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Plasma cell dyscrasia: a case of POEMS syndrome with a unique dermatologic presentation.

POEMS (polyneuropathy, organomegaly [hepatosplenomegaly or lymphadenopathy], endocrinopathy, M protein, and skin changes) syndrome is an uncommon plasma cell dyscrasia with diverse manifestations, including lymphadenopathy as well as those that comprise the acronym. Dermatologic changes may include hyperpigmentation, thickened skin, hypertrichosis, and papular angiomas. These changes are believed to result from humoral products of the plasma cell clone and frequently resolve with treatment of the neoplasm. We describe a man with typical POEMS syndrome with a unique skin lesion: a large, sharply circumscribed, pigmented plaque on the anterior chest wall centered over a plasmacytoma of the sternum. Results of histologic examination showed a proliferation of capillaries of varying size, enlarged fibroblasts, and increased amounts of collagen and proteoglycan from the dermis to the periosteum. Because the outer cortex of the sternum was eroded, peptide mediators of vascular and fibroblast proliferation may have diffused directly from the plasma cell lesion into the tissues of the chest wall.

Adult↗

Cyclosporine in dermatology.

Cyclosporine is a potent immunosuppressive agent with no appreciable effect on the bone marrow and a selective inhibitory effect on helper T cells. Oral cyclosporine was first used to prevent organ rejection but also has been reported to be effective in other disorders. In cutaneous diseases that respond to oral cyclosporine helper T cells appear to be involved in their pathogenesis. This article reviews the cutaneous diseases that have been treated with cyclosporine and its pharmacology and side effects. Two significant adverse side effects are renal dysfunction and hypertension, both of which are reversible when short-term low-dose (less than 5 mg/kg per day) oral cyclosporine is discontinued. Lymphoma is unlikely in an otherwise healthy patient who has received low-dose oral cyclosporine for limited periods. The use of oral cyclosporine in any patient should be carefully considered in terms of the risk/benefit ratio and needs to be carried out under close medical supervision. In view of the limited experience with cyclosporine in dermatology, whenever possible its use should be confined to formal clinical studies with established protocols and guidelines. Further controlled studies need to be performed to evaluate the efficacy of low-dose cyclosporine in many dermatoses and its side-effect profile, particularly over the long term.

Animals↗

Full proceedings from the National Conference to Develop a National Skin Cancer Agenda. American Academy of Dermatology and Centers for Disease Control and Prevention, Washington, D.C., April 8-10, 1995.

National efforts to reduce skin cancer incidence and mortality require scientifically coordinated efforts. This report summarizes the first American Academy of Dermatology/Centers for Disease Control and Prevention national conference to develop a skin cancer agenda. Leading experts in dermatology, public health, medicine, health education, nursing, behavioral sciences, environmental health and epidemiology identified and prioritized skin cancer control issues in five key areas. Discussion centered around strategies for reducing UV exposure and increasing public and professional awareness of skin cancer. Panelists in five sessions developed consensus on several public and professional recommendations and a series of research strategies.

Health Education↗

Pediatric dermatology: advances in therapy.

Several recent therapeutic advances in pediatric dermatology have been made. Of particular importance are new developments in the use of antimicrobials, antivirals, antifungals, retinoids, calcipotriene, and intravenous gamma globulin. We review safety and efficacy data of these drugs in their use in children with cutaneous disease.

Anti-Bacterial Agents↗

New therapeutic agents in veterinary dermatology.

The rapid development of therapeutic agents will continue to provide veterinarians with new options in treating dermatologic disease. However, we must not overlook the possibility of new applications for older drugs. Ideally, before a drug can be recommended for routine use, it should be evaluated for safety and efficacy using unbiased scientific methods. Unfortunately, this type of testing is expensive and may take years to complete. Veterinarians faced with managing a difficult skin disease may have to make decisions based on anecdotal information or case reports. The importance of good client communication cannot be overemphasized. The veterinarian must explain the benefits and risks of each therapeutic option. A written informed consent statement is advisable when using a drug in a manner not approved by the FDA.

4-Quinolones↗

[Secondary cutaneous effects of hydroxyurea: prospective study of 26 patients from a dermatologic consultation].

PURPOSE: Hydroxyurea is a treatment of myeloproliferative syndromes. Its cutaneous side-effects are underestimated, because they are usually benign. We undertook a prospective study to evaluate their frequency. METHODS: During a 2-year period, all patients taking hydroxyurea for more than 6 months who had consultations at the dermatology department were systematically examined, regarding cutaneous side effects. RESULTS: Twenty-six patients were examined. All but one had cutaneous side-effects, including dryness (n = 16), moderate alopecia (n = 2), increased skin pigmentation (n = 5), melanonychia, single (n = 1) or multiple (n = 7), cutaneous atrophy (n = 4), leg ulcers (n = 8), plantar keratoderma (n = 3), pseudodermatomyositis (n = 1), lichen planus-like eruption on the dorsum of the hands (n = 2), actinic keratosis (n = 8), squamous cell carcinomas (n = 2), and mouth ulcerations (n = 1). CONCLUSION: This study shows that the frequency of hydroxyurea cutaneous side-effects diagnosed in 95% of studied patients is underestimated. They are usually benign, but some of them, in particular leg ulcers and squamous cell carcinomas, lead to modification of the treatment (39% of studied patients).

Antineoplastic Agents↗

[Problems posed by the therapeutic use of photosensitizers in dermatology].

Three phototherapeutic regimens with photosensitization are now used in dermatology: PUVA (psoralen + UVA), TUV (crude coaltar + UV), PRT (phototherapy with hematoporphyrin derivative). The efficiency of PUVA and TUV is well known in several dermatoses. PRT is now being tested experimentally. For TUV, the lack of a standardized regimen does not allow a clear-cut evaluation of the therapy. For PUVA, late side-effects, particularly carcinogenicity have to be considered. To improve efficiency and minimize the side-effects of PUVA some procedures, such as association with retinoïds, pharmaco-kinetic studies for individual adaptation of the therapeutic regimen and the use of new less mutagenic psoralens are helpful. The persistent phototoxicity following treatments with hematoporphyrin derivative constitutes the major side-effect observed, for this phototherapy.

5-Methoxypsoralen↗

High-performance liquid chromatography of unmodified rosin and its applications in contact dermatology.

Rosin is a well recognised skin sensitiser and is also amongst the most common causes of occupational asthma. Due to its complex chemical composition, it is difficult to isolate its many components and this has hindered progress in the identification of the specific respiratory and contact allergens it contains. This paper reports the application of high-performance liquid chromatography and other analytical techniques to the isolation and identification of contact allergens in complex mixtures such as rosin. HPLC methods were developed in order to isolate as many rosin components as possible and these were then patch tested on rosin sensitive individuals. The structure of the most dermatologically active component was then determined using mass spectrometry, nuclear magnetic resonance and infrared techniques. An HPLC method has also been developed which will enable the identification of rosin in commercial products, providing a valuable tool for determining the cause of rosin contact allergy. Furthermore, mass spectral data for the common abieitic-type resin acids are compiled which were used to confirm the identification of the HPLC resin acid peaks and have not been reported previously.

Allergens↗

Regional anesthesia with monitored anesthesia care for dermatologic laser surgery.

This article describes an anesthetic technique that the authors have found useful for cutaneous laser surgery in keeping with the standards for office-based anesthesia practice. Although still in its infancy, office-based anesthesia for dermatologic laser procedures has become one of the most challenging yet rewarding fields of anesthesia today. As laser procedures continue to flourish, with seemingly endless technologic advances, surgeons, anesthesia providers, and other medical personnel must work collaboratively in developing appropriate office-based practice. The authors' experience using the anesthetic technique described here has demonstrated that cutaneous laser resurfacing can be conducted safely and efficiently using a combination of facial nerve blocks with intravenous sedation.

Ambulatory Surgical Procedures↗

Antimicrobial therapy and resistance in dermatologic pathogens of the elderly.

Dermatologic infections are treated to improve the elderly patient's quality of life and prevent disability and death. Pharmacokinetic changes, social issues, and the risk for adverse effects and drug interactions, however, increase the challenge to achieve successful treatment of those over age 65 as compared with younger patients. This challenge grows ever larger when coupled with the increasing trends of resistance among bacteria and fungi. The clinician can effectively face this challenge through careful consideration of diagnosis, patient risk factors, antimicrobial pharmacology, and resistance patterns.

Aged↗

Radiotherapy in the treatment of dermatologic malignancies.

Although advances in surgical techniques, particularly micrographic surgery, have considerably expanded its role in the management of dermatologic malignancies, radiotherapy remains of considerable value. Its use should be considered in particular for the appropriate solid tumors in older patients, for metastatic disease, and for the more radioresponsive tumors including Merkel cell carcinoma, Kaposi's sarcoma, and the cutaneous lymphomas.

Clinical Trials as Topic↗

The prevention and treatment of cutaneous injury secondary to chemical warfare agents. Application of these finding to other dermatologic conditions and wound healing.

Chemical warfare agents are easily and inexpensively produced and are therefore potentially accessible to even underdeveloped nations and are a threat to civilian populations as well as advancing troops. Sulfur mustard is by far the most significant chemical warfare agent that produces cutaneous injury. Significant advances over the past few years have been made in understanding the pathophysiology of the lesions produced by sulfur mustard, as well as development of barrier creams and pre and post exposure therapies to moderate the damage and accelerate healing. Not only have these advances improved our understanding of the sulfur mustard injury and the care of the patients, these are potentially numerous applications for these findings in other dermatologic conditions including the treatment of chronic wounds.

Burns, Chemical↗

New and emerging therapies in pediatric dermatology.

Many of the dermatologic conditions for which children seek medical attention are caused by infectious organisms. Several medications have recently become available or are on the horizon for the treatment of pediatric skin infections and infestations. Treatment of tinea capitis with fluconazole, itraconazole, and terbinafine, antibiotic therapy for staphylococcal skin infections, cidofovir for the treatment of verrucae vulgaris and molluscum contagiosum and ivermectin for scabies and head lice are discussed.

Child↗

The effect of health care delivery systems on admission to and treatment at an inpatient dermatology unit.

The University of Miami Department of Dermatology has maintained an active inpatient unit. Analysis of data from a 12 month period from 1995-1996 showed 562 admissions. Cutaneous lymphoma, psoriasis, and chronic wounds accounted for over half the admissions. Most patients were insured by a fee-for-service system, and compared to patients insured by managed care systems or patients who were indigent, fee-for-service patients had the shortest length of stay. Using a case mix severity index, indigent patients had the greatest disease severity followed by fee-for-service patients. Patients enrolled in managed care systems had the least severity suggesting that factors other than disease severity alone may play a role in determining why patients are admitted.

Adult↗

Cutaneous T-cell lymphoma and cutaneous graft-versus-host disease. Two indications for photopheresis in dermatology.

Dermatologists are frequently involved in the management of cutaneous T-cell lymphoma (CTCL) and graft-versus-host disease (GVHD). The similarities of these two entities are reviewed in the context of clinical and histologic findings, pathogenesis, and therapy. Photopheresis therapy (extracorporeal photochemotherapy) is used in the treatment of both entities, and the mechanisms underlying the responses represent yet another striking similarity of these two crippling dermatologic diseases.

Graft vs Host Disease↗

Update on systemic glucocorticosteroids in dermatology.

Although dermatology now has the most extensive group of systemic medications available for the treatment of skin diseases at any time, GCSs remain the most important agents for managing inflammatory disorders. It is important that the dermatologist have a broad knowledge of guidelines for clinical use, pharmacology, and adverse effects of these drugs. Acute and chronic side reactions should be well recognized. An understanding of the HPA axis and reasons for administering GCSs in different ways is of great value. A good medical history should be taken on any patient treated with GCSs, including knowledge of conditions that would make GCSs inadvisable and other concomitant systemic medications that might produce drug interactions. During the course of therapy, physical examination should include all systems pertinent to side effects caused by these agents, including frequent evaluations of weight and blood pressure. Blood chemistries should be performed on a regular basis, including glucose, electrolytes, and serum lipids. Osteoporosis is one of the most significant adverse affects to be evaluated, with bone mineral density studies recommended on an annual basis for persons continuing on GCS therapy. If hip or other joint pain develops, MR imaging is the most specific and sensitive radiologic examination for evaluating the possibility of osteonecrosis. An ophthalmology examination should be performed every 6 to 12 months to detect early cataract or glaucoma development. Any early signs of infection should be evaluated by appropriate smears, wet preparations, and cultures. Many other studies, including gastrointestinal and pulmonary examinations, may be dictated by specific acute situations. It is important to begin early prevention of the bone loss that occurs with GCS-induced osteoporosis. The 1996 guidelines of the American College of Rheumatology, including adequate calcium and vitamin D intake, should be followed. Hormonal replacement, a bisphosphonate, calcitonin, or a thiazide diuretic may be indicated. Restriction of sodium in the diet is important, as well as adequate potassium intake. The diet should be low in saturated fat and calories and should be high in vegetable protein. Because osteoporosis is so prevalent with GCSs, keeping the patient as active as possible with mild-to-moderate exercise is important. Whenever possible, exposure to persons with infectious processes should be avoided, and proper treatment should be instituted at the initial signs of systemic or cutaneous infection. Oral doses of GCSs are best taken with food to prevent gastrointestinal irritation, and agents for gastric acidity occasionally may be indicated. Significant trauma should be prevented, as should severe exposure to the sun. Many situations may call for consultation with other medical or surgical subspecialists. The patient must be aware of the importance of regular physician evaluations and reporting of any adverse effects while on long-term GCSs. A good relationship and understanding between the patient and physician are vital in minimizing potential problems from these agents. If the dermatologist maintains the proper guidelines of care, patients on GCSs have the highest benefits and lowest risks possible.

Drug Interactions↗

Antiviral treatment of diseases in pediatric dermatology.

Pediatric dermatologic disease that have specific therapies and/or specific prophylactic measures are reviewed. Herpes simplex virus and varicella-zoster virus infections, human papillomavirus infections, and molluscum contagiosum infections are discussed with special emphasis on recent advances of therapy and prophylaxis.

Chickenpox↗

Dermatologic side effects of psychopharmacologic agents.

Exanthematous eruptions, urticaria, photosensitivity, pigmentary problems, acne, alopecia, fixed drug eruptions, and lichenoid reactions are the most common dermatologic side effects associated with the administration of psychopharmacologic agents. The cutaneous findings associated with the various eruptions and the most common inciting psychiatric medications are discussed.

Antipsychotic Agents↗