Majumdar Banashree, Jain Atul, Sen Debashis, Bala Sanchaita, Mishra Pranshu, Sen Sumit, Biswyas Projna, Behra Akhilesh
Department of Dermatology, The Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.
Department of Dermatology, KPC Medical College and Hospital, Kolkata, West Bengal, India.
Indian Dermatol Online J. 2016 Jul-Aug;7(4):293-6. doi: 10.4103/2229-5178.185465.
We report clinical, radiological, and pathological features of three cases of macrodystrophia lipomatosa, which is characterized by progressive proliferation of all mesenchymal elements, with a disproportionate increase in fibroadipose tissue. The peculiarity in our report is the involvement of contralateral hand and feet in our 1(st) case in contrast to usual presentations of this rare condition, which is often unilateral. In our 2(nd) case, the lateral aspect of foot was involved as against the usual finding of involvement of medial aspect in lower limbs, also another surprising finding in this case is increase in the size of lesion after puberty. Coming to our 3(rd) case, enlargement of almost whole of an upper extremity with profound involvement of middle, ring and little finger along with total sparing of the thumb is in itself an extremely rare occurrence as in upper limb, mainly the lateral aspect of hand and related digits bear the burnt and more over involvement of whole limb is itself an unique phenomenon.
我们报告了三例巨大脂肪瘤病的临床、放射学和病理学特征，该病的特点是所有间充质成分进行性增生，纤维脂肪组织不成比例地增加。我们报告中的特殊之处在于，与这种罕见疾病通常单侧发病的表现不同，我们的第一例患者双侧手和脚均受累。在我们的第二例患者中，足部外侧受累，而不是下肢内侧通常受累的情况，此外，该病例另一个令人惊讶的发现是青春期后病变大小增加。再看我们的第三例患者，几乎整个上肢肿大，中指、环指和小指严重受累，而拇指完全未受累，这本身就是极为罕见的情况，因为在上肢，主要是手部外侧及相关手指受累，而且整个肢体受累本身就是一种独特现象。
