Acro-osteolysis.

Acro-osteolysis is an osteolysis of the distal phalanges of the hands and feet and can affect the terminal tuft or the shaft of the distal phalanx (transverse or band acro-osteolysis). It is often associated with distal digital ischemia, digital calcinosis, or severe sensory neuropathy. Acro-osteolysis has been associated with a heterogeneous group of disorders, including occupational activities, infections, rheumatic disorders (systemic sclerosis, psoriatic arthritis), endocrinopathies, genetic disorders, and lysosomal storage disorders. Plain radiography is the gold standard for the detection of acro-osteolysis.

Exotic pathology of the hand and foot. A pictorial review.

In this article, the imaging findings of the most frequently encountered import pathology of the hand and foot are reviewed, including leprosy, loiasis, snake bites or penetration of spines of sea urchins in the hand and foot. Our objective is to familiarize the radiologist of the Western countries with these diseases, which are still prevalent in developing areas. Due to the rising traveling to foreign countries and continuous immigration, it is important that these disorders be considered in the differential diagnosis in a specific population of asylum-seekers, economic refugees and any other group of persons traveling around the globe. Imaging findings on conventional radiography will be emphasized.

Profile of systemic sclerosis in a tertiary care center in North India.

AIM: To study the clinical and immunological profile in patients of systemic sclerosis from North India and compare it with other ethnic groups. METHODS: Patients presenting to us between the years 2001 and 2004 and fulfilling the American Rheumatism Association (ARA) criteria for systemic sclerosis were included. There were 84 females and 16 males with the mean age of 32.5 +/-11.62 years and a mean duration of 6.49 +/- 4.34 years. All patients were admitted to the dermatology ward for detailed history and examination including Rodnan score. Investigations including hemogram, hepatic and renal functions, serum electrolytes, urine for albumin, sugar, microscopy and 24h urinary protein estimation, antinuclear antibody, chest X-ray, barium swallow, pulmonary function test, electrocardiogram and skin biopsy were done. RESULTS: The most common presenting symptoms were skin binding-down (98.5%), Raynaud's phenomenon 92.9%, pigmentary changes 91%, contracture of fingers 64.6%, fingertip ulcer 58.6%, restriction of mouth opening 55.5%, dyspnea 51.1%, joint complaints 36.7% and dysphagia in 35.2%. The mean Rodnan score was 25.81 +/- 10.04 and the mean mouth opening was 24.6 +/- 19.01 mm. The laboratory abnormalities included raised ESR in 87.8%, ANA positive in 89.1%, proteinuria in 6.0%, abnormal chest X-ray in 65.3%, abnormal barium swallow in 70.2% and reduced pulmonary function test in 85.8%. CONCLUSION: The clinical and immunological profile of systemic sclerosis in North India is similar to that of other ethnic groups except that pigmentary changes are commoner and renal involvement is relatively uncommon.

Leprous osteitis presenting as bone cyst and erosions.

A 30-year-old man presented to the Hansen outpatient department with swelling and ulceration of toes for 2 months and swelling of the right fifth and fourth fingers and the left second finger for 1 month. In addition to skin lesions of lepromatous leprosy (subpolar type), there was nontender, non-fluctuant swelling of the right fifth and fourth fingers and left second finger. Skin over the right fifth finger showed sinus-like openings with associated purulent discharge. He also had swelling and ulceration of second left toe. Slit-skin smear (SSS) showed a bacterial index of 6+ from the ear lobes and cutaneous nodules, 4+ from the patch, and 3+ from normal skin. Modified Ziehl-Neelsen staining of the discharge extruding from the sinuses on the right fifth finger also showed abundant acid-fast bacilli. Radiography of the hands and feet showed lytic lesions in the distal epimetaphyseal region o proximal phalanx of the right fifth finger and left second finger and erosion of distal end of proximal phalanges of both second toes. Histopathological examination of biopsy specimen from the patch (back) showed features of lepromatous leprosy, and Fite-Faraco stain for tissue acid-fast bacteria (AFB) was strongly positive. Fine-needle-aspiration cytology (FNAC) from the lytic lesion in the bone also showed predominantly foamy macrophages with strongly positive staining for AFB with a few interspersed lymphocytes, epithelioid cells and Langhans giant cells. On the basis of these features, a clinical diagnosis of subpolar lepromatous leprosy with leprous osteitis was made. In today's clinical era of improved case detection and prompt treatment with effective multidrug regimens, advanced bone changes are rarely encountered. We describe this case of lepromatous leprosy that developed cavitating lesions of the phalanges of the hand, seen on x-ray as well-defined bone cyst and erosions.

Radiological changes in hands and feet in disabled leprosy patients: a clinico-radiological correlation.

Seventy-six consecutive leprosy patients with disabilities were subjected to radiological examination of hands and feet, and bone changes were found in 63 of them (82.9%). Specific, nonspecific and osteoporotic bone changes were observed in 22.4%, 78.9% and 28.9% of cases respectively. Bone cysts (10.5%), subarticular erosions (10.5%) and enlargement of nutrient foramina (5.3%) were the common specific bone changes whereas bone absorptive changes (59.2%), soft tissue changes (39.5%) and concentric absorption (39.5%) were the most frequent nonspecific bone changes. Specific bone changes were more common in older patients (age 40 years) and nonspecific bone changes correlated with duration of disease, duration of deformity, and disability index. Osteoporotic bone changes were found to be affected by ageing and severity of disability of hands and feet.

The rheumatic manifestations of leprosy.

We investigated the rheumatic and laboratory features in 25 patients with Hansen's disease. Sixteen (64%) developed a broad range of rheumatic manifestations, the most common being a distinctive syndrome of swollen hands observed in 10 patients (66.5%). These manifestations were more frequent in patients with lepromatous leprosy. There was no correlation between articular clinical findings and X-ray or laboratory abnormalities. A careful clinical history and the recognition of rheumatic features will help to differentiate patients with leprosy and rheumatic manifestations from those with a primary rheumatic disease.

Bone loss in limbs with decreased or absent sensation: ten year follow-up of the hands in leprosy.

Three hundred and sixty-seven patients with insensitive hands have been studied by correlating radiologic findings with occupational and medical history in order to better define causal factors in bone resorption. This study indicates that nonspecific infection and trauma are the reasons for bone resorption in 98% of cases. The role of intermittent pressure seems to be in soft tissue breakdown, which then allows bone to become infected. Bone resorption can be arrested at any stage of the disease by appropriate therapy of splinting and control of infection.

[Bone changes in Hansen's disease]. / Knochenveränderungen bei Morbus Hansen.

Bone lesion is a frequent organic manifestation in leprosy. Osseal destructions caused by granulomatous process induced by M. leprae are so-called specific lesions in contrast to non-specific lesions based on nerval or arterial diseases. The specific osseal alterations are characterized by cystic brightenings in roentgenograms while non-specific osseal changes show absorption to bone structure as akroosterolysis and osteoporosis. Typical radiologic findings in different stages of mutilation are demonstrated.

Arteriographic evaluation of vascular changes in leprosy.

Two hundred and eighty-four arteriograms were performed in the hands and feet 178 leprosy patients. Narrowing and tortuosity of the digital vessels, especially in the presence of bone absorption, were constant features. Digital tufts revealed signs of ischemia. However, in the presence of infection, hyperemia was a definite observation. Diminution in the caliber of the digital arteries and thinning or absence of the vascular end loops prove that ischemia plays a vital role in the production of various lesions in leprosy.

Peripheral arteriographic studies in leprosy.

There is a considerable controversy regarding the frequency and significance of vascular lesions in leprosy. Twenty-six cases of leprosy below the age of 40 years, without any local or systemic arterial disease and normal looking hands were subjected to brachial arteriography. One patient had posterior tibial arteriography also. The diagnosis of leprosy was confirmed by histology or bacteriological staining techniques. Biopsy material was studied from the involved areas and similar material was obtained from nine matched control subjects at autopsy. Skin thermometry and reflex vasodilatation was studied in eight cases. Arteriographic abnormalities such as occlusion, narrowing, tortuosity, dilatation, irregularity and incomplete filling were seen in more than two vessels in the hand in 50%, and in the digits in 75% of the cases. The ulnar artery was more frequently involved than the radial. Superficial and deep palmar arches were equally affected. Marked increase in arcuate vessels was noticed in nearly three-quarters of the patients, and in some, arcuate vessles were serving as collaterals. Histologically all sizes of vessles, including the capillaries, showed changes in nearly half the cases. Reflex vasodilation was lost in three cases of dimorphous leprosy and impaired or absent in three out of five cases of lepromatous leprosy. There was no predilection for any form of leprosy to show arterial changes more than the other. This study clearly demonstrates that the arterial involvement in leprosy is very frequent and must be playing an important role in causing mutilation and deformities of hands and feet.