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[POEMS syndrome with primary adrenocortical insufficiency].

BACKGROUND: POEMS syndrome is a multisystem disorder. It usually presents as severe polyneuropathy and monoclonal gammapathy associated with endocrinopathies, organomegaly, skin hyperpigmentation. CASE REPORT: A patient with POEMS syndrome developed primary adrenocortical deficiency revelated by asthenia and hyperpigmentation. Hydrocortisone replacement therapy produced a rapidly beneficial effect on asthenia and hyperpigmentation. DISCUSSION: Although asthenia and skin pigmentation are common in POEMS syndrome, adrenocortical deficiency is seldom reported. This endocrinopathy might be underestimated in POEMS patients. We suggest systematic screening with a rapid ACTH test in all POEMS patients in order to detect underlying adrenal deficiency.

Adrenal Cortex Function Tests↗

Leg telangiectasia treatment with a 1.5 ms pulsed dye laser, ice cube cooling of the skin and 595 vs 600 nm: preliminary results.

BACKGROUND AND OBJECTIVE: Preliminary results indicate that pulsed dye lasers (PDL) with 1.5 ms pulsewidth and 595 nm wavelength are effective in the treatment of leg telangiectasia. The aim of this study was to evaluate if the clinical results could be improved by a) an effective skin cooling with ice cubes and b) the longer wavelength of 600 nm. STUDY DESIGN/MATERIALS AND METHODS: In 87 patients with vessels up to 1 mm in diameter, 257 single test treatments were performed using wavelengths of 595 and 600 nm, fluences of 16, 18, and 20 J/cm2, a 1.5 ms pulse duration, and an elliptical spot of 2 x 7 mm. In 7 patients, the skin surface temperature curve was measured after cooling with ice cubes vs hydrogel dressings, and spot geometry and fluence were investigated with and without the gel dressing. RESULTS: Vessel clearance was evaluated 6-8 weeks after treatment. 20 J/cm2 were most effective (80% clearance >50%), and 18 J/cm2 were more effective than 16 J/cm2 (66.2 vs 52.5% clearance >50%). There was a tendency towards better results with 595 nm, but the differences were not significant. Vessels with a diameter <0.5 mm cleared significantly better than those with 0.5-1 mm (69.1 vs 31.9% clearance >50%). Hypo- and hyperpigmentation were seen in 32% of the patients. Cooling with ice cubes proved to be far more effective than with hydrogel dressings (temperature decrease approx 15 vs 5 degrees C). Additionally, the gel dressing caused an energy loss of approx 35% and an irregular spot geometry as shown on burn paper. CONCLUSIONS: Treatment of leg telangiectasia with the 1.5 ms-PDL is safe and effective, especially in vessels smaller than 0.5 mm in diameter; 595 nm and 18 J/cm2 seem to be somewhat more effective as 600 nm and 16 J/cm2; and 20 J/cm2 are even more effective, but persistent hyperpigmentation cannot yet be excluded due to insufficient follow-up time. Cooling with ice cubes is more effective and less expensive than gel dressings, and the short term clinical results are equivalent, even if the frequency of transient pigmentary changes is increased.

Bandages↗

Linear and whorled nevoid hypermelanosis with bilateral giant cerebral aneurysms.

A 24-year-old woman presented with bilateral giant intracavernous carotid artery aneurysms manifesting as a cavernous sinus syndrome on the left side, and anisocoria, ophthalmic pain, and oculomotor paresis on the left side. Physical examination showed mild hyperextensibility of the metacarpophalangeal joints, amelogenesis imperfecta, and hyperpigmentation following Blaschko lines. Analysis of the NEMO gene for incontinentia pigmenti syndrome and of collagen III for Ehlers-Danlos type IV was normal. Skewed X-inactivation patterns in blood lymphocytes were detected. To the best of our knowledge, this association of linear hyperpigmentation and cerebral aneurysms has never been previously reported.

Adult↗

Optimal pulse durations for the treatment of leg telangiectasias with a neodymium YAG laser.

BACKGROUND: Leg veins can be effectively treated with lasers. However, the optimal pulse duration for small leg veins has not been established in human studies with a Nd:YAG laser. OBJECTIVES: The purpose of this study was to investigate a range of pulse durations to determine an optimal pulse duration for clearance of leg veins. STUDY DESIGN/MATERIALS AND METHODS: After mapping and photo documentation of the leg veins to be treated, a variable pulse duration Neodymium:Yttrium Aluminum Garnet (Nd:YAG) laser (3-100 milliseconds) was used in a single test site session. Pulse durations of 3, 20, 40, 60, 80, and 100 milliseconds were used. At the 3-week follow-up, the optimal pulse duration was defined as that pulse duration which resulted in the most complete clearance of vessels with the least side effects. Up to 20 vessels were then treated using the established "optimal" pulse duration. Final evaluation was at 16 weeks after the initial visit. Three blinded observers rated the percent of vessels completely cleared based on initial and final photographs. RESULTS: Eighteen patients completed the study. Fluence thresholds for immediate vessel changes varied depending on spot size and vessel diameter, with larger fluences required for smaller spot sizes and smaller vessels. Shorter pulse durations (< or =20 milliseconds) were associated with occasional spot sized purpura and spot sized post-inflammatory hyperpigmentation. Longer pulse durations (40-60 milliseconds) achieved superior vessel elimination with less post-inflammatory hyperpigmentation. With a single laser treatment, 71% of the treated vessels cleared. CONCLUSIONS: Compared to shorter pulses (<20 milliseconds), longer pulses may provide gentler heating of the vessel and a greater ratio of contraction to thrombosis.

Adult↗

Chediak-Higashi syndrome.

A case of Chediak-Higashi syndrome is reported in a four-year-old boy who presented with recurrent chest infection, partial albinism, hyperpigmentation of the extremities and presence of giant granules in leucocytes and melanocytes in the skin. Parental consanguinity was present. Though uncommon, hyperpigmentation of sun exposed areas may be the initial symptom in Chediak-Higashi syndrome.

Anti-Bacterial Agents↗

[Increasing pigmentation in Schmidt syndrome (polyglandular autoimmune syndrome type II].

An 18-year old man presented with increasing pigmentation in multiple nevi as well as so-called "eruptive nevi" over a 24 month period. The first excisional biopsy was from a plantar nevus which showed melanocytic hyperplasia. Following excisions of both hyperpigmented and newly appearing nevi showed only increased pigmentation. Two years after the first examination Addison-like hyperpigmentation was noticed in typical locations. Both Addison disease and Hashimoto thyroiditis were then confirmed by elevated serum levels of ACTH, TSH, and decreased levels of cortisol and L-thyroxine. This confirmed the diagnosis of Schmidt syndrome as part of polyglandular autoimmune disease (PGAS) type II. After another 18 months of treatment with hormone substitution of L-thyroxine and hydrocortisone, the hyperpigmentation resolved and the hyperpigmented nevi decreased in number.

Addison Disease↗

Human recombinant stem-cell factor induces melanocytic hyperplasia in susceptible patients.

BACKGROUND: Recombinant human stem-cell factor (SCF), a cytokine acting on hematopoietic progenitor cells, has potential for the treatment of several hematologic and oncologic disorders. In a hematology-oncology phase I trial of SCF, several patients had cutaneous hyperpigmentation at the SCF subcutaneous injection sites. OBJECTIVE: Our purpose was to investigate the pathogenesis of this hyperpigmentation phenomenon. METHODS: Skin biopsy specimens were obtained before, at the completion of, and after SCF therapy and were processed for histology, immunohistology, and electron microscopy. RESULTS: Skin at the site of SCF injection had an increased number of melanocytes, increased melanocytic dendrite extension, and melanin as compared with noninjected tissue. Immunohistochemical stains revealed an increase in staining with melanocyte-specific monoclonal antibodies HMB-45 and NKI/beteb, and a monoclonal antibody to the receptor for SCF, c-kit. CONCLUSION: Subcutaneous injection of SCF results in hyperplasia of melanocytes. SCF may be useful in the treatment of melanocytopenic disorders, but caution may be necessary in patients with disorders of melanocyte proliferation.

Antibodies, Monoclonal↗

A multicenter, randomized, double-blind trial of tazarotene 0.1% cream in the treatment of photodamage.

BACKGROUND: Previous studies indicate that tazarotene is efficacious in reducing signs of photodamage. Objective We sought to confirm the efficacy and tolerability of tazarotene 0.1% cream in the treatment of facial photodamage. METHODS: A total of 568 patients with at least moderate fine wrinkling or mottled hyperpigmentation applied tazarotene 0.1% cream or vehicle cream to their face once daily for 24 weeks. RESULTS: Tazarotene cream was significantly more effective than vehicle in reducing fine wrinkles, mottled hyperpigmentation, lentigines, irregular depigmentation, apparent pore size, elastosis, tactile roughness, and an overall integrated assessment of photodamage. Significance was achieved as early as week 2 for some parameters and had not plateaued by week 24. The majority of patients reported improvements in their photodamage as early as week 4. Adverse events were predominantly mild or moderate signs or symptoms of skin irritation. CONCLUSION: Once-daily tazarotene 0.1% cream is effective in ameliorating multiple signs of facial photodamage.

Adult↗

Increasing our understanding of pigmentary disorders.

This article focuses on developments in pigmentary disorders that extend dermatologists' understanding of the field. Areas that are reviewed include the basic biochemistry, pharmacology, and physiology of the melanocortin system; melanosome development; genetic diseases associated with pigmentary disorders; pigmentary disorders secondary to systemic disease; drug-induced hyperpigmentation; environmental exposure to chemicals; and primary disorders of hyperpigmentation such as melasma and lentigines. Basic, clinical, and epidemiological research, along with a number of clinical case reports, were included in the review. This article also reports on the new health-related quality-of-life instrument (MELASQOL) that has been developed for women with melasma.

Animals↗

Topical tretinoin (retinoic acid) treatment of hyperpigmented lesions associated with photoaging in Chinese and Japanese patients: a vehicle-controlled trial.

BACKGROUND: Hyperpigmented lesions are a predominant component of photoaging in Chinese and Japanese persons. Topical 0.1% tretinoin cream improves the hyperpigmentation associated with photoaging in Caucasian persons. OBJECTIVE: Our purpose was to assess the efficacy of 0.1% tretinoin cream treatment of hyperpigmented lesions associated with photoaging in Chinese and Japanese patients. METHODS: Forty-five photoaged patients (23 Chinese, 22 Japanese) completed a double-blind, randomized study in which 21 applied 0.1% tretinoin cream and 24 applied vehicle cream once daily to face and/or hands for 40 weeks. Patients' hyperpigmented lesions were evaluated clinically and by colorimetry throughout the study and by histologic analysis of skin biopsy specimens taken before therapy and at the end of treatment. RESULTS: At the end of treatment, hyperpigmented lesions of the face and hands were lighter or much lighter in 90% of patients receiving tretinoin compared with 33% receiving vehicle (p < 0.0001). Colorimetry demonstrated significant lightening of lesions after tretinoin compared with vehicle (p < 0.05). Histologic analysis of hyperpigmented lesions demonstrated a statistically significant 41% decrease in epidermal pigmentation with tretinoin therapy as compared with a 37% increase in the vehicle group (p = 0.0004). No patient withdrew for adverse effects. CONCLUSION: By clinical, colorimetric, and histologic evaluation, 0.1% tretinoin cream significantly lightens the hyperpigmentation of photoaging in Chinese and Japanese patients.

Administration, Topical↗

Phytophotodermatitis: the other "lime" disease.

Phytophotodermatitis is a skin eruption that occurs after contact with photosensitizing compounds in plants and exposure to UV light. There are two common presentations of phytophotodermatitis. Acutely, erythema and vesiculation similar to a severe sunburn are noted. After resolution of the inflammation, the involved skin has marked hyperpigmentation. Many plants have been identified that contain furocoumarins (psoralens), including limes, lemons, and celery. We present a patient with an acute phototoxic eruption and hyperpigmentation after contact with limes during a beach vacation.

Adult↗

Protective effect of hyperpigmented skin on UV-mediated cutaneous cancer development.

Recently, we crossed an original haired RET-transgenic mouse of line 242 with a hairless mouse and established a hairless RET-(HL/RET)-transgenic mouse line (242-hr/hr) with hyperpigmented skin but no tumors. In this study, we examined the effect of hyperpigmented skin in HL/RET-transgenic mice on UV irradiation-mediated cutaneous cancer development. UV irradiation to this mouse line never induced melanoma despite the presence of melanoma-inducible transgenic RET oncogenes. On the contrary, the hyperpigmented skin efficiently protected UV-mediated squamous carcinoma development in the skin. Probably underlying this result, hyperpigmentation protected the skin from damage and blocked the accompanying signal transduction for tyrosine phosphorylation of multiple cellular proteins and activation/phosphorylation of extracellular signal-regulated, c-Jun N-terminal, and p38 kinases. Thus, we demonstrated hyperpigmentation-mediated in vivo protection against UV irradiation-induced skin cancer.

Animals↗

Addison's disease in Africa--a teaching hospital experience.

OBJECTIVE: Addison's disease may present with diverse and non-specific clinical and biochemical features. Contentious issues include the appropriate criteria for the interpretation of the ACTH stimulation test and the necessary extent of investigation to identify a specific aetiology for the hypoadrenalism. The experience of Addison's disease at a South African teaching hospital was reviewed to (1) record the aetiology and spectrum of presentation, (2) examine the performance of the ACTH stimulation test and (3) determine the utility of adrenal CT scan and biopsy. METHODS: Retrospective study of patients admitted to a South African teaching hospital from 1980 to 1997 with a diagnosis of acute Addison's disease. PATIENTS AND MEASUREMENTS: Fifty patients presenting with acute Addison's disease were identified by a search of hospital records. Pretreatment biochemical and haematological parameters were recorded. The cortisol response at 20 and 60 min to an intravenous injection of 250 micrograms synacthen (Cortrosyn) was analysed. In a subgroup of affected subjects, the bone mineral density (BMD) in the lumbar spine and femoral neck was measured during long-term follow-up. RESULTS: Presenting features included hyperpigmentation (86%), weight loss (67%), abdominal pain (20%) and diarrhoea (16%). Thirty-nine patients (78%) were hyponatraemic, while 26 (53%) were hyperkalaemic. Nine patients (18%) were hypoglycaemic and 21% had hypercalcaemia. The mean basal cortisol was 148 nmol/l (range 10-487) and 16 patients (40%) had a normal basal cortisol. The mean cortisol 20 min after ACTH stimulation was 172 nmol/l (range 19-588). There was no significant increase in serum cortisol following ACTH stimulation (P > 0.05). Adrenal CT scans were performed in only 24 patients (48%) and were normal in 10, while abnormalities were detected in 14 patients (bilateral enlargement in 11, calcification in two and atrophic adrenals in one). Eight patients had a DEXA scan performed during follow-up--four were osteopaenic in the lumbar spine and five at the femoral neck. The probable aetiology of Addison's was idiopathic in 42%, related to active TB in 18%, old TB in 16%, autoimmune in 12% and malignancy with metastases in 6%--single cases were due to sarcoid, iron overload and adrenoleukodystrophy. Adrenal biopsy was performed in two patients and was diagnostic of malignancy in both cases. The mortality within the first month after hospitalization was 12%. CONCLUSIONS: In our experience, Addison's disease is frequently idiopathic, presents with protean manifestations and should be considered in patients with unexplained hyperpigmentation or gastrointestinal complaints, particularly when associated with hyponatraemia and hyperkalaemia. A normal basal cortisol does not exclude the diagnosis which requires ACTH stimulation testing.

Abdominal Pain↗

The Laugier-Hunziker syndrome.

Laugier and Hunziker described a syndrome consisting of asymptomatic benign areas of hyperpigmentation affecting the lips, buccal mucosa and, in 50%, the fingernails. We report a 67-year-old woman with the clinical features of Laugier-Hunziker syndrome in association with vulval pigmentation. Histology, immunohistochemistry and electron microscopy from the various areas of pigmentation on the body confirmed the benign nature of the pigmentation. We review potential causes of oral and genital pigmentation, and suggest an expansion of the original syndrome described by Laugier and Hunziker to include more widespread areas of benign hyperpigmentation, which may associated.

Aged↗

Comparison of the 585 nm pulse dye laser and the short pulsed CO2 laser in the treatment of striae distensae in skin types IV and VI.

BACKGROUND: Striae distensae, or stretch marks, are a very common cosmetic problem. The successful management of stretch marks has long been a source of frustration and curiosity for both the clinician and the researcher. Recent studies suggest lasers may have a role in their management. As yet, no study has reported on the effects of either of these lasers in the treatment of stretch marks on persons with skin types 4 to 6. OBJECTIVE: To assess the efficacy of short pulsed CO2 laser and pulsed dye laser for the treatment of stretch marks in skin types 4, 5, and 6. METHODS: Four patients were initially enrolled in the study. All were female with abdominal stretch marks, present for a range of 8 to 19 years. The patients had skin types ranging from 4 to 6. The test area consisted of a stretch mark long enough to be divided into three contiguous 2 cm sections, labeled A, B, and C. Section A served as the short pulsed CO2 test site, section B served as a control, while section C served as the 585 nm pulsed dye site. Patients were seen for evaluation after 1 week, then every 4 weeks for a total of 20 weeks. Patients were evaluated subjectively by the investigators, and the patients' own self-evaluation was reported as well. RESULTS: Following the 585 nm pulse dye laser, at 20 week follow-up patients with type 4 skin showed no improvement, while type 6 skin showed hyperpigmentation. The short pulsed CO2 test site showed persistent erythema in type 4 skin and marked hyperpigmentation in type 6 skin. CONCLUSIONS: For patients with types 4, 5, and 6 skin, laser treatment of striae should be avoided or used with great caution.

Carbon Dioxide↗

The safety and efficacy of salicylic acid chemical peels in darker racial-ethnic groups.

BACKGROUND: There is a dearth of published data regarding chemical peels in darker racial-ethnic groups. OBJECTIVE: The purpose of the present investigation was to assess the clinical efficacy and safety of a new superficial salicylic acid peel in individuals of skin types V and VI. METHODS: Twenty-five patients were included in this pilot investigation. Nine had acne vulgaris, 5 had post-inflammatory hyperpigmentation, 6 had melasma, and 5 had rough, oily skin with enlarged pores. The patients were pre-treated for 2 weeks with hydroquinone 4% prior to undergoing a series of five salicylic acid chemical peels. The concentrations of salicylic acid were 20% and 30%. The peels were performed at 2 week intervals. RESULTS. Moderate to significant improvement was observed in 88% of the patients. Minimal to mild side effects occurred in 16%. CONCLUSION: The results of this study suggest that superficial salicylic acid peels are both safe and efficacious for treatment of acne vulgaris, oily skin, textural changes, melasma, and post-inflammatory hyperpigmentation in patients with skin types V and VI.

Acne Vulgaris↗

An in vivo trial comparing the use of different types of 532 nm Nd:YAG lasers in the treatment of facial lentigines in Oriental patients.

BACKGROUND: Versapulse is a system that consists of four laser modalities and was developed with the aim of increasing cost-effectiveness. However, as these lasers share a common power supply, for the Q-switched (QS) Nd:YAG 532 nm laser a large spot diameter is necessary to lower the fluence to a suitable level. This can increase the risk of hyperpigmentation when used for the treatment of lentigines in dark-skinned patients. OBJECTIVE: The aim of our study was to access the clinical efficacy and the complication rate of the Versapulse QS Nd:YAG 532 nm laser, the Versapulse long-pulsed Nd:YAG 532 nm laser (without the chill tip), and a conventional QS Nd:YAG 532 nm laser in the treatment of lentigines in Chinese patients. METHODS: Thirty-four Chinese patients with lentigines were randomized to receive laser surgery, with one side of the face treated by one laser and the other side treated by a different system. Patients were monitored for 6 weeks to assess the degree of clearing, hyperpigmentation, hypopigmentation, and erythema. Assessments involved patients interviewed using a visual analog questionnaire and two blinded observers who evaluated the pre- and posttreatment clinical photographs. RESULTS: The Versapulse QS Nd:YAG 532 nm laser was associated with a statistically higher risk of complications. The Versapulse long-pulsed Nd:YAG 532 nm laser was compatible with the conventional QS Nd:YAG 532 nm laser in terms of clinical efficacy and complication rates. CONCLUSION: The Versapulse long-pulsed 532 nm laser is more effective and should be used instead of the Versapulse QS Nd:YAG 532 nm laser for the treatment of lentigines in dark-skinned patients.

Adult↗

Clinical trial of bleaching treatment with 10% all-trans retinol gel.

BACKGROUND: Although an aggressive use of tretinoin along with hydroquinone enables an efficient treatment of hyperpigmented skin lesions, irritant dermatitis remains to be solved. OBJECTIVE: To evaluate the efficiency and adverse effects of 10% all-trans retinol (ROL) gel for improvement of skin hyperpigmentation. METHODS: Ten-percent ROL gel was used instead of 0.1% tretinoin gel in our two-phased bleaching protocol (bleaching and healing phases); 5% hydroquinone and 7% lactic acid ointment were used along with ROL gel in the bleaching phase (2 to 6 weeks). Five-percent hydroquinone and 7% ascorbic acid ointment were used alone during the healing phase (4 to 6 weeks). Twenty-one Japanese patients with hyperpigmented lesions on the face were enrolled in this study, and 18 patients who were followed for more than 10 weeks were analyzed. RESULTS: Improvement of pigmentation was seen in 16 of 18 patients after an average treatment period of 11.3 weeks, and in 6 patients, pigmentation was almost eliminated after treatment. Erythema and scaling were seen, however, during the bleaching phase as well as the bleaching treatment with tretinoin gel. CONCLUSION: ROL can improve skin hyperpigmentation to a similar extent to tretinoin when used at high concentration, whereas it induces irritant dermatitis as well.

Adult↗