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.

Nail fold dermoscopy in collagen vascular disorders: A cross-sectional study.

BACKGROUND: The collagen vascular disorders, particularly systemic sclerosis, dermatomyositis, systemic lupus erythematosus and mixed connective tissue disorder, are often characterized by microangiopathic abnormalities of the nail folds. Nail fold dermoscopy is a well-established technique to assess these vascular changes. AIMS: To evaluate finger nail capillary vascular abnormalities by dermoscopy and their correlation with cutaneous and systemic involvement in the patients of collagen vascular disorders. METHODS: This was a cross-sectional study involving patients of collagen vascular disorders presenting to Government Medical College, Amritsar over a period of 2 years. Nail fold dermoscopy was done in these patients and correlated with cutaneous and systemic involvement. Statistical analysis was done using SPSS 17.0 version. RESULTS: A total of 30 patients were enrolled in the study. Sixteen (53.3%), 11 (36.7%) and 3 (10%) patients of systemic sclerosis, systemic lupus erythematosus and mixed connective tissue disorder, respectively were included for nail fold dermoscopy. The commonest change recorded in our study was dilated capillaries in 21 (70%) patients, followed by capillary dropouts in 17 (56.7%) patients and avascular areas in 16 (53.3%) patients. Of 17 patients presenting with sclerodactyly, active, early and late patterns were seen in 7 (41.2%), 2 (11.8%) and 7 (41.2%) patients, respectively. Out of 13 patients with respiratory involvement, active, early and late patterns were seen in 1, 1 and 7 (53.8%) patients, respectively (P value = 0.004). LIMITATIONS: Owing to lesser number of patients in our study, it is difficult to draw conclusive recommendations, and more studies with a larger sample size are required. CONCLUSION: Dermoscopy is a valuable tool not only to diagnose collagen vascular disorders but also for prognostication by correlating with systemic involvement.

Raynaud's Phenomenon: Its Prevalence in Systemic Sclerosis and Its Relationship with the Onset and with the Subsets of Systemic Sclerosis - An Experience from the Kashmir Valley, India / Fenómeno de Raynaud: su prevalencia en la esclerosis sistémica y sus relación con el inicio y los subconjuntos de la esclerosis sistémica - una experiencia del Valle de Kashmir, India

ABSTRACT Background: Raynaud's phenomenon (RP) is a very common clinical sign in patients with systemic sclerosis (SSc). Within the same country, its prevalence may vary depending on climactic changes. Usually, it predates the onset of cutaneous involvement in SSc, but in rare cases, it can follow the skin changes in these patients. Its evolution differs in the two subsets of SSc (limited and diffuse) and can serve as a clinical pointer to differentiate between the two disease subsets. Objective: To study the prevalence of RP in SSc and report its relationship with the onset and with the subsets of SSc. Methods: A prospective observational study of 56 patients with SSc was carried out at the Postgraduate Department of Dermatology, STDs and Leprosy of Shri Maharaja Hari Singh Hospital, Kashmir, India. Results: Of the 56 patients, 40 (71.4%) had limited SSc (lSSc) and 16 (28.6%) had diffuse SSc (dSSc). Raynaud's phenomenon was seen in 54 (96.4%) of the 56 patients, comprising 39 (97.5%) of the 40 patients with lSSc and 15 (93.8%) of the 16 patients with dSSc. Thirteen (81.3%) patients with dSSc and two (5%) patients with lSSc had a short history (less than one year) of RP preceding the skin changes. Twenty-six (65%) patients with lSSc and only two (12.5%) patients with dSSc had a long history (more than one year) of RP preceding the cutaneous manifestations. Six (15%) of the 40 patients with lSSc had a simultaneous onset of RP and skin changes. In five (12.5%) of the 40 patients with lSSc, RP followed the skin changes. Conclusion: Raynaud's phenomenon was very common in these patients with SSc. Patients with lSSc had a longer history of RP compared with those with dSSc. It could occur simultaneously with skin changes or even postdate the onset of skin changes.

RESUMEN Antecedentes: El fenómeno de Raynaud (FR) es una manifestación clínica muy común en pacientes con esclerosis sistémica (ES). Dentro del mismo país, su prevalencia puede variar en función de los cambios climáticos. Generalmente, precede al inicio de la afección cutánea en la ES, pero en raros casos puede seguir a los cambios de piel en estos pacientes. Su evolución difiere en los dos subconjuntos de ES (limitada y difusa), y puede servir como indicador clínico para poder diferenciar entre estos dos subconjuntos. Objetivo: Estudiar la prevalencia de FR en la ES y reportar sus relación con el inicio y los subconjuntos de ES. Métodos: Se realizó un estudio observacional prospectivo de 56 pacientes con ES en el Departamento de Posgrado de Dermatología, Enfermedades de Transmisión Sexual y Lepra del Hospital Shri Maharaja Hari Singh, India. Resultados: De los 56 pacientes, 40 (71.4%) tenían ES limitada (ESL) y 16 (28.6%) tenían ES difusa (ESD). El fenómeno de Raynaud se observó en 54 (96.4%) de los 56 pacientes, abarcando 39 (97.5%) de los 40 pacientes con la variante ESL y 15 (93.8%) de los 16 pacientes con la variante ESD. Trece (81.3%) pacientes con ESD y dos (5%) pacientes con ESL tenían una historia corta (menos de un año) de FR, que precedía a los cambios cutáneos. Veintiséis pacientes (65%) con ESL y solamente dos (12.5%) pacientes con ESD tenían una historia larga (más de un año) de FR, que precedía a las manifestaciones cutáneas. Seis (15%) de los 40 pacientes con ESL tuvieron un inicio simultáneo de FR y cambios de piel. En cinco (12.5%) de los 40 pacientes con ESL, FR siguió a los cambios de la piel. Conclusión: El fenómeno de Raynaud fue muy común en estos pacientes con ES. Los pacientes con ESL tuvieron una historia más larga de FR, a diferencia de aquellos con ESD. El fenómeno de Raynaud podía ocurrir simultáneamente con cambios cutáneos o incluso presentarse tras el comienzo de los cambios cutáneos.

Cardio-pulmonary involvement in systemic sclerosis: A study at a tertiary care center.

BACKGROUND: Systemic sclerosis is a multisystem disorder characterized by microangiopathy, dysregulation of the immune system and massive deposition of collagen in the connective tissue of the skin, blood vessels and various internal organs. Although the mortality from renal crises has dropped significantly due to the use of angiotensin-converting enzyme inhibitors, cardiac and pulmonary involvement accounts for significant morbidity and mortality. We studied 28 patients with systemic sclerosis at Gandhi Medical College and Hospital, Hyderabad, over a period of two years for cardiopulmonary involvement. AIM: The aim of this study was to analyze the cardiopulmonary involvement in systemic sclerosis. METHODS: All patients with systemic sclerosis attending the dermatology outpatient department were included in the study. The diagnosis of systemic sclerosis was made based on the American Rheumatology Association criteria, and was further confirmed by skin biopsy and serological investigations. X-ray chest, electrocardiogram, two-dimensional ECHO, high-resolution computed tomography chest, pulmonary function tests and bronchoalveolar lavage were done to evaluate cardiopulmonary involvement. OBSERVATIONS: Out of 28 patients, 17 had diffuse systemic sclerosis and 11 had limited systemic sclerosis. Mean duration of symptoms was 2.9 years. Abnormalities in chest X-ray were found in 16 patients. Pulmonary function tests showed a restrictive pattern in 23 patients. High-resolution computed tomography of the chest showed evidence of interstitial lung disease in 21 patients, while five patients each had pleural effusion and cardiomegaly. Bronchoalveolar lavage showed different cellular patterns such as neutrophilia, eosinophilia and lymphocytosis. Pulmonary arterial hypertension was observed in seven patients and isolated pulmonary arterial hypertension in two patients. Electrocardiogram abnormalities were found in twenty patients. Two-dimensional ECHO was abnormal in 17 patients with valvular abnormalities being the most common finding. Overall, pulmonary involvement was observed in 27 patients and cardiac involvement in 17 patients. LIMITATIONS: A small sample size was a limitation of this study. Diffusing capacity of lung for carbon monoxide, and right cardiac catheterization were not done, as these were not available at our centre. CONCLUSIONS: In our patients, pulmonary involvement was more common than cardiac involvement. Interstitial lung disease and cardiac involvement were more commonly seen in diffuse systemic sclerosis whereas pulmonary hypertension was more frequent in limited systemic sclerosis. Hence, it is important to screen the patients for cardiopulmonary involvement for early diagnosis and treatment and a better prognostic outcome.

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.

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.

Dermatology life quality index in Thai patients with systemic sclerosis: a cross-sectional study.

BACKGROUND: Systemic sclerosis (SSc) is a multisystem disorder that affects various organ systems. Although SSc patients have both physical and psychological illness, psychological distress is sometimes underestimated as most physicians usually pay more attention to physical problems. AIMS: To evaluate dermatology-specific health-related quality of life (QoL) in Thai patients with SSc. METHODS: SSc patients, who attended the Department of Dermatology, Siriraj Hospital, Bangkok, Thailand, between August 2009 and April 2010, were enrolled. The demographic data and skin manifestations of the patients were recorded. Skin thickness of each patient was analyzed by modified Rodnan skin score. QoL was evaluated by using the validated Thai version of dermatology life quality index (DLQI) questionnaire. RESULTS: A total of 80 patients of SSc were enrolled in this study. Twelve patients had limited SSc, while 68 patients had diffuse SSc. The mean (SD) disease duration before period of evaluation was 9 (12.8) years. The mean total of DLQI score was 6.3 (range, 0-23). Patients with low DLQI score have longer disease duration than patients with high DLQI score (P<0.05). Pain/pruritus was the most significant problem to the patients. Salt and pepper appearance was the cutaneous finding that had association with high DLQI score. CONCLUSIONS: SSc had moderate impact on patient's dermatology-specific health-related QoL. Pain, pruritus, and obvious skin findings caused a significant impairment to SSc patients. Therefore, the treatment of pain and pruritus and prominent cutaneous findings should be taken into account to improve QoL of SSc patients.

Scleroderma in children: emerging management issues.

Scleroderma is a set of rare connective tissue diseases of unknown etiology. It is characterized by thickening and hardening of the skin. Scleroderma is divided into two main subgroups: systemic and localized. The systemic form, also known as systemic sclerosis, involves diffuse skin involvement associated with fibrotic changes in internal organs. Juvenile localized scleroderma is a more common entity and is usually confined to a specific region of the body with no internal organ involvement. Therapeutics are divided into three main subgroups for juvenile systemic sclerosis: antifibrotics, anti-inflammatories, and vasodilators. For localized disease, anti-inflammatories, vitamin D analogues, and UV irradiation have been investigated. The rarity of scleroderma in children and the self-limiting nature of the disease together make randomized controlled trials very difficult. Therefore, most data on therapeutic modalities for this condition have to be extrapolated from studies conducted on adults. International cooperation, following a standardized operation protocol, is needed to validate these and future interventions such as autologous stem cell transplant and cytokine-directed therapies.