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1/39. vulvodynia and vulvar vestibulitis: challenges in diagnosis and management.

    vulvodynia is a problem most family physicians can expect to encounter. It is a syndrome of unexplained vulvar pain, frequently accompanied by physical disabilities, limitation of daily activities, sexual dysfunction and psychologic distress. The patient's vulvar pain usually has an acute onset and, in most cases, becomes a chronic problem lasting months to years. The pain is often described as burning or stinging, or a feeling of rawness or irritation. vulvodynia may have multiple causes, with several subsets, including cyclic vulvovaginitis, vulvar vestibulitis syndrome, essential (dysesthetic) vulvodynia and vulvar dermatoses. Evaluation should include a thorough history and physical examination as well as cultures for bacteria and fungus, KOH microscopic examination and biopsy of any suspicious areas. Proper treatment mandates that the correct type of vulvodynia be identified. Depending on the specific diagnosis, treatment may include fluconazole, calcium citrate, tricyclic antidepressants, topical corticosteroids, physical therapy with biofeedback, surgery or laser therapy. Since vulvodynia is often a chronic condition, regular medical follow-up and referral to a support group are helpful for most patients.
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2/39. Topical 5-fluorouracil as primary therapy for keratoacanthoma.

    keratoacanthoma is a common, benign cutaneous neoplasm that displays rapid growth on sun-exposed skin. Keratoacanthomas usually involute spontaneously after several months but rarely progress to squamous cell carcinoma. Because this is a benign, self-limited lesion of exposed skin, effective treatment should emphasize patient comfort and cosmetic results in addition to effectiveness. The authors present 2 patients with keratoacanthomas treated with topical 5-fluorouracil. Both patients had complete resolution of their lesions within 8 weeks. The cosmetic result was superb in both patients. patient satisfaction with this therapy was excellent. Treatment can be instituted based on a clinical diagnosis; no diagnostic biopsy is necessary. Most keratoacanthomas respond to topical 5-fluorouracil therapy within 3 weeks, whereas squamous cell carcinomas respond poorly. Any lesion that shows a poor response after 3 weeks of therapy or that does not resolve within 8 weeks should undergo prompt excisional biopsy for definitive diagnosis and treatment. Topical 5-fluorouracil is an effective, convenient, relatively inexpensive treatment for keratoacanthoma that produces excellent cosmetic results. It should be added to the therapeutic armamentarium of all physicians who treat keratoacanthoma.
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3/39. Bilateral carpal tunnel syndrome as a clue for the diagnosis of systemic amyloidosis.

    A 60-year-old Swiss woman presented with a 1-year history of periorbital hemorrhagic papules, a tendency to develop hematoma due to minor trauma and shortness of breath. The personal medical history included surgery for bilateral carpal tunnel syndrome (CTS) 2 years ago. Clinical and laboratory findings included macroglossia, Bence-Jones proteinuria, reticular lung infiltrates, thickening of the ventricular walls in echocardiography and increase in atypical plasma cells in the bone marrow. The coexistence of the specific symptoms of CTS, macroglossia and cutaneous lesions should immediately alert the physician to the probable existence of plasma-cell-dyscrasia-related systemic amyloidosis.
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4/39. White sponge nevus of the tongue.

    To my knowledge, this is the fifth case of white sponge nevus of the tongue to be reported. This lesion is probably much more common than has been reported in the past. The disorder is benign and almost always asymptomatic. The recognition of this disorder is important in that it must be differentiated from other congenital or familial disorders of more widespread clinical significance. It is important for any physician, and especially for dermatologists, to do a thorough examination of the oral mucous membranes as a part of any physical examination.
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5/39. 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.
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6/39. Cowden disease. Report of two additional cases.

    Two patients with Cowden disease, a syndrome consisting of cutaneous papules and nodules on the face, oral mucous membranes and dorsal aspects of the forearms and hands in association with tumours of the thyroid gland, breasts, gastro-intestinal tract, and female reproductive tract, are reported. This disease is important to the physician because recognition of these cutaneous lesions may allow early diagnosis of internal neoplasia.
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7/39. Pilomotor seizures: symptomatic vs. idiopathic report of two cases and literature review.

    We report pilomotor seizures in two patients who presented with piloerection or gooseflesh spreading in a pattern similar to the 'Jacksonian march'. Gooseflesh was confined to the ipsilateral side in most of the episodes. Occasionally it spread to the contralateral side. It was also associated with other autonomic symptoms and complex partial features of temporal lobe origin. Simple partial status that progressed to complex partial status occurred in the second patient. Very rarely secondary generalization occurred. The cause was left sphenoid meningioma and temporal tip contusion in the first case. It was idiopathic in the second case, although a positive family history of complex partial seizures was obtained in this patient.Interictal electroencephalogram (EEG) showed left temporal focus in the first and bitemporal foci with right fronto-temporal dominance in the second. Parenteral phenytoin controlled the partial complex status in the second and carbamazepine controlled the episodes in both cases. To our knowledge all reported cases were symptomatic and our case of idiopathic aetiology is the first to be recorded. We endorse that pilomotor seizures are autonomic in nature and constitute a subtype of simple partial seizures. These autonomic simple partial seizures may progress to, or be a component of, complex partial seizures of temporal lobe origin. Based on their dominance in such a symptom complex and careful interpretation of the ictal history, it can be logically concluded that pilomotor seizures may be underestimated by both patients and physicians.
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8/39. Incomplete Kawasaki disease with recurrent skin peeling: a case report with the review of literature.

    Kawasaki disease (KD) is an acute systemic vasculitis of unknown aetiology that has largely replaced rheumatic heart disease as a cause of acquired heart disease in children of many developed countries. We report a case of incomplete KD in a five-year-old girl. The diagnosis of incomplete KD was made after exclusion of conditions with similar presentation. She was treated with intravenous immunoglobulin following which she made an uneventful recovery but demonstrated thrombocytosis in the second week of convalescence. During the six-month follow up period, she had two episodes of recurrent skin peeling a phenomenon, which is recently reported with KD but not with atypical or incomplete KD. It is important for the treating physicians to become aware of the incomplete KD as prompt diagnosis and early treatment of these patients with intravenous immunoglobulin is vital for the prevention of lethal coronary complications. physicians need to have a "high index of suspicion" for KD and even, higher for IKD.
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9/39. Immune complex disease of the skin.

    The physician can now recognize clinically and histopathologically the cutaneous manifestations of immune complex disease. The usual clinical environment in which this type of reaction occurs has been very specifically delineated. Studies of immunoglobulins, complement components, and B cells in the blood may confirm the nature of the reaction. Special studies of cryoproteins of C1q precipitin or radioimmunoassay procedures may demonstrate directly the complexes in the blood. biopsy of skin for immunofluorescence is confirmative of the skin disease and the presence of immune complexes. biopsy of normal skin may be prognostic and indicate severity of the disease. Lesions may be induced by epinephrine, trauma, and controlled imflammation for clinical and pathologic study and confirmation of diagnosis. Treatment of the disease with corticosteroids, sulfapyridine, nicotinic acid, and antimalarial drugs may be useful. clofazimine is an intriguing experimental drug. Plasmaphoresis has worked well with some patients.
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10/39. Refractory sarcoidosis responding to infliximab.

    Despite aggressive treatment with conventional therapy, sarcoidosis may be progressive and debilitating. Tumor necrosis factor (TNF)-alpha is critical in the genesis and maintenance of granulomatous inflammation. Agents developed to inhibit TNF-alpha have been approved to treat rheumatoid arthritis and inflammatory bowel disease with unprecedented success. As such, physicians are increasingly using these agents to treat patients with other inflammatory diseases, including sarcoidosis. We report a case of refractory sarcoidosis, involving the lung, eyes, skin, and heart, which flared despite aggressive therapy. Oculocutaneous sarcoid dramatically improved after treatment with the anti-TNF antibody infliximab.
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