Nail-fold capillaroscopy for the dermatologists.

Nail fold is one of the most accessible sites for studying changes in the microcirculation in various microangiopathies. The characterization of changes in microvasculature can provide useful clues towards the diagnosis and prognosis of a disease. The diagnostic utility of nail fold capillaroscopy has improved and expanded over the past couple of decades. Beyond connective tissue diseases, it is now explored for its role in various systemic and dermatological diseases. Incorporation of nail-fold capillaroscopy in the diagnostic criteria of systemic sclerosis has generated interest among dermatologists. The current review is aimed at providing knowledge about nail-fold capillaroscopy to dermatologists. For the purpose of review, a PubMed search was done using the keywords "nail fold capillaries" and "nail fold capillaroscopy". All the articles were retrieved and classified into reviews and clinical studies of various types. The final data were then analyzed and presented in a narrative fashion.

Case of lepromatous leprosy misdiagnosed as systemic sclerosis.

Hansen's disease (HD) is a chronic granulomatous infectious disease caused by Mycobacterium leprae. The worldwide prevalence rate of HD has decreased gradually over the years. The clinical manifestations of HD are extensive, with involvement of the skin and various organs, and these can resemble those of many rheumatic diseases. Our patient initially presented with gradual sclerotic skin change and slight sclerodactyly with Raynaud's phenomenon, which is frequently observed in systemic sclerosis. However, a skin biopsy with acid-fast stain later confirmed lepromatous leprosy. We report this case to emphasize the role of dermatologists for applying a systematic approach to the skin lesions of HD, which has become difficult to detect because of its rapidly declining prevalence rate.

Therapeutic trials for systemic sclerosis: an update.

The pathogenesis of systemic sclerosis (SSc) is complex, and the final story is yet to be elucidated. The clinical heterogeneity of the disease, its various autoimmune and antibody profiles, its long course and tendency for spontaneous cure makes the design of clinical trials difficult. The overwhelming need in this disease is to diagnose it early and identify those patients who will benefit most from early, aggressive treatment. We attempt to review data from recent clinical trials and the lessons derived.

Childhood sclerodermatomyositis with generalized morphea.

Systemic sclerosis (SS) and dermatomyositis (DM) are both multisystem disorders and share some common clinical features. We report here an 11 year-old girl whose disease showed a changing clinical pattern from juvenile systemic sclerosis (JSS) to slowly progressing juvenile dermatomyositis (JDM) and had associated generalized morphea. Serological studies revealed antinuclear antibodies (ANA) with a speckled pattern. Topoisomerase-I (Scl-70), U1 RNP (ribonucleoprotein), anti-Ro, anti-La and anti Jo-1 antibody tests were negative. Electromyography (EMG) was suggestive of primary muscle disease and histopathological findings indicated scleroderma. The patient fulfilled the American College Rheumatology (ACR) diagnostic criteria for JSS as well as Bohan and Peter criteria for JDM separately and hence, was diagnosed to have sclerodermatomyositis (SDM). Mixed connective tissue disease (MCTD) and antisynthetase antibody syndrome (ASS) which share same clinical features with SS and DM were excluded by immunological studies.

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.

The often overlooked digital tuft: clues to diagnosis and pathophysiology of neuropathic disease and spondyloarthropathy.

OBJECTIVE: To assess diagnostic implications of abnormalities of the pedal digital tufts and to identify features to facilitate distinguishing of spondyloarthropathy and leprosy. BACKGROUND: Better criteria for distinguishing between these disorders are necessary if their character, natural history, and evolution are to be understood. METHODS: Pedal x rays of 91 consecutive patients with diabetes, 21 alcoholic patients, 100 with spondyloarthropathy, 8 with scleroderma, and 137 with leprosy, and 188 defleshed skeletons of individuals with alcoholism, syphilis, cerebrovascular disease, and paraplegia from the Terry and Hamman-Todd collections were examined for evidence of osseous and articular pathologies. Digital tuft abnormalities were divided into irregularity, divot, flattening, resorption, whittling, and fragmentation. RESULTS: Tuft divots were more common in alcoholics than in diabetic, and were more common in both than in the other groups studied. Tuft flattening was limited to alcoholic and neurosyphilis groups. Tuft whittling was especially prominent among individuals with spondyloarthropathy, contrasted with leprosy and diabetes. Aligned fractures were more common in diabetics than individuals with leprosy. Misaligned fractures were limited to individuals with leprosy and neurosyphilis. Leprosy and spondyloarthropathy were complicated by phalangeal and metatarsal whittling more commonly than other diseases studied. Background pedal abnormalities, derived from individuals with cardiovascular syphilis, cerebrovascular accidents, and paraplegia, was limited to abnormal divots only. CONCLUSIONS: Pedal digital tufts undergo a variety of pathological alterations useful in the recognition of disorders traditionally considered neuropathic in aetiology and in distinguishing differential considerations. Tuft flattening appears specific for alcoholism and neurosyphilis, and misaligned fractures seem specific for neurosyphilis and leprosy, providing differential assistance related to spondyloarthropathy. Conversely, periosteal reaction distinguishes spondyloarthropathy from leprosy.

[Pseudoscleroderma and sclerodermiform states]. / Pseudo-sclérodermies et états sclérodermiformes.

Pseudo-scleroderma should not be confused with true scleroderma, the prognosis of which is unpredictable and often serious. Progressive acrosclerosis must be differentiated from Raynaud's disease, congenital or hereditary disorders of unknown aetiology: Werner's syndrome, acrogeria and progeria; Rothmund-Thomson's syndrome, Steinert's disease, phenylketonuria, disorders of glycogen metabolism; metabolic disorders: mutilating acropathies, scleromyxoedema, porphyria cutanea tarda; occupational and iatrogenic disorders: acroosteolysis, toxic epidermic syndrome (Spain), scleroderma-like change induced by bleomycin, chronic graft-versus-host disease; and leprosy. Acute diffuse scleroderma should not be confused with Buschke's scleroedema, sclerema neonatorum, systemic amyloidosis and scleroderma-like changes in hypothyroidism. Linear pseudo-scleroderma is suggested by the following scleroderma-like conditions: facial hemiatrophy, acrodermatitis atrophicans, melorheostosis, pseudo-scleroderma after corticosteroid injection, and cutaneous lesions in carcinoid syndrome. Scleroderma in plaque must be differentiated from hypodermitis sclerotisans, panatrophy and localized lipoatrophies, hypodermitis after vitamin K injection, basal cell carcinoma, necrobiosis lipoidica, vitiligo, chronic radiodermatitis, cutaneous lymphatic invasion. Scleroderma-like changes after drug injection (vitamin B12, progestin), anetoderma barely resemble morphea guttata.

Case of suspected connective tissue disease presenting as chronic edema or swelling of face, neck, and upper trunk.

An adequate biopsy that includes the subcutaneous tissue is very helpful in the evaluation of patients with suspected connective tissue disease. However, the histologic features must often be correlated with the clinical features and other laboratory tests for a definitive diagnosis. An objective histologic diagnosis can usually be made in scleroderma, lupus erythematosus panniculitis, amyloidosis, and angioedema, and in diseases such as lepromatous leprosy and mycosis fungoids (which are sometimes confused with connective tissue diseases). Correlation with clinical features and, sometimes, other laboratory tests is often required to establish a diagnosis of scleredema, dermatomyositis, myxedema, and lichen myxedematosus. The features in cheilitis granulomatosa usually are not specific, but a biopsy is helpful to rule out other diseases.

Progressive systemic sclerosis (scleroderma). First case report in a Nigerian.

The first case of progressive systemic sclerosis in a Nigerian is described. In addition to the typical features of the disease, the case shows affection of the peripheral nerves, a very rare complication. The latter led to a mistaken diagnosis of leprosy. The reasons for the rarity of this disorder in the indigenous Africans and its differentiating features from leprosy are discussed.