tissue. An increase in insulin-binding capacity is found on the edge of lipoatrophic lesions. Inflammatory changes are characteristically scant but immunofluorescence may show deposition of immunoglobulin M (IgM), C3 in the dermis and C3 in dermal blood vessels [38]. Accumulation of tryptase-positive, chymase-positive degranulated mast cells has been described with human insulin [42].
Clinical Features.
Insulin atrophy was previously seen more frequently than insulin hypertrophy, but not now. In a series of 281 patients treated with purified insulins, the prevalence of lipohypertrophy was 27% and lipoatrophy 2.5% [35]. It is a cosmetically distressing complication, which presents as a non-inflammatory, painless, small to large dimple at insulin injection sites. It usually develops within 3 years of starting insulin and is more common in children and women. Most cases are associated with higher levels of insulin requirements, as insulin absorption can be delayed or variable due to the formation of avascular, fibrous scar tissue. Lipoatrophic lesions distant from the sites of injection may occur, as well as the co-existence of both fat atrophy and hypertrophy [43].
Treatment.
The use of highly purified porcine insulins with a reinforcement of careful rotational routine of injection sites resulted in a marked decrease, but not disappearance, of insulin lipoatrophy. The use of human insulin preparations, injected directly into the lipoatrophic area, is usually curative [43,44]. This often results in both a regression of the localized lipoatrophy and a reduction in insulin requirements. However, cautious optimism should prevail as lipoatrophy may occasionally complicate human insulin injections [45]. In such cases, topical cromolyn therapy may reverse early and prevent new lipoatrophic lesions [42].
References
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