1/77. The role of trisomy 8 in the pathogenesis of chronic eosinophilic leukemia.A case of chronic eosinophilic leukemia (CEL) manifesting as spinal cord compression by an extradural eosinophilic chloroma in a 32-year-old Chinese man was presented, who subsequently developed extramedullary transformation at the skin and then peritoneal cavity. Cytogenetic study of bone marrow cells at diagnosis showed a clonal karyotypic abnormality of trisomy 8 ( 8), which on fluorescence in situ hybridization (FISH) was shown to be present in a clone of abnormal eosinophils, hence showing the neoplastic nature of the eosinophilic proliferation. There was another population of abnormal eosinophils that did not show 8. At blastic transformation, all blast cells in ascitic fluid were shown by FISH to harbor 8. These findings suggest that 8 in this case may have arisen from clonal evolution and is not the primary genetic event in leukemogenesis, but 8 most probably imparts a further survival advantage to the clone responsible for subsequent blastic transformation.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/77. Cutaneous Rosai-Dorfman disease presenting as pustular and acneiform lesions.We report 2 cases of cutaneous Rosai-Dorfman disease (CRD) presenting with pustular and acneiform lesions. To our knowledge, these are the first reports of CRD presenting in this pattern. One patient had spontaneous resolution of the lesions, whereas the other was treated with cryotherapy with clinical improvement. We present a review of the literature regarding CRD and highlight the benign nature of this condition.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/77. Primary localized cutaneous amyloidosis--lichen amyloidosus. A case report.We report a case of primary localized cutaneous amyloidosis-lichen amyloidosus in a 55-year-old man. immunohistochemistry using antibodies against cytokeratin and AL immunoglobulins revealed the presence of both components in amyloid foci located subepidermally, mainly in dermal papillae. The results of histochemical reactions confirm the keratin-derived nature of amyloid in primary cutaneous amyloidosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/77. Inflammatory myofibroblastic tumor of the skin.We report a case of inflammatory myofibroblastic tumor (IMF) of the skin in a female with a history of Wegeners granulomatosis. The patient had a painless, erythematous, and indurated lesion of the left elbow. The resected specimen revealed a 4 cm x 3 cm nodule involving the entire dermis and superficial portions of subcutis with a stellate profile at scanning magnification. There were spindle cells in fascicles and whorls and a mixed inflammatory cell infiltrate of plasma cells, lymphocytes, neutrophils, and eosinophils. The spindle cells were immunoreactive for vimentin, muscle specific actin, and smooth muscle actin. The polyclonal and polymorphous nature of the inflammatory cells was confirmed by immunohistochemical studies. This is the first case of IMF of the skin documented by immunostaining.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/77. Secondary cutaneous amyloidosis in disseminated superficial porokeratosis: a case report.Disseminated superficial porokeratosis (DSP) is a rare cause of secondary cutaneous amyloidosis. An 83-year-old male patient showed an increase in both size and number of DSP lesions after contracting pulmonary tuberculosis. The DSP lesions of the patient consisted of numerous annular eruptions on both sun-exposed and sun-protected areas, which occurred over a period of 20 years. Multiple skin biopsies were taken from normal or lesional/sun-exposed or sun-protected skin samples. Histopathologic examination included routine H&E stains, congo red stains, thioflavin-T stains and anticytokeratin antibodies (AE1, AE3). And the results were as follows; 1) Positive staining with congo red and thioflavin-T indicated an amyloid nature for the deposits, 2) confinement of the amyloid deposition just below the lesional epidermis (while sparing the neighboring uninvolved or distant normal skin) indicated some role of the lesional epidermis, and 3) positive staining with AE3 further indicated an epidermal origin-type II epithelial keratin-of the amyloid. We present a case of DSP with a local amyloid deposit, characterized by association of positive familial background, severe pruritus and pulmonary tuberculosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/77. Chronic infantile neurological cutaneous and articular/neonatal onset multisystem inflammatory disease syndrome: ocular manifestations in a recently recognized chronic inflammatory disease of childhood.OBJECTIVE: To report on the ocular manifestations of the Chronic Infantile Neurological Cutaneous and Articular/Neonatal Onset Multisystem Inflammatory disease (CINCA/NOMID) syndrome, a rare, recently identified, pediatric multisystem inflammatory disease with chronic cutaneous, neurological, and articular manifestations. DESIGN: Descriptive case-report study. SETTING: International collaborative study based on a questionnaire. RESULTS: We included 31 patients. The mean age at onset of eye manifestations was 4.5 years. Optic disc changes were the most common feature, occurring in 26 patients (83%), including optic disc edema, pseudopapilledema, and optic atrophy. Anterior segment manifestations varying from mild to severe were seen in 13 patients (42%); chronic anterior uveitis, in 17 patients (55%). Moderate to severe visual acuity loss in at least 1 eye was seen in 8 patients (26%) as a consequence of the disease. Posterior synechia, glaucoma, and white iritis were not observed in any patient. CONCLUSION: Ocular manifestations with potentially sight-threatening complications occur commonly in the CINCA/NOMID syndrome. The distinctive nature of these complications may assist the ophthalmologist in recognizing this rare disorder and distinguishing it from juvenile rheumatoid arthritis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/77. Cutaneous pseudolymphoma of the breast with late homozonal relapse.Cutaneous pseudolymphomas refer to a group of lymphoproliferative disorders of difficult classification and diagnosis. Considered as very low-grade lymphomas by some authors, they are actually able to progress to overt cutaneous lymphomas in a minority of cases. We report a case of cutaneous pseudolymphoma of the breast in a 21-year old woman which relapsed locally ten years later. The case is of interest for the unusual localisation of the lesion, for its recurrence after such a long period of time, and for the homozonal site of recurrence. The reported features support both the long course and the lymphomatous nature of such lymphocytic proliferations.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/77. Langerhans cell histiocytosis in a child while in remission for acute lymphocytic leukemia.The occurrence of Langerhans cell histiocytosis (LCH) and malignancy in the same patient is rare. When LCH occurs concomitantly with acute leukemia, distinct temporal patterns often exist; acute myelogenous leukemia (AML) typically succeeds LCH, whereas acute lymphocytic leukemia (ALL) usually precedes it. We report a case of LCH developing in a child while in remission for ALL. Unique features of this case include the disseminated nature of the LCH and the death of the patient from LCH rather than ALL.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/77. Nonpharmacologic treatments in psychodermatology.The author believes that psychocutaneous medicine has indeed come of age and is being incorporated into mainstream medical practice. patients presenting to dermatologists today are more sophisticated and are frequently dissatisfied with traditional medical therapies. They actively seek alternative approaches and adjuncts to standard treatments. In contrast to many other "alternative" (or) "holistic" treatments offered through non-medical venues, dermatologists can assure their patients that controlled studies support the efficacy of psychocutaneous techniques in improving many dermatologic conditions. psoriasis, rosacea, herpes simplex, body dysmorphic disorder, acne, eczema, urticaria, neurotic excoriations, excoriated acne, trichotillomania, dysesthetic syndromes, and delusions parasitosis are included in this incomplete list. The author believes it is helpful for both the patient and therapist to define concrete and realistic goals for psychocutaneous intervention. Concrete observable or measurable goals can help the patient and clinician gauge therapeutic progress and success. Specifically, goals can include reduction in pruritus (rating severity from 1-10), decreased scratching activity, decreased plaque extent or thickness, decreased number of urticarial plaques, decreased flushing, decreased anxiety, decreased anger, decreased social embarrassment, decreased social withdrawal, and improved sleep. More global goals can include an improved sense of well-being, increased sense of control, and enhanced acceptance of some of the inevitable aspects of a given skin disease. Cure should never be a goal, because most disorders amenable to psychocutaneous techniques are chronic in nature; thus, cure as an endpoint would only lead to disappointment. The author encourages dermatologists to align themselves with what he euphemistically calls "a skin-emotion specialist." The skin-emotion specialist may be a psychiatrist, psychologist, social worker, biofeedback therapist, or other mental health or behavioral specialist. patients are more likely to accept a referral to a "skin-emotion specialist," because this term destigmatizes psychologic interventions. Incorporating these techniques and specialists into a clinical practice will expand therapeutic horizons and improve the quality of life of many of the patients afflicted with chronic skin disease. A final caveat must be offered about attempting to make prognostic statements regarding the likelihood of therapeutic success. Although all patients can potentially benefit from psychocutaneous interventions, those with severe psychopathology and poor pretreatment functional status are likely to be more difficult to treat and to achieve less optimal outcomes. patients with personality disorders such as borderline, narcissistic, and schizotypal disorders, and patients with any active psychotic process certainly constitute a more resistant and difficult population with whom therapeutic success is less likely. These patients, however, are often the ones in the greatest subjective distress and certainly can profit from any of the described interventions. Quoting W. Mitchell Sams, Jr., "although the physician is a scientist and clinician, he or she is and must be something more. A doctor is a caretaker of the patient's person--a professional advisor, guiding the patient through some of life's most difficult journeys. Only the clergy share this responsibility with us." This commitment is and must always be the guiding force in the provision of comprehensive and compatient patient care.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/77. Artefactual skin disease in children and adolescents.As in adults, artefactual skin disease in children and adolescents has heterogenous presentations with multifactorial aetiology. We report a series of 32 young patients aged 8-16 years. There were 24 females and 8 males. In over half of the cases the lesions were on the head and neck. The types of lesions encountered included physical injury producing grazing, erosions and deep ulcers, chemical and thermal burns, hair cutting and shaving and skin painting. We discuss the approach that we believe should be taken with these patients, emphasizing the role of dermatology-psychiatry liaison in their management. Of great importance is the avoidance of confrontation of the patient but the clear exposition of the nature of the problem to the parents. The major aim should be to have the family accept the need for expert psychiatric assistance.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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