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Case 17

Case 17. * Female, 6 years old. * Annular lesions in both legs from one month. * High erythrocyte sedimentation rate (54 mm). * Reactive C protein: 80 mg/L. * Rheumatoid factor: 21.4 IU/mL. CD4. CD8. Ki67. Case 17 Diagnosis: ?. Case 17

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Case 17

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  1. Case 17

  2. * Female, 6 years old. * Annular lesions in both legs from one month.

  3. * High erythrocyte sedimentation rate (54 mm). * Reactive C protein: 80 mg/L. * Rheumatoid factor: 21.4 IU/mL.

  4. CD4

  5. CD8

  6. Ki67

  7. Case 17 Diagnosis: ?

  8. Case 17 Diagnosis: lipoatrophicpanniculitis of theankles in childhood.

  9. Reports of localizedlipoatrophyinvovingtheankles and lowerlegs • Gilchrist T.C. et al. John Hopkins Hosp Bull 1916;27:291-294. • Makai E. KlinWochenschr1928;7:2343-2346. • Cummins L.J. et al. ArchDermatolSyphil1938;38:415-426. • PinettiP. G ItalDermatolSiffilo1941:82:297-340. • Larkin V de P et al. Am J DisChild1944;67:120-125. • Duperrat B. MedInfant (Paris) 1960;2:5-8. • Fournier A. et al. Pediatrie1965;20:595-600. • Témime P. et al. Bull SocFrancDermatolSyphiligr1968;75:206-207. • Undeutsch et al. Hautarzt 1970;21:221-225. • Shelley et al. ArchDermatol1970;102:326-329. * • Stanoeva L. et al. Dermatologica 1972;145:48-55. • Jablonska S. et al. Acta DermVenereol1975;55:135-140. • Nowińska-Lebiosa H. et al. PrzaglDermatol1977;64:579-482. • Taylor G.A. ClinPediatr (Phila) 1981;20:521-523. • Edge J. et al. Eur J Pediatr1986;145:227-229. • Nelson H.M. ClinExpDermatol1988;13:111-113. • MoragonM. et al. Eur J Pediatr1986;145:227-229. • Winkelmann R.K. J Am AcadDermatol1989;21:971-978. • Roth et al. J Am AcadDermatol1989;21:1152-1156. • Melchiorre L.P. et al. J Rheumatol2000;27:504-506. • Martinez A. et al. Br J Dermatol2000;142:1034-1039. • Masala M.V. Dermatology2001;203:81-82. • Falcini F. et al. ClinExpRheumatol2002;20:432. • Mirza B. et al. Australas J Dermatol2006;47:49-52. • Madasseri A. et al. ClinExpDermatol2006;31:303-305. • Dimson O.G. et al. J Am AcadDermatol2006;54:S40-S42. • Shen et al. ArchDermatol2010;146:877-881. • Kerns M.J. et al. PediatrDermatol 2011;28:142-145. • Corredera et al. PediatrDermatol2011;28:146-148. • Santonja et al. Am J Dermatopathol2012;34:295-300. • Fernandez-Flores et al. Am J Dermatopathol2012: Epubehead of print.

  10. Annular lipoatrophy of the ankles • Lipoatrophic and lipophagic panniculitis of childhood • Connective tissue panniculitis • Cytophagic panniculitis • Lipoatrophy annularis

  11. First concept: lipoatrophy Lipoatrophicpanniculitis: after inflammation. Contrary to lipodystrophy: no previous inflammation

  12. Second concept: localized • Extense variants: • Congenital total. • Acquired total. • Cephalothoracic. • Parcial face-sparing. • Localized: • Semicircular atrophy of the thighs. • Centrifugalis abdominalis infantilis. • Annular lipoatrophy of the ankles.

  13. Third concept: etiology? • Previous infections • Connective tissue disease Many of these patients have had a history of autoimmune antibodies and autoimmune diseases, such as diabetes and thyroid diseases.

  14. The entity has a well defined clinical presentation: • Recurrent crops of • eythematous nodules or plaques • involving mainly the legs, especially the ankles • but also sometimes accompanying lesions in the arms. • They develop lipoatrophy. • Associated laboratory findings: • Elevated ESR. • Thrombocytosis. Microcytic anemia.

  15. The entity has a well defined morphology: • Lobular panniculitis. • Predominance of histiocytes, many of them foamy, including Touton-like lipophages. • Some lymphocytes, neutrophils, plasma cells and eosinophils. • No necrosis. • No vasculitis. • No mucin

  16. Curiosity Shelley et al. Arch Dermatol 1970;102:326-329. “One year after the lesions were initially evidenced, they had become atrophic, with an increase in the venous pattern and hair growth.”

  17. Immunohistochemistry Umbert I.J. and Winkelmann R.K. Br J Dermatol 1991;124:291-295: - CD3+. CD4+ (30-40%) with some CD8+. Martinez A. et al. Br J Dermatol 2000;142:1034-1039: - Predominance of CD3 lymphocytes. Santonja et al. Am J Dermatopathol 2012;34:295-300: - CD4/CD8 even. - High proliferative activity with MIB-1 (early stage?) (This case also presented lymphocytic rimming) Fernandez-Flores et al. Am J Dermatopathol 2013;35:524-526. - Low proliferation rate (MIB-1 10%). - CD4/CD8:70/30%. - No loss of CD2, CD7, CD5. - Groups of PD-1 + cells.

  18. Thankyou

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