Annular lipoatrophy of the ankle: Four new pediatric cases and a review of the literature

Abstract

Annular lipoatrophy of the ankle (ALA) is a rare acquired lipoatrophic panniculitis first reported in 1953 by Ferreira-Marques by the name lipoatrophia annularis 1 and then described by Winkelmann as connective tissue panniculitis (CTP), 2 but it would appear that ALA is a more localized form of CTP. 3 It mainly affects children who are otherwise healthy but may present with autoimmune diseases 3-5 and has a stereotyped presentation, starting with inflammatory nodules or plaques around the ankles which progress into circumferential subcutaneous atrophy within a few months. There is no consensus on treatment, and long-term outcomes are poorly documented. We present four new cases of childhood ALA, including a patient with an extended follow-up of more than 10 years. | C A S E REP ORTSFour children (two boys and two girls), aged between 5 and 9 years, were diagnosed with ALA. All were otherwise healthy. Their characteristics are presented in Table 1. ALA has a classic history starting with inflammatory nodules or plaques around the ankles and calves evolving over months to subcutaneous atrophy. Some had triggering events, such as Patient 1 (Figure 1) who reported the appearance of lesions after wearing a new pair of high-top shoes that may have caused local trauma. All had a classic history of ALA except for Patient 2, who reported no initial inflammatory stage. Only, Patient 1 reported initial debilitating symptoms (severe cramps and myalgia).The lesions were located on the lower extremities for all patients except for Patient 3 (Figure 2), who had mild lesions on the buttocks.

Extensive Progressive Atrophy of Lower Extremities in an Adult Woman: Answer

“…The etiopathogenesis is not yet completely clear. [1][2][3][4][5][6] Infectious causes have been suggested, and a potential role of autoimmunity, especially that several autoimmune disorders have been associated with this panniculitis such as Hashimoto's thyroiditis, Grave's disease, rheumatoid arthritis, type I diabetes mellitus vitiligo, alopecia areata, among others. Antinuclear antibody-positive serology has also been reported in association.…”

“…2 Lipophagic panniculitis needs to be differentiated from several entities that can mimic it clinically and histopathologically, such as lupus profundus, subcutaneous panniculitis-like T-cell lymphoma (SPTCL), gamma-delta T-cell lymphoma, subcutaneous morphea, localized involutional lipoatrophy, and cytophagic histiocytic panniculitis. [1][2][3][4][5][6][7][8] Lupus profundus typically presents as firm, deep, tender erythematous nodules and plaques that heal with lipoatrophy. Microscopically, it exhibits a lymphocytic lobular panniculitis with secondary lobular hyalinization and sclerosis, lymphoid follicles, and commonly with overlying changes of discoid lupus erythematosus.…”

“…First described in 1980, Umbert and Winkelmann reported on a distinctive inflammatory panniculitis case that they termed lipophagic granulomatous lipoatrophy. 1–6 Later, several similar cases were reported using other terms such as annular atrophic panniculitis of the ankles, lipoatrophic panniculitis, lipophagic panniculitis, annular and semicircular lipoatrophy, abdominal lipoatrophy, and connective tissue panniculitis. 2–6 A hallmark characteristic of this unique panniculitis, which has mostly been described in children and women, 1–6 is a clinical evolution from a nodular/infiltrative inflammatory plaque-like phase typically associated with constitutional symptoms to a late residual irreversible atrophy.…”

“…1–6 Later, several similar cases were reported using other terms such as annular atrophic panniculitis of the ankles, lipoatrophic panniculitis, lipophagic panniculitis, annular and semicircular lipoatrophy, abdominal lipoatrophy, and connective tissue panniculitis. 2–6 A hallmark characteristic of this unique panniculitis, which has mostly been described in children and women, 1–6 is a clinical evolution from a nodular/infiltrative inflammatory plaque-like phase typically associated with constitutional symptoms to a late residual irreversible atrophy. Ankles and legs are commonly affected, although proximal extremity and diffuse involvement can occur.…”