Lipoatrophic Panniculitis of the Ankles in Childhood

Abstract

We describe the clinical, histopathologic, immunohistochemical, and molecular findings in an additional case of lipoatrophic panniculitis selectively involving the ankles in a 12-year-old boy. This idiopathic, rarely reported entity is presumed to have an autoimmune pathogenesis. Histological findings are a lipophagic lobular panniculits, that in our case featured focally increased numbers of lymphocytes rimming the adipocytes, and infiltration of vascular walls by mildly atypical lymphoid cells, raising the differential diagnosis of subcutaneous panniculitis-like T-cell lymphoma.

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.…”

Subcutaneous Fat Necrosis During Nivolumab Therapy for Metastatic Melanoma

“…Recognition of this novel and rare reaction is important, as it may mimic metastatic disease, panniculitis (erythematous subcutaneous nodules/plaques mimicking erythema nodosum), eosinophilic fasciitis (early edema followed by woody induration and tethered appearance to skin), or acquired partial lipodystrophy (central obesity, facial and lower extremity lipohypertrophy with associated hyperglycemia, hypertriglyceridemia, and nonalcoholic steatohepatitis), all of which may occur during PD-1 inhibitor therapy . Lipoatrophic panniculitis has been reported previously and could also overlap in pathogenesis with fat necrosis . While imaging such as PET CT may be nonspecific and result in increased signal intensity, histopathology, including fascial biopsy, should be obtained to establish an accurate diagnosis and rule out metastatic disease.…”

A case of lipoatrophic panniculitis in a 2-year-old boy

“…In our case, the patient was previously diagnosed with morphea, probably because of lack of experience in recognizing lipoatrophic panniculitis, which is rare. As described by some authors, it may be difficult to distinguish between lipoatrophic panniculitis and panniculitis of Weber-Christian or its variant – Rothmann-Makai panniculitis [ 10 ]. Histopathological examination of the deep skin biopsy seems to be crucial for differentiation.…”