Syndrome in question

Abstract The Iso-Kikuchi Syndrome is a rare condition characterized by nail dysplasia involving the index fingers, including micronychia, polyonychia, anonychia, irregular lunula, malalignment and hemionychogryphosis. On the antero-posterior image, radiologic examination reveals a narrowing of the distal phalanx. The lateral image shows a Y-shaped bifurcation of the distal phalanx. We report a case of a patient with typical clinical and radiologic signs of Iso-Kikuchi Syndrome.

Poland's syndrome with unusual hand and chest anomalies: A rare case report.

Poland’s syndrome is a rare congenital anomaly consisting of a unilateral absence of the pectoralis major, ipsilateral muscle, hand anomaly and occasionally associated other malformations of the chest wall and breast. Many structural and functional abnormalities have been described in association with this syndrome. We report an incidentally diagnosed case in a 27-year-old male patient who presented to us with symbrachydactyly. In addition to this, anterior depression of 2nd, 3rd and 4th ribs and bifid (forked) 5th rib was present on radiological investigations. The body of sternum was short and deformed on the right side with absence of xiphoid process. All middle phalanges were absent on right hand. It is a rare variant of Poland’s syndrome.

Kirner's deformity misdiagnosed as fracture: a case report

Kirner's deformity or dystelephalangy is a rare entity which presents with painless, progressive, bilateral radiovolar curving of the terminal phalanges of the little fingers. It is a clinicoradiological diagnosis. Herein, we present a case where the patient was being treated as having a fracture of the distal phalanx because of misdiagnosis of Kirner's deformity. Given the rarity of the deformity, we believe it useful to present our case report as a contribution to the literature

Acro-osteolysis and mononeuritis multiplex as a presenting symptom of systemic angiitis of Wegener's type.

Wegener's granulomatosis is a multisystem disorder involving small- and medium-sized vessels, leading to granuloma formation and involvement of upper and lower respiratory tract with or without glomerulonephritis. However, limited forms of angiitis and granulomatosis of the Wegener's type with oligosymptomatic and atypical site involvement are known to occur. We present here a rare case of limited form of angiitis and granulomatosis of Wegener's type who presented sequentially with spontaneous resorption of digits with acro-osteolysis and mononeuritis multiplex over a period of 10 months. His vasculitic workup revealed high proteinase 3 antibodies (c-ANCA) titers and an almost asymptomatic lung involvement, detected on high-resolution computed tomography of chest. The patient was aggressively treated with immunosuppressive therapy, following which he showed good improvement.

Pachydermodactyly: Report of two cases

Pachydermodactyly is a rare, benign, mostly asymptomatic digital fibromatosis, characterized by swelling on the back and sides of proximal phalanges and [or] proximal interphalangeal joints. It occurs predominantly in young males although a few women including a familial case were recently described. A history of repeated trauma is sometimes available but the etiology remains unknown We report two cases one of which had a history of repeated trauma [rubbing] to the involved area

Macrodactylism Associated with Neurofibroma of the Median Nerve: A Case Report

A case of macrodactyly associated with neurofiborma of the median nerve, a congenital anomaly of the hand, affecting only one(left middle) finger is reported with a review of the literature. Macrodactyly which is also termed local gigantism, megalodactylism, megalodactylia, or macrodactylism in other literature, is a rare congenital malformation characterized by overgrowth of one or more fingers of hand. Macrodactyly associated with neurofibroma of the median nerve is especially rare. For this reason the following case is presented together with a review of the literature.