case 17
DESCRIPTION
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 - PowerPoint PPT PresentationTRANSCRIPT
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Case 17Case 17
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* Female, 6 years old.
* Annular lesions in both legs from one month.
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* High erythrocyte sedimentation rate (54 mm).
* Reactive C protein: 80 mg/L.
* Rheumatoid factor: 21.4 IU/mL.
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CD4
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CD8
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Ki67
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Case 17Case 17
Diagnosis: ?
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Case 17Case 17
Diagnosis: lipoatrophic panniculitis of the ankles in childhood.
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1) Gilchrist T.C. et al. John Hopkins Hosp Bull 1916;27:291-294.2) Makai E. Klin Wochenschr 1928;7:2343-2346.3) Cummins L.J. et al. Arch Dermatol Syphil 1938;38:415-426.4) Pinetti P. G Ital Dermatol Siffilo 1941:82:297-340.5) Larkin V de P et al. Am J Dis Child 1944;67:120-125.6) Duperrat B. Med Infant (Paris) 1960;2:5-8.7) Fournier A. et al. Pediatrie 1965;20:595-600.8) Témime P. et al. Bull Soc Franc Dermatol Syphiligr 1968;75:206-207.9) Undeutsch et al. Hautarzt 1970;21:221-225.10) Shelley et al. Arch Dermatol 1970;102:326-329. *11) Stanoeva L. et al. Dermatologica 1972;145:48-55.12) Jablonska S. et al. Acta Derm Venereol 1975;55:135-140.13) Nowińska-Lebiosa H. et al. Przagl Dermatol 1977;64:579-482.14) Taylor G.A. Clin Pediatr (Phila) 1981;20:521-523.15) Edge J. et al. Eur J Pediatr 1986;145:227-229.16) Nelson H.M. Clin Exp Dermatol 1988;13:111-113.17) Moragon M. et al. Eur J Pediatr 1986;145:227-229.18) Winkelmann R.K. J Am Acad Dermatol 1989;21:971-978.19) Roth et al. J Am Acad Dermatol 1989;21:1152-1156.20) Melchiorre L.P. et al. J Rheumatol 2000;27:504-506.21) Martinez A. et al. Br J Dermatol 2000;142:1034-1039.22) Masala M.V. Dermatology 2001;203:81-82.23) Falcini F. et al. Clin Exp Rheumatol 2002;20:432.24) Mirza B. et al. Australas J Dermatol 2006;47:49-52.25) Madasseri A. et al. Clin Exp Dermatol 2006;31:303-305.26) Dimson O.G. et al. J Am Acad Dermatol 2006;54:S40-S42.27) Shen et al. Arch Dermatol 2010;146:877-881.28) Kerns M.J. et al. Pediatr Dermatol 2011;28:142-145. 29) Corredera et al. Pediatr Dermatol 2011;28:146-148.30) Santonja et al. Am J Dermatopathol 2012;34:295-300.31) Fernandez-Flores et al. Am J Dermatopathol 2012: Epub ehead of print.
Reports of localized lipoatrophy invoving the ankles Reports of localized lipoatrophy invoving the ankles and lower legsand lower legs
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•Annular lipoatrophy of the ankles•Lipoatrophic and lipophagic panniculitis of childhood•Connective tissue panniculitis•Cytophagic panniculitis•Lipoatrophy annularis
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Lipoatrophic panniculitis: after inflammation.
Contrary to lipodystrophy: no previous inflammation
First concept: lipoatrophyFirst concept: lipoatrophy
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• Extense variants:• Congenital total.• Acquired total.• Cephalothoracic.• Parcial face-sparing.
• Localized:• Semicircular atrophy of the thighs.• Centrifugalis abdominalis infantilis.
• Annular lipoatrophy of the ankles..
Second concept: localizedSecond concept: localized
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Third concept: etiology?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.
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The entity has a well defined clinical The entity has a well defined clinical presentation: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.
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The entity has a well defined morphology: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
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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.”
CuriosityCuriosity
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ImmunohistochemistryImmunohistochemistry
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.
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