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2013, Number 3

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Rev Cubana Hematol Inmunol Hemoter 2013; 29 (3)

Progressive hemifacial atrophy or Parry Romberg Syndrome associated with immunodeficiency

Sánchez SMC, Marsán SV, Macías AC, García GA, Valcárcel LJ, del Valle PLO, Socarrás FB, Arce HAA

Language: Spanish
References: 29
Page:
PDF size: 93.91 Kb.


ABSTRACT

The progressive hemifacial atrophy (AHP) or Parry Romberg syndrome, is a rare degenerative disease, characterized by slowly progressive unilateral facial atrophy involving the subcutaneous tissue, cartilage, fat tissue and underlying bone structures, which often overlaps with a condition known as linear scleroderma «en coup of sabre». To our knowledge has not been reported the association between immunodeficiency and this syndrome. We report the case of a child of 5 years with AHP, with a history of recurrent infectious processes, some serious, since he was 7 months old. The immunological study showed T cell immunodeficiency, lymphocyte subpopulations showed T/CD4 T/CD3 + cells values decreased and normal value B/CD19 + cells. The presence of antinuclear homogeneous pattern and anti-dsDNA antibodies confirm de autoimmune disorders described in these patients. The cellular immunodeficiency with AHP is an expression of great clinical variability of this disease and the importance of early diagnosis.


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