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Centennial Paper. November 1951 (Arch Dermatol Syphilol: Cortisone acetate administered orally in dermatologic therapy by Marion B. Sulzberger, Victor H. Witten and Stanley N. Yaffe.

Thirty-two patients with various severe or selected dermatoses were chosen for treatment with cortisone acetate by mouth. The criteria for selection included refractoriness to previous therapy and absence of ascertainable contraindications. The initial dose of cortisone acetate varied between 100 and 200 mg. per day. The dose was reduced as quickly as each patient's response permitted, with the object of reaching the lowest effective dose as quickly as possible. Response of most patients to the hormone was dramatic, with abatement of symptoms within 24 hours and substantial improvement of clinical signs within 24 to 48 hours. Details of the results are tabulated. Adverse effects, possibly attributable to the hormone, were noted in five patients. In two instances, moon facies developed, one with hypertrichosis and a 20-lb. (9.1-kg.) gain in weight. However, both of these patients had received corticotropin (ACTH) prior to the cortisone. A third patient showed hyperpigmentation of the areas of skin usually exposed and not covered by clothing. Two additional patients each complained of hyperexcitability and insomnia. All these undesirable effects diminished or disappeared after the dose was reduced or administration of cortisone was discontinued. The effectiveness of this new therapeutic approach in a wide variety of skin diseases is clearly demonstrated by the excellent response noted in this series of selected cases. No other modality known to us has comparable beneficial effects. It is to be stressed, however, that the benefits generally stop soon after cortisone therapy is discontinued, unless the disease or the attack is one with spontaneous remissions. Disagreeable and sometimes dangerous effects still preclude the use of this treatment except in serious diseases and situations, and unless the patient can be kept under sufficiently close and expert surveillance.

Administration, Oral↗

Pityriasis rotunda. A cutaneous marker of hepatocellular carcinoma in South African blacks.

Although paraneoplastic phenomena occur frequently in patients with hepatocellular carcinoma, cutaneous changes have rarely been reported. During the past two years, ten South African blacks with hepatocellular carcinoma and pityriasis rotunda have been seen in a single hospital. The rash affected the trunk, especially the lower back and buttocks. The lesions ranged in size from 1.5 to 25 cm and were always multiple. They had a characteristic circular or arcuate configuration with scaling and varying degrees of hyperpigmentation. Pityriasis rotunda may prove to be a useful cutaneous marker of hepatocellular carcinoma in South African blacks.

Aged↗

Treatment of port-wine stains with a noncoherent pulsed light source: a retrospective study.

OBJECTIVE: We investigated whether a noncoherent intense pulsed light source (IPLS) would be effective in therapy of port-wine stains (PWSs). DESIGN: To evaluate the efficacy in treatment of PWSs with IPLS, a retrospective study was initiated. SETTING: The data were collected by physicians working in private practices and departments of university hospitals and medical centers, respectively. PATIENTS: A total of 37 randomly selected patients with a total of 40 PWSs were included in the study. Clinical PWS characteristics recorded were color and location of the PWS. INTERVENTIONS: All patients were treated with IPLS. MAIN OUTCOME MEASURES: Data collected included treatment parameter (filters, pulse duration, fluence, and pulse sequencing), percentage of clearance, and side effects (purpura, blisters, crusting, altered pigmentation, and scarring). RESULTS: Good and complete (70%-100%) clearance was achieved in 28 of 40 PWSs treated with IPLS. The average number of treatment sessions in PWSs reaching 100% clearance included 4.0 for pink PWSs and 1.5 for red PWSs. The average number of sessions for purple PWSs reaching good clearance (70%-99%) was 4.2 sessions. Parameters used most frequently were 515- and 550-nm cut-off filters, pulse duration of 2.5 to 5.0 milliseconds, and fluences of 24 to 60 J/cm2. Side effects included purpura in 133 (76%), superficial blisters in 14 (8%), and crusting in 35 (20%). Transient pigmentation changes were seen in 10.8% of patients (hypopigmentation in 3 [8.1%], hyperpigmentation in 1 [2.7%]). No scarring was observed. CONCLUSION: Intense pulsed light source presents an effective and safe method for treating PWSs, especially purple PWSs.

Adolescent↗

Cryogen spray cooling in combination with nonablative laser treatment of facial rhytides.

BACKGROUND: Cryogen spray cooling can be used to provide epidermal protection while still achieving spatially selective photocoagulation in the upper dermis. The objective of this study is to determine the efficacy and safety of cryogen spray cooling in combination with a nonablative Nd:YAG (lambda = 1320 nm) laser treatment of facial rhytides in human volunteers. OBSERVATIONS: Thirty-five adults with bilateral periorbital rhytides were treated with cryogen spray cooling in combination with 3 nonablative laser treatments performed sequentially at intervals of 2 weeks. Small but statistically significant improvements were noted in the mild, moderate, and severe rhytid groups 12 weeks after the final laser treatment. A final assessment performed 24 weeks after the last treatment showed statistically significant improvement only in the severe rhytid group. The procedure was found to be safe; 4 sites (5.6%) developed transient hyperpigmentation. Two sites (2.8%) subsequently developed barely perceptible pinpoint pitted scars. CONCLUSIONS: Cryogen spray cooling is a safe and effective method for protecting the epidermis during nonablative laser treatment of facial rhytides thereby avoiding much of the morbidity associated with other resurfacing procedures. Minor improvements in rhytides can be achieved with the current technology. Optimization of treatment parameters may further improve these results.

Adult↗

Electrosurgical facial resurfacing: a prospective multicenter study of efficacy and safety.

BACKGROUND: A novel electrosurgical technology that uses a bipolar electrode-tipped stylet to deliver relatively low-radiofrequency energy through an electrically conductive medium has been developed. OBJECTIVE: To evaluate the efficacy and safety of the radiofrequency resurfacing system for the treatment of facial wrinkles. DESIGN: Multicenter, prospective, noncomparative study with longitudinal follow-up. SETTING: Four US academic dermatologic surgery clinics. PATIENTS: Ninety-five patients with mild to severe photodamage (Fitzpatrick classes I-III) involving periorbital (75 treatment sites) and perioral (50 sites) facial skin. INTERVENTION: Radiofrequency resurfacing with the use of 2 to 3 passes at 125 or 139 V. MAIN OUTCOME MEASURES: Wrinkle and cosmetic improvements evaluated by patients, investigators, and, by means of photographs, an independent panel of 5 evaluators. RESULTS: All evaluators determined a positive mean improvement in wrinkles for both periorbital and perioral anatomic sites, with greater improvement for patients with more severe wrinkles at baseline. An increased number of passes and higher voltage settings had a positive impact on wrinkle improvement. Transient postinflammatory hyperpigmentation occurred in 26% of periorbital and 4% of perioral sites. Hypertrophic scars occurred in 3.8% of treatment sites, with all but 1 scar resolving by 6 months. For the most part, healing was rapid, pain was minimal, and erythema largely resolved within 2 months. Other untoward effects were relatively few and short-lived. CONCLUSIONS: At the study settings used, radiofrequency resurfacing is an effective modality in the treatment of periorbital and perioral wrinkles in patients with Fitzpatrick class I, II, and III photodamage. There is less severe postoperative morbidity than seen with carbon dioxide or coagulating erbium:YAG lasers. The potential risks are similar to those seen with other resurfacing modalities.

Electrosurgery↗

Long-term photoepilation using a broad-spectrum intense pulsed light source.

BACKGROUND: The goal of laser or flashlamp photoepilation is to produce long-term, cosmetically significant hair removal. We document the long-term efficacy achieved with an intense pulsed light source for photoepilation. DESIGN: Prospective study comparing long-term results of single vs multiple treatments, and effects of anatomic site and skin type on efficacy of photoepilation with a device emitting broad-spectrum, noncoherent (nonlaser) radiation from 550- to 1200-nm wavelengths, in macropulses divided into 2 to 5 minipulses. SETTING: Private dermatology practice. PATIENTS: Thirty-four patients (8 men, 26 women) with hirsutism. INTERVENTIONS: Parameters for the study were wavelength of 615 to 695 nm, pulse duration of 2.6 to 3.3 milliseconds, fluence of 34 to 42 J/cm(2), 10 x 45-mm exposure field, and application of 1 degrees C cooling gel. MAIN OUTCOME MEASURES: Hair removal efficiency, calculated as percentage ratio of the number of hairs present compared with baseline counts, and patient satisfaction questionnaire completed at last follow-up. RESULTS: The mean hair removal efficiency achieved was 76% after a mean of 3.7 treatments. More than 94% of the sites reached mean hair removal efficiency values greater than 50%. Hair removal efficiency was not significantly related to skin type, hair color, anatomic site, or number of treatments. Side effects were mild and reversible and occurred in a minority of patients (hyperpigmentation in 3 and superficial crusting in 2). CONCLUSIONS: Our data document the long-term clinical efficacy of intense pulsed light source-induced hair removal in light and dark skin phenotypes. Maximal photoepilation was achieved from the initial 1 to 3 treatments; only a small added benefit was seen after more treatments.

Adolescent↗

Pulsed alexandrite laser for the treatment of leg telangiectasia and reticular veins.

OBJECTIVE: To examine the safety and efficacy of a pulsed alexandrite laser for treatment of leg telangiectasia and reticular veins. DESIGN: Observational study. SETTING: Laser and Skin Surgery Center of New York, New York, NY. SUBJECTS: Twenty women with skin phototypes I to III and with 54 patches of leg veins measuring 0.3 to 2.0 mm in diameter. INTERVENTIONS: Each patch was treated once using 1 to 3 passes of a 755-mm, 3-millisecond alexandrite laser. An 8-mm spot and fluences of 60 to 80 J/cm(2) were used, with dynamic epidermal cooling. MAIN OUTCOME MEASURES: Subjects underwent evaluation at 4 and 12 weeks for degree of clearance, based on a quartile grading system, and incidence of adverse effects. RESULTS: At the 4-week follow-up, 17 (35%) of 48 treatment sites showed greater than 75% clearance and an additional 16 (33%) showed greater than 50% clearance. By 12 weeks, 33 (65%) of 51 treatment sites showed greater than 75% clearance, and there was greater than 50% clearance in an additional 11 (22%). Hyperpigmentation was observed in 18 (35%) of 51 treatment sites. CONCLUSION: A single treatment with a 755-nm, 3-millisecond alexandrite laser at high fluence in conjunction with cryogen skin cooling produced excellent clearance of telangiectasia and reticular veins of the leg with minimal adverse effects.

Beryllium↗

The use of tetracyclines for the treatment of sarcoidosis.

BACKGROUND: To evaluate the safety and efficacy of minocycline in the treatment of sarcoidosis, a nonrandomized, open study was performed in patients with cutaneous sarcoidosis. OBSERVATIONS: Twelve patients with cutaneous sarcoidosis were treated with minocycline, 200 mg/d, for a median duration of 12 months. Three patients had extracutaneous lesions at the time of the study. The median follow-up was 26 months. A clinical response was observed in 10 patients, consisting of complete responses in 8 patients and partial responses in 2 patients. A progression of skin lesions was observed in 1 patient, and lesions remained stable in another patient. Adverse effects were minimal, except in 1 patient, who developed hypersensitivity syndrome. A slight hyperpigmentation occurred in 2 patients at the site of previous lesions, which completely disappeared after minocycline use was discontinued. A relapse of skin symptoms occurred after minocycline withdrawal in 3 patients, who further received doxycycline, 200 mg/d, allowing a complete remission of lesions. CONCLUSIONS: These results support that minocycline and doxycycline may be beneficial for the treatment of cutaneous sarcoidosis. Randomized controlled studies are warranted for the evaluation of the true efficacy of tetracyclines in these patients.

Adolescent↗

The combined continuous-wave/pulsed carbon dioxide laser for treatment of pyogenic granuloma.

BACKGROUND: Pyogenic granuloma is a frequently diagnosed, benign vascular lesion. OBJECTIVES: To present the use of the combined continuous-wave/pulsed carbon dioxide (CO2) laser as an innovative therapeutic method, to compare it with established methods, and to assess its results. DESIGN: Prospective observational study between March 1998 and July 2000, comprising 1 treatment session with 6-week and 6-month follow-up examinations and evaluations. SETTING: Private or institutional practices as well as ambulatory or hospitalized care. PATIENTS: One hundred patients with pyogenic granuloma selected from a population-based sample. INTERVENTIONS: Treatment with CO2 laser. The laser was first used in continuous mode (power, 15 W) and then in pulsed mode (pulse length, 0.6-0.9 milliseconds; energy fluence, 500 mJ/pulse). MAIN OUTCOME MEASURE: Complete resolution of treated granuloma pyogenicum. RESULTS: Pyogenic granuloma was removed completely in 1 treatment session in 98 patients without recurrence. In 88 cases there were no visible scars; in 10 cases slight textural changes of the skin were observed. Hypertrophic scars or keloids did not occur. Sixty-three patients were very satisfied with the result of the treatment, 37 were satisfied (ie, 100% patient satisfaction), and none indicated that they were not satisfied. No permanent hypopigmentation, hyperpigmentation, or erythema was observed. CONCLUSIONS: The combined continuous-wave/pulsed CO2 laser is our treatment of choice for pyogenic granuloma because this kind of laser is widely available, produces excellent results with few adverse effects, is easy to use, yields low recurrence rates, and is well tolerated by most patients.

Adolescent↗

Late recurrence of inflammatory first-stage lesions in incontinentia pigmenti: an unusual phenomenon and a fascinating pathologic mechanism.

BACKGROUND: Incontinentia pigmenti (IP) is an X-linked genodermatosis that is manifested by neonatal inflammatory vesicles localized along the lines of Blaschko. These lesions usually clear spontaneously within a few months, leaving hyperpigmentation. Ophthalmologic and neurologic symptoms can be associated with IP. Late recurrences of the first-stage inflammatory lesions after the initial rash are uncommon and have been reported infrequently. The mechanism involved in this phenomenon is unclear. However, the recent identification of NEMO/IKKgamma as the gene responsible for IP sheds new light on its pathophysiologic origins. OBSERVATIONS: We report 5 cases of children who experienced episodes of late reactivation of IP. In all cases, the recurrences occurred on the previously hyperpigmented streaks several months or years after resolution of the initial eruptions. In most cases, the recurrences were preceded by an infectious episode. CONCLUSIONS: These IP recurrences suggest that mutated cells can persist a long time in the epidermis. We theorize that infections trigger the reactivations. The NEMO/IKKgamma gene encodes a protein essential in nuclear factor kappaB activation, which is required for resistance to tumor necrosis factor alpha-induced apoptosis. We discuss the role of a proinflammatory cytokine such as tumor necrosis factor alpha as a triggering factor for the reactivation.

Biopsy, Needle↗

Clinical study of 40 cases of incontinentia pigmenti.

OBJECTIVE: To analyze the distribution of manifestations in a pediatric cohort and define guidelines for follow-up of incontinentia pigmenti (IP). DESIGN: Retrospective study of 47 children referred to the Department of Pediatric Dermatology with a diagnosis of IP between 1986 and 1999. SETTING: The private or institutional practice of participating dermatologists and pediatricians. MAIN OUTCOME MEASURES: Evaluation of IP clinical diagnosis using the Landy and Donnai criteria. RESULTS: Because hyperpigmentation following the Blaschko lines may be observed in several pigmented disorders, 7 patients were found misdiagnosed. During the neonatal period, erythema, vesicles, and hyperkeratotic le sions were rarely absent in the patients with IP. Ocular and neurological abnormalities were frequent (20% and 30%, respectively) but rarely severe (8% and 7.5%, respectively). CONCLUSIONS: Clinical diagnosis is the first main step for a correct phenotype/genotype correlation, which remains indispensable to better understand the pathological mechanisms of IP and develop new therapies. In doubtful cases, molecular analysis is helpful but characteristic histological features must be added as major criteria for IP diagnosis. Multidisciplinary follow-up is needed, particularly during the first year of life, to detect possible ophthalmologic and neurological complications. Neuroimaging ought to be performed in the case of abnormal neurological examination results or when vascular retinopathy is detected.

Child↗

Targeted UV-B phototherapy for plaque-type psoriasis.

OBJECTIVE: To determine whether targeted UV-B phototherapy is efficacious and safe in the treatment of localized psoriasis and whether there is a dose-response relationship. DESIGN: Randomized, evaluator-blind, controlled study. SETTING: Dermatology clinic in a large university-based hospital in Bangkok, Thailand. PATIENTS: Fourteen patients with stable, localized, plaque-type psoriasis. INTERVENTIONS: Patients were randomized to receive different fluences of targeted UV-B phototherapy 3 times weekly based on predetermined minimal erythema doses (MEDs). Treatment fluences were constant throughout the study period of 4 weeks. Follow-up was carried out until lesions returned to original state. MAIN OUTCOME MEASURES: Modified psoriasis area and severity index. RESULTS: All fluences of UV-B produced some clinical improvement and were very well tolerated. Fluences ranging from 1 to 6 multiples of MEDs resulted in clearance of lesions in some patients with 6 MEDs producing clearance in 77% of patients. The number of treatments required to clear psoriatic lesions when 2 to 6 MEDs were used was 5.0 to 6.1 treatments. The only adverse events observed were erythema, which was asymptomatic in most subjects, and hyperpigmentation. CONCLUSIONS: Incoherent, targeted UV-B phototherapy is a safe and efficacious treatment modality for localized psoriasis. Its value in other UV-B responsive conditions should be further investigated.

Adult↗

Telemedical wound care using a new generation of mobile telephones: a feasibility study.

BACKGROUND: Leg ulcers are an important cost factor in health care systems. It has been shown that a telemedical wound care consultation can improve quality of care and help reduce costs. In this study, we evaluated the feasibility of telemedical wound care using a new generation of mobile telephones with integrated cameras. OBSERVATIONS: Three physicians separately evaluated 61 leg ulcers for the following 9 variables: epithelialization, fibrin, necrosis, and granulation tissue at the center and normal border, erythema, cyanosis, eczema, and hyperpigmentation at the periphery. One physician performed the face-to-face consultation (gold standard), and 2 others performed the remote evaluation. The image was obtained with the mobile telephone and immediately sent via e-mail. To measure the agreement of the evaluation among the 3 physicians, we used Cohen kappa statistics. Overall, the agreement between the remote and face-to-face evaluations was very good, with kappa values of up to 0.94 The image quality was judged to be good in 36 cases (59%) and very good in 12 (20%). The participants felt comfortable making a diagnosis based on the pictures in 50 cases (82%). CONCLUSION: Although this study was performed with the first generation of these devices, we were able to demonstrate the feasibility of such a telemedical wound care consultation.

Adult↗

Warfarin therapy for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia.

BACKGROUND: Livedoid vasculopathy is an idiopathic, chronic disorder manifested by painful, purpuric macules on the lower extremities that superficially ulcerate, resulting in atrophic, stellate scars with peripheral telangiectasias and hyperpigmentation. OBSERVATIONS: A 50-year-old man presented with recurrent, painful ulcerations on the medial aspect of his malleoli and calves. The clinical presentation, histologic findings, and results of laboratory evaluation confirmed the diagnosis of livedoid vasculopathy in this case. Despite being refractory to treatment with multiple other medications, the lesions responded dramatically to oral warfarin sodium therapy. CONCLUSION: Treatment with warfarin may be a beneficial therapy for patients with livedoid vasculopathy.

Anticoagulants↗

Keratosis pilaris rubra: a common but underrecognized condition.

BACKGROUND: Keratosis pilaris is a common skin disorder of childhood that often improves with age. Less common variants of keratosis pilaris include keratosis pilaris atrophicans and atrophodermia vermiculata. OBSERVATIONS: In this case series from dermatology practices in the United States, Canada, Israel, and Australia, the clinical characteristics of 27 patients with keratosis pilaris rubra are described. Marked erythema with follicular prominence was noted in all patients, most commonly affecting the lateral aspects of the cheeks and the proximal arms and legs, with both more marked erythema and widespread extent of disease than in keratosis pilaris. The mean age at onset was 5 years (range, birth to 12 years). Sixty-three percent of patients were male. No patients had atrophy or scarring from their lesions. Various treatments were used, with minimal or no improvement in most cases. CONCLUSIONS: Keratosis pilaris rubra is a variant of keratosis pilaris, with more prominent erythema and with more widespread areas of skin involvement in some cases, but without the atrophy or hyperpigmentation noted in certain keratosis pilaris variants. It seems to be a relatively common but uncommonly reported condition.

Adolescent↗

Vitiligolike macules in systemic scleroderma.

Changes of hyperpigmentation and depigmentation in systemic scleroderma have been known since the last century, but their relationship to the pathogenesis of the condition is poorly understood. Clinical, histologic, histochemical, immunopathologic, and electron microscopic studies of the depigmented lesions in seven patients with systemic scleroderma demonstrated changes similar to those of vitiligo, but with subtle differences. Immunologic mechanisms seem to be involved in the induction of depigmentation in both vitiligo and systemic scleroderma.

Adult↗

The Jewish Hospital of St. Louis therapeutic grand rounds no. 13. Cushing disease.

Although several effective therapies for Cushing disease have been demonstrated, associated complications, side-effects, and production of adrenal insufficiency indicate the continuing need for a completely satisfactory treatment. Of the surgical approaches to the adrenal gland, total adrenalectomy offers the most predictable amelioration, but is necessitates lifelong replacement and augmentation therapy and can be followed by progressive sella enlargement and hyperpigmentation. Drugs can inhibit adrenal steroid biosynthesis or selectively destroy the zona fasciculata. Inhibitors can be overwhelmed by endogenous ACTH increases, and cause variable electrolyte changes and side-effects. The adrenocorticolytic drug (o,pDDD) is slow in onset, irreversibly destroys adrenocortical reserve, and is poorly tolerated in the gastrointestinal tract. Conventional pituitary irradiation is unpredictably effective in only half of the cases, but is not often followed by the serious neurologic sequelae and hypopitiutarism observed after implantation and heavy particle therapy. Selective hypophysectomy via modern techniques is relatively new, but apparently offers a direct, and possibly, definitive therapeutic option in this disorder.

Adult↗

POEMS syndrome: studies in a patient with an IgG-kappa M protein but no polyneuropathy.

A 48-year-old woman had a variation of the syndrome of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (the so-called POEMS syndrome). The patient's neurological findings were entirely normal, but she had splenomegaly, hyperprolactinemia with galactorrhea and oligomenorrhea, a thyroid nodule with evidence of mild thyroiditis on aspiration biopsy specimen, and IgG-kappa monoclonal gammopathy, and hyperpigmentation and thickening of the skin. A short course of plasmapheresis (twelve 4-L exchanges in one month) did not alter any of the clinical abnormalities, but did result in a 70% decrease in the monoclonal IgG level (from 2.2 to 0.7 g/dl).

Biopsy, Needle↗