Lipoatrophic Connective Tissue Panniculitis

Abstract

Many causes of what was formerly called Weber-Christian and Rothmann-Makai diseases are being clarified as specific forms of panniculitis. Among them, an autoimmune process involving the subcutaneous fat without criteria for another defined disorder coined "connective tissue panniculitis" by Winckelman et al in 1980 has been described. We describe this disease in a 4-year-old boy who presented with multiple subcutaneous inflammatory nodules that extended in an annular fashion, resolved leaving lipoatrophy, with recurrence 8 years later. The histologic findings were consistent with a granulomatous lipophagic panniculitis. We review previous reports and emphasize the limited therapeutic options, chronic evolution, severe esthetic sequelae and possible association with other autoimmune disorders of this uncommon condition.

Acquired idiopathic lipoatrophic panniculitis in a 12‐month‐old infant

“…Lipoatrophy is a term describing the lack of adipose tissue, which can be partial or generalised, and present at birth or acquired later in life . Lipoatrophic panniculitis is a rare condition affecting mainly children aged between 3 and 13 years of age . A characteristic form of lipoatrophic panniculitis localised to the ankles and described mainly in children has been named annular lipoatrophic panniculitis of the ankles .…”

“…It has been postulated that an autoimmune mechanism may be involved, as many of the reported cases of lipoatrophic panniculitis have shown an association with autoimmune disorders, including Hashimoto's thyroiditis, Graves' disease, juvenile rheumatoid arthritis, insulin‐dependent diabetes mellitus and alopecia areata . Circulating autoantibodies, namely ANA, have been demonstrated in some patients . Another report of lipoatrophic panniculitis in association with a chromosome 10 abnormality suggested a possible association with human pancreatic lipase gene, which maps to the same chromosome …”

Update on management of connective tissue panniculitides

“…These may also be variants of connective tissue panniculitides 73,79,80 . Response to therapies such as prednisone, hydroxychloroquine, dapsone, azathioprine and methotrexate support this possible relatinship 81 .…”

“…Other diseases, such as annular lipoatrophy of the ankles and lipoatrophic panniculitis of childhood, are uncommon childhood conditions characterized by striking circumferential atrophy and a lymphocytic lobular panniculitis sometimes accompanied by lipophagocytosis. These may also be variants of connective tissue panniculitides (73,79,80). Response to therapies such as prednisone, hydroxychloroquine, dapsone, azathioprine, and methotrexate support this possible relationship (81).…”

Panniculitis as the first sign of systemic lupus erythematosus

“…Panniculitis is inflammation of subcutaneous adipose tissue and may be a sign of systemic disease. [2] Lupus panniculitis is an unusual variant of lupus erythematosus occurring in 2%–5% of patients. Conversely, 10%–15% of the patients with lupus panniculitis develop SLE.…”