Abstract:
:Cowden syndrome (CS) is an infrequent autosomal dominant multisystem genodermatosis, generally involving the skin, oral mucosa, thyroid, breast and gastrointestinal tract. It is characterized by a late onset in the 2(nd) or 3(rd) decade of life, an extraordinary potential for malignant transformation, especially of breast and thyroid, and an identifiable germline mutation. In 80% cases, the human tumor suppressor gene, phosphatase and tensin homolog (PTEN) is mutated; mutations involving KILLIN, SDH B/D, PIK3CA and AKT1 genes account for the rest of the cases. Its clinical signs are not only the "essential pearls" for early and accurate diagnosis of CS but also help timely detection of neoplasia as they precede development of cancer by several years. We describe the first Indian and the third world report of polydactyly with CS, review this entity highlighting on recent clinical developments and emphasize on regular and thorough screening for prompt identification and management of the potentially malignant growths. We have also designed a baseline workup routine as well as a detailed screening program for these patients.
journal_name
Indian J Dermatoljournal_title
Indian journal of dermatologyauthors
Molvi M,Sharma YK,Dash Kdoi
10.4103/0019-5154.156360subject
Has Abstractpub_date
2015-05-01 00:00:00pages
255-9issue
3eissn
0019-5154issn
1998-3611pii
IJD-60-255journal_volume
60pub_type
杂志文章abstract::Cutaneous leiomyomas are benign tumors derived from the smooth muscles of the skin. They are firm, skin-colored to brownish, frequently painful papules and nodules presenting either as a solitary tumor or multiple clustered lesions. Uncommon patterns of multiple leiomyoma cutis include linear, zosteriform, or dermatom...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.156388
更新日期:2015-05-01 00:00:00
abstract:BACKGROUND:Palmoplantar psoriasis is a frequently encountered variant of psoriasis. It is difficult to treat and even more difficult to maintain remission as it is exacerbated by friction and trauma of the patient's daily activities. Existing topical modalities of treatment are often inadequate and show unpredictable r...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.77550
更新日期:2011-01-01 00:00:00
abstract::Aquagenic keratoderma (AK) is a rare acquired skin condition characterized by recurrent and transient white papules and plaques associated with a burning sensation, pain, pruritus and/or hyperhidrosis on the palms and more rarely, soles triggered by sweat or contact with water. Often AK cause significant discomfort, t...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.152568
更新日期:2015-03-01 00:00:00
abstract::We present the case of a male born prematurely at 32 weeks gestation by cesarean section following overt symptoms of maternal preeclampsia. He developed severe penoscrotal edema anew one month from birth. No remarkable exposure or trauma was identified. This unexplained swelling remained uniform till 4 months of age, ...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.117340
更新日期:2013-09-01 00:00:00
abstract::A 55-year-old woman presented with a 5-year history of livedo racemosa on her limbs. Histology showed vasculitis of medium-sized arteries with a circumferential, hyalinised, intraluminal fibrin ring. Her laboratory investigations did not indicate any underlying systemic disease. The findings were consistent with lymph...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.139907
更新日期:2014-09-01 00:00:00
abstract:BACKGROUND:Acne vulgaris causes erythematous papulopustular lesions in active stage and often leave behind residual scarring and pigmentation. Its onset in adolescence may add to the emotional and psychological challenges experienced during this period. AIMS:To assess the impact of acne on the various psychosocial dom...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.190102
更新日期:2016-09-01 00:00:00
abstract::A 58-year-old lady presented with polyarthritis followed by multiple shiny erythematous dome-shaped papulonodules mainly over the face, around joints and coral bead sign on fingers without any mucosal involvement. Characteristic histopathology with well-defined dermal infiltrate consisting of multinucleated giant cell...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.97678
更新日期:2012-07-01 00:00:00
abstract::Palmo-plantar keratodermas (PPKD) are a diverse group of acquired and hereditary disorders, characterized by excessive thickening of the skin of palms and soles. Here, we report a case of Type I or Buschke-Fischer-Brauer variant of punctate palmo-plantar keratoderma, in a 66-year-old gentleman. The association of our ...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.156452
更新日期:2015-05-01 00:00:00
abstract:Background:Leprosy is a chronic granulomatous infection primarily affecting the peripheral nervous system, skin and reticuloendothelial system. Cutaneous nerves are severely affected in lepra reaction and this leads to morbidity. Objective:To study electrophysiological pattern of different nerves involved in Type-II r...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/ijd.IJD_38_17
更新日期:2017-11-01 00:00:00
abstract:UNLABELLED:Vitiligo is a common skin disorder, and the pathogenesis is unknown. An increased prevalence of autoimmune thyroid diseases has been described in these patients. The aim of this study is to assess the prevalence of thyroid dysfunction and hypoparathyroidism in patients with vitiligo. MATERIALS AND METHODS:O...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.39733
更新日期:2008-01-01 00:00:00
abstract:BACKGROUND:Basement membrane (BM) is a thick sheet of extracellular matrix molecules, upon which epithelial cells attach. Various immunohistochemical studies in the past have been carried out but these advanced staining techniques are expensive and not feasible in routine laboratories. Although hematoxylin and eosin (H...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.159626
更新日期:2015-09-01 00:00:00
abstract::The various lesions seen in the clinical presentation of post kala-azar dermal leishmaniasis (PKDL) are reflected in the histopathology of the type of lesion biopsied. The cells that form the dermal infiltrate include lymphocytes, histiocytes, and plasma cells in varying proportions. The infiltrate, which is mild and ...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/ijd.IJD_307_19
更新日期:2020-11-01 00:00:00
abstract:BACKGROUND:Acanthosis nigricans (AN) is a dermatosis characterized by thickened, hyperpigmented plaques, typically on the intertriginous surfaces and neck. Common in some populations, its prevalence depends on race. Clinicians should recognize AN; it heralds disorders ranging from endocrinologic disturbances to maligna...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.91828
更新日期:2011-11-01 00:00:00
abstract:BACKGROUND:The mycological study of pityriasis versicolor is usually done by potassium hydroxide (KOH) mount and culture. However, KOH mount lacks a color contrast and requires a trained eye to interpret, while culture is difficult to perform, time consuming and has low sensitivity. Chicago Sky Blue 6B (CSB) is a new c...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.160476
更新日期:2015-07-01 00:00:00
abstract::A systematic review is a summary of existing evidence that answers a specific clinical question, contains a thorough, unbiased search of the relevant literature, explicit criteria for assessing studies and structured presentation of the results. A systematic review that incorporates quantitative pooling of similar stu...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.127671
更新日期:2014-03-01 00:00:00
abstract::Erythema annulare centrifugum (EAC) is characterised by slowly enlarging annular erythematous lesions and is thought to represent a clinical reaction pattern to infections, medications, and rarely, underlying malignancy. Causative drugs include chloroquine, cimetidine, gold sodium thiomalate, amitriptyline, finasterid...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/ijd.IJD_728_16
更新日期:2018-01-01 00:00:00
abstract::A 1-year old girl having a giant pigmented nevus over trunk and widespread smaller pigmented nevi presented with focal motor seizures. This combination of features is rate. ...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:
更新日期:1989-12-01 00:00:00
abstract:Background:Chronic spontaneous urticaria (CSU) is a skin disorder with an important immunologic profile. S100A8, S100A9, and S100A12 are the members of S100 family that have been reported to play important role in autoimmune diseases, but the characteristics of these three S100 members have not been defined in CSU. Ai...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/ijd.IJD_375_18
更新日期:2019-11-01 00:00:00
abstract:INTRODUCTION:Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive potentially life-threatening condition, characterized by glucocorticoid deficiency, preserved aldosterone/renin secretion, and secondary rise in plasma adrenocorticotropic hormone level. This occurs due to either mutation in adrenocorti...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/ijd.IJD_716_16
更新日期:2017-03-01 00:00:00
abstract:BACKGROUND:Onychomycosis is a difficult condition to treat and cure rates are disappointing. Moreover fungicidal action of antifungal agents in NCCLS assays and their rapid accumulation in nails in vivo are not compatible with the duration of treatment. AIMS:This study aimed to find the effectiveness of 4 different an...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.43216
更新日期:2008-01-01 00:00:00
abstract::Cohort design is a type of nonexperimental or observational study design. In a cohort study, the participants do not have the outcome of interest to begin with. They are selected based on the exposure status of the individual. They are then followed over time to evaluate for the occurrence of the outcome of interest. ...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.174011
更新日期:2016-01-01 00:00:00
abstract::The true existence of naevoid psoriasis and inflammatory linear verrucous naevus as distinct entities has been a debatable issue. Each has been opined to be a variant of the other. Considerable clinical and histological resemblance is seen between the two conditions. We describe three cases which attempt to throw more...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.103072
更新日期:2012-11-01 00:00:00
abstract::An eccrine poroma is a solitary tumor arising from the eccrine duct epithelium in the epidermis. The lesions commonly occur on the sole of the foot, the hands, and occasionally on the nose, eyelids, neck, and chest. We report a patient who presented with a slow-growing nodular lesion over her left cheek, prompting a d...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.123515
更新日期:2014-01-01 00:00:00
abstract::Tufted angioma (TA) is a rare benign vascular neoplasm, localized to the skin and subcutaneous tissues, occurring primarily on the trunk and extremities of children. The lesions are usually asymptomatic but, rarely, paroxysmal painful episodes may be associated. The occurrence of eruptive TA is still rarer and had bee...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.84741
更新日期:2011-07-01 00:00:00
abstract::Primary mucinous adenocarcinoma is a rare adnexal neoplasm of eccrine gland. Fewer than 200 cases are reported in literature. Clinically, it can be mistaken as a benign lesion. The primary challenge in these lesions is to differentiate these rare primary lesions from more frequent mucinous secondary deposits. Morpholo...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/ijd.IJD_619_16
更新日期:2018-03-01 00:00:00
abstract::A 19 year old girl had mutilating palmoplantar keratoderma with generalised ichthyosiform dermatosis and linear verrucous plaques in popliteal fossae. Her mother had similar disorder. ...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:
更新日期:1989-03-01 00:00:00
abstract::Dermatofibromas are benign skin lesions that consist of pigmented papules or nodules. They produce the dimple sign when laterally squeezed and are usually found on the legs. These clinical features lead to the diagnosis in most cases. However, the differential diagnosis with other lesions, such as atypical nevi and me...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.110862
更新日期:2013-05-01 00:00:00
abstract:Background:Deep venous thrombosis (DVT), even though resolved, may damage the valves and may lead to chronic venous insufficiency (CVI). We designed the present study to examine the thrombotic markers or other ultrasound features in the absence of active thrombosis in patients presenting with features suggestive of CVI...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/ijd.IJD_271_18
更新日期:2019-09-01 00:00:00
abstract::A Case of faun-tail nevus is presented. A long tuft of hair over the back in the lumbar region hid a bony defect of the fifth lumbar spine. No neurological symptoms were encountered. ...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:
更新日期:1989-09-01 00:00:00
abstract:BACKGROUND:The evaluation for skin disorders forms an important component of primary health care practice for all including children. The prevalence of certain skin diseases in children can reflect status of health, hygiene, and personal cleanliness of a community. AIMS:This study was planned to evaluate the magnitude...
journal_title:Indian journal of dermatology
pub_type: 杂志文章
doi:10.4103/0019-5154.97665
更新日期:2012-07-01 00:00:00