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Postsclerotherapy pigmentation. Is serum ferritin level an accurate indicator?

BACKGROUND: Human beings have suffered and sought treatment for disease of veins as early as the recordings of the old testament. The use of irritating sclerosing agents have been and are widely used today to treat varicose veins and telangiectasia. One of the most common and cosmetically significant side effects of sclerosing agents is varying degrees of hyperpigmentation. It has been reported that elevated serum ferritin level plays a role in this postsclerotherapy pigmentation. OBJECTIVE: To support or negate the possibility of a direct correlation between serum ferritin levels and pigmentation postsclerotherapy using for our investigation a patient with hemochromatosis. METHODS: A patient with hemochromatosis having a serum ferritin level of 1200 was treated for spider veins. Clinical and histologic studies were performed pretreatment and posttreatment. RESULTS: There was no clinically apparent hyperpigmentation noted on the patient after sclerotherapy over a 6-month period. Histology reports revealed macrophagic pigmentation both pretreatment and posttreatment. CONCLUSION: Our results do not confirm the theory that lab values of elevated serum ferritin correlate with pigmentation postsclerotherapy. Further study of the correlation between postsclerotic pigmentation and serum ferritin levels are needed. One would anticipate that if a true correlation existed, then an extreme case such as this would clearly support this theory.

Ferritins↗

Reflex sympathetic dystrophy. Occurrence of inflammatory skin lesions in patients with stages II and III disease.

Reflex sympathetic dystrophy is a poorly understood syndrome of posttraumatic pain and sympathetic nervous aberration. We have observed previously unreported cutaneous manifestations of reflex sympathetic dystrophy. Seven patients with reflex sympathetic dystrophy were referred to our institution because of skin disorders. Three had recurrent ulcerating papules, and two had reticulate hyperpigmentation. Xerosis was common, and cutaneous atrophy was infrequent. Cutaneous ulceration and reticulate hyperpigmentation are previously unappreciated aspects of reflex sympathetic dystrophy. Further investigation regarding neural influences on the skin is warranted.

Adult↗

'Dyschromia in confetti' as a side effect of topical immunotherapy with diphenylcyclopropenone.

"Dyschromia in confetti" is a pigmentary disturbance that so far has not been reported to have occurred during topical immunotherapy. In a group of 243 patients treated with diphenylcyclopropenone for alopecia areata, four patients showed a similar pattern of hyperpigmentation and hypopigmentation. In one patient the pigmentary disturbance was confined to the site of diphenylcyclopropenone application on the scalp. Besides dyschromic skin changes on her scalp, one patient had development of a hyperpigmentation on her forearms. In the remaining two patients, dyschromia in confetti became manifest exclusively in areas other than the scalp. This type of pigmentary disturbance appeared 4 to 14 months after the start of treatment, and it has remained unchanged in three patients during a follow-up period of 3 to 10 months. In one patient, the pigmentary changes disappeared when therapy was discontinued but recurred, albeit to a lesser extent, after treatment had been resumed. We conclude that dyschromia in confetti should be regarded as a possible, but probably rare, side effect of topical immunotherapy with diphenylcyclopropenone, especially in individuals with a rather dark complexion.

Adult↗

Radiation lentigo. A distinct cutaneous lesion after accidental radiation exposure.

BACKGROUND: Accidental exposure of skin to ionizing radiation leads to long-term alterations such as fibrosis, keratosis, and teleangiectasias. Also, noncharacteristic hyperpigmentation and hypopigmentation may be noted. OBSERVATIONS: A distinct lesion is described on the calves of a white male survivor of the 1986 nuclear accident at Chernobyl, Ukraine. Several years after the accident at Chernobyl, characteristic pigmented macules developed in the areas of skin that had previously been exposed to ionizing radiation: there was a marked, sharply demarcated lentiginous hyperpigmentation of epidermal and basal keratinocytes and melanocytes, as well as an increase in the number of melanocytes. No cellular atypia was noted. CONCLUSIONS: This case demonstrates the potential of high single doses of ionizing radiation to induce pigmented lesions with similar clinical and histological features as they have been described after exposure to natural UV radiation or radiation from a tanning bed or sunlamp or after therapy with oral psoralen with long-wave UV-A radiation (PUVA), described as solar, tanning bed, and PUVA lentigines. The absence of cellular atypia may account for a favorable prognosis and enables clear distinction from more serious diagnoses such as lentigo maligna melanoma.

Humans↗

Efficacy of 0.1% tazarotene cream for the treatment of photodamage: a 12-month multicenter, randomized trial.

OBJECTIVE: To determine the efficacy and safety of 0.1% tazarotene cream for the treatment of photodamage. DESIGN: A 24-week multicenter, double-blind, randomized, vehicle-controlled intervention study followed by a 28-week open-label extension. SETTING: Ambulatory patients in private and institutional practice. PATIENTS: Of 563 patients with facial photodamage, 91% and 86% completed the double-blind and open-label phases, respectively. In the double-blind phase, 20 of 283 tazarotene-treated patients and 1 of 280 vehicle-treated patients discontinued treatment owing to adverse events. INTERVENTION: Once-daily application of 0.1% tazarotene cream or nonmedicated vehicle cream to the face for 24 weeks. Then, all continuing patients received treatment with 0.1% tazarotene cream for another 28 weeks. MAIN OUTCOME MEASURES: Primarily, fine wrinkling and mottled hyperpigmentation. Also, lentigines, elastosis, pore size, irregular depigmentation, tactile roughness, coarse wrinkling, telangiectasia, actinic keratoses, overall integrated assessment of photodamage, global response to treatment, patients' overall assessment of photodamage, and plasma levels of tazarotenic acid. RESULTS: Compared with the vehicle, at week 24 tazarotene resulted in a significantly greater incidence of patients achieving treatment success (>or=50% global improvement) and at least a 1-grade improvement in fine wrinkling, mottled hyperpigmentation, lentigines, elastosis, pore size, irregular depigmentation, tactile roughness, coarse wrinkling, and the overall integrated assessment of photodamage (P<.01). Additional clinical improvement occurred with continued tazarotene treatment and had not plateaued by week 52. Plasma tazarotenic acid concentrations did not exceed 0.71 ng/mL. CONCLUSIONS: Once-daily applications of 0.1% tazarotene cream significantly reduced multiple signs of photodamage. Plasma levels of tazarotenic acid remained below those of endogenous retinoids.

Administration, Topical↗

Junctional CD8+ cutaneous lymphomas with nonaggressive clinical behavior: a CD8+ variant of mycosis fungoides?

OBJECTIVE: To evaluate the clinical and prognostic features in primary cutaneous CD8+ T-cell lymphomas, which are rare and considered to be aggressive cutaneous lymphoproliferative disorders. DESIGN: Single-center retrospective study. SETTING: Lymphoma clinic (referral center) of a university hospital. PATIENTS: Three patients presented with CD8+ cutaneous lymphoma characterized by a patchlike pattern and hyperpigmentation. RESULTS: Histological analysis revealed a CD3+, CD8+ small-cell infiltrate showing a remarkable affinity to the dermoepidermal junction zone. Clonality for the T-cell receptor gamma chain was detected by polymerase chain reaction followed by denaturing gradient gel electrophoresis. The clinical presentation lasted several years (6 and 9 years, respectively) before the correct diagnosis was made. Treatment with nontoxic approaches (UV-B and local steroids) was successful. Aggressive clinical behavior was not observed. CONCLUSIONS: Our 3 cases of junctional CD8+ cutaneous T-cell lymphomas were characterized by hyperpigmentation and nonaggressive clinical behavior. This type of lymphoma, which can be considered a CD8+ mycosis fungoides variant, must be distinguished from other types of cutaneous CD8+ lymphomas so that overtreatment can be avoided.

Adult↗

Choroidal melanoma in a 14-year-old patient with ocular melanocytosis.

A 14-year-old male adolescent with ocular melanocytosis and secondary glaucoma in the left eye had a 2-year history of a progressively enlarging fundus lesion. Ocular examination revealed diffuse hyperpigmentation of the episclera and a smooth velvety thickening and hyperpigmentation of the left iris. Ophthalmoscopy disclosed diffuse choroidal pigmentation and a pigmented mass that occupied the macular area and surrounded the optic nerve. Ultrasonography showed an acoustically hollow lesion with scleral bowing and choroidal excavation. Based on clinical and ultrasonographic findings, the diagnosis was choroidal melanoma in a young patient with ocular melanocytosis. The eye was enucleated. Histopathologic examination revealed ocular melanocytosis with diffuse uveal melanocytosis and amelanotic malignant melanoma of the choroid. The choroidal melanoma apparently arose from a preexisting choroidal nevus. Even young patients with ocular melanocytosis should have regular follow-up examinations for early detection of uveal melanoma.

Adolescent↗

Comparing the effectiveness of 585-nm vs 595-nm wavelength pulsed dye laser treatment of port wine stains in conjunction with cryogen spray cooling.

BACKGROUND AND OBJECTIVES: The objective of this study was to compare the efficacy and safety of cryogen spray cooled laser treatment (CSC-LT) at wavelengths of 585 nm vs. 595 nm for port wine stain (PWS) birthmarks in a large series of patients. STUDY DESIGN/MATERIALS AND METHODS: A retrospective review was conducted of 64 patients with PWS treated with the ScleroPLUS [Candela (Wayland, MA)] pulsed dye laser (lambda = 585 or 595 nm wavelength; spot size 7 mm, tau(p) = 1,500 microseconds) over a 3-year period. Subjects' ages ranged between 3 months and 64 years; there were 42 females and 22 males, all of whom were Asian. Number of treatments ranged from 1 to 6. Duration of treatment ranged from 6 months to 2 years 11 months, with a mean of 12 months. Patients (n = 32) received CSC-LT (585 nm) using radiant exposures of 7-10 J/cm(2). A second group of patients (n = 32) received CSC-LT (595 nm) using radiant exposures of 7-10 J/cm(2). The primary efficacy measurement was the quantitative assessment of blanching response scores for CSC-LT (585 nm) versus CSC-LT (595 nm). Patients were monitored for adverse effects. RESULTS: Based on chi-squared analysis, there were clinical, and statistically significant, differences in blanching response scores favoring PWS receiving CSC-LT (585 nm) as compared to CSC-LT (595 nm) (P <.001). Transient hyperpigmentation was noted in 43.7% (n = 14) and 37.5% (n = 12) of patients in the CSC-LT (585 nm) and CSC-LT (595 nm) groups, respectively. In both groups, transient hyperpigmentation resolved in all patients within 1 year. Permanent hypopigmentation or scarring was not observed in either group. CONCLUSIONS: CSC-LT (585 nm) resulted in superior blanching as compared to CSC-LT (595 nm). Further study is required to optimize wavelength selection on an individual patient basis during PWS therapy in order to improve treatment results.

Adolescent↗

Bilateral testicular enlargement due to adrenal remnant in a patient with C11 hydroxylase deficiency congenital adrenal hyperplasia.

The case of a 15-yr-old boy with C11 hydroxylase deficiency congenital adrenal hyperplasia is reported who was diagnosed and treated as true precocious puberty at the age of 2 yr because of virilization and bilateral testicular enlargement. He later developed hyperpigmentation, hypertension and short stature and because of an increase in testes size he underwent testicular biopsy with the assumption of Leydig cell tumor. With the intake of glucocorticoids his testes size, hypertension and hyperpigmentation improved markedly. We could find only 6 such cases in the literature and have reviewed their clinical and laboratory data. All patients showed the picture of virilization with hypertension. Leydig cell tumor was proposed as the differential diagnosis in all cases except ours. Ultrasonography was able to show testicular adrenal-like tissue in all those in whom the procedure was undertaken. In the 5 patients of whom we could find enough data, 1 responded partially and 4 responded markedly to corticosteroid therapy with shrinkage of testicular tumors. We conclude that clinical findings and US are very important in the early diagnosis of these patients and with adequate treatment most cases show shrinkage in testicular tumors.

Adolescent↗

[Manifestation of Cushing syndrome and osteoporotic fractures in pregnancy in a patient with Carney complex].

HISTORY AND CLINICAL FINDINGS: In a 31-year-old patient a conventional X-ray was performed due to persistent pain at the lumbar spine level after a cesarean section. It revealed compression fractures of L2 and L3. Besides very clear clinical signs of hypercortisolism, multiple hyperpigmentations and naevi in the patient's face including the lips and the conjunctiva of the right eye were visible, suggesting a Carney complex. INVESTIGATIONS: Insuppressible cortisol levels confirmed an adrenal origin of hypercortisolism. A selective catheterization of adrenal veins supported the presence of bilateral adrenal cortisol production. The computed tomography showed nodular lesions in the right and a hyperplasia of the left adrenal gland. TREATMENT AND COURSE: A bilateral adrenalectomy was performed and a primary pigmented nodular adrenal hyperplasia was confirmed histologically. Clinical signs of hypercortisolism rapidly resolved after adrenalectomy. CONCLUSIONS: The diagnosis of Cushing's syndrome as a part of Carney complex was diagnosed at the end of a pregnancy although signs of hypercortisolism were present a long time before. The rare diagnosis of Carney complex should be considered in patients exhibiting symptoms of hypercortisolim and the typical clinical signs (hyperpigmentations).

Adrenal Cortex↗

[Q-switched ruby laser in dermatologic therapy. Use and indications].

The Q-switched ruby laser (QSRL) with its wavelength of 694 nm and a pulse duration of around 40 nsec is an effective modality for the removal of tattoos and cutaneous pigmented lesions. Based on the principle of selective photothermolysis, selective damage to cutaneous pigment or pigmented cells is possible, allowing the scar-free elimination of endogenous or exogenous pigment in the skin. Main indications for the treatment with the QSRL are tattoos (amateur, professional, accidental, or cosmetic) and lentigines but the QSRL can also be used for lightening or even removing other pigmented lesions such as nevus spilus or café au lait macules. Furthermore, pigmented lesions of mucous membranes can be removed easily. Since treatment results in postinflammatory hyperpigmentation, myoplasma, and Becker' nevus have proven to be inconsistent, the QSRL cannot be routinely recommended for these lesions. Melanocytic lesions are generally not treated, with the exception of nevus of Ota and nevus of ito where there exacts a lack of therapeutic alternatives. Non-pigmented cells, which exist in nearly all melanocytic lesions, do not absorb the light of the QSRL and, therefore, do not react to this particular treatment. No information is available on the risk of partially damaged cells to become malignant after QSRL treatment. The QSRL is an excellent therapy for the removal of endogenous and exogenous pigment because of both the excellent treatment results and the lack of side effects, which are limited to transient hypo- and hyperpigmentation. The QSRL has occurred a wide range of applications within the field of dermatology.

Humans↗

[Pigmented retinopathy as a presenting sign of mitochondrial encephalomyopathy without external ophthalmoplegia].

BACKGROUND: Mitochondrial encephalomyopathies result from deletions in the nuclear or mitochondrial (mt) DNA. Deletions in the mtDNA are often sporadic. Mitochondriopathies are commonly associated with chronic progessive external ophthalmoplegia (CPEO). Here we describe a patient with a structural mtDNA aberration whose presenting sign was impaired visual acuity in the presence of a pigmented retinopathy but lack of impaired ocular motility. PATIENT: A 7-year-old girl presented with impaired visual acuity (0.4 OD and 0.5 OS), coarse hyperpigmentation of the posterior pole and diffuse hyperpigmentation with irregular depigmentation in the periphery. Scotopic and photopic as well as multifocal ERG were abnormal. Further symptoms included an incomplete inner ear deafness, ataxia, lapses of coordination and an intention tremor. Compared with her twin sister, the patient's speech was less modulated and slower. MRI scanning disclosed symmetric changes of density in the basal ganglia and nucleus dentatus as well as in the brainstem. ECG yielded no evidence of an AV-node block. Molecular biological analysis showed a structural rearrangement of the mtDNA. CONCLUSIONS: Mitochondrial encephalomyopathies in early ages may present with pronounced retinal changes in the absence of external ophthalmoplegia.Therefore, it appears prudent to include a neuropediatric evaluation as well as a mutation screening of the mtDNA in the evaluation of pediatric patients with diffuse non-specific pigmented retinopathies.

Blotting, Southern↗

Infantile systemic hyalinosis: report of three Iranian children and review of the literature.

Infantile systemic hyalinosis (ISH) is a rare autosomal recessive disease characterized by diffuse hyaline deposits in the skin, gastrointestinal tract, muscle and endocrine glands. The clinical features are evident either at birth or within 6 months of life. The disease is manifested by painful progressive joint contractures, thick skin with hyperpigmentation, susceptibility to bone fractures, infections, failure to thrive and persistent diarrhea due to protein-losing enteropathy. Here, we report three unrelated Iranian children with a limited range of joint movements in the first month of life, skin hyperpigmentation and painful joint contractures. Pathological findings also confirmed the diagnosis of ISH in these patients.

Contracture↗

Annular macular dystrophy.

Of 21 individuals from three generations of a single family with annular macular dystrophy, six showed only dyschromatopsia, one had dyschromatopsia and foveal hyperpigmentation, and four individuals had dyschromatopsia, foveal hyperpigmentation, and perifoveal circular pigment epithelial atrophy. Normal findings obtained on electrophysiologic testing suggest that this is a focal (macular) disorder rather than a generalized fundus disorder.

Adolescent↗

Pigment changes in human skin after cryotherapy.

We have investigated the changes in pigmentation and melanocyte distribution in human skin after a standardized freeze injury. All lesions developed hypopigmentation with a peripheral rim of hyperpigmentation. Abnormalities in pigmentation persisted for at least 6 months. Hyperpigmentation was predominantly an epidermal phenomenon. After brief freezes, hypopigmentation persisted despite the presence of functional melanocytes. After prolonged freezes, the consistent finding was an absence of melanosomes in keratinocytes, although melanocytes were present. We conclude that prolonged changes in skin color are frequent after brief freezes and that hypopigmentation is not synonymous with an absence of melanocytes. This suggests that hypopigmentation after the cryosurgical treatment of malignant melanocytic tumors may not equate with cure.

Adult↗

Oral melanotic macules that develop after radiation therapy.

External beam radiotherapy treatment of malignant conditions in the head and neck can give rise to several adverse oral effects if the oral or salivary tissues are within the field of beam. We report on a patient with widespread oral melanotic hyperpigmentation that developed after a course of radiotherapy for metastatic carcinoma in the cervical lymph nodes. As no other local or systemic cause was evident it is possible this abnormal hyperpigmentation was a result of the radiotherapy. The development of oral melanotic macules as a consequence of radiotherapy has not been previously described.

Carcinoma, Squamous Cell↗

Porphyria cutanea tarda in Ethiopia.

The clinical, biochemical and histological features of 75 Ethiopians with porphyria cutanea tarda (PCT) are described. PCT in Ethiopia is definitely related to alcohol abuse and there is no clinical evidence for hereditary predisposition. Significant elevation of transaminases and bromsulphthalein retention, moderate to marked hepatic siderosis and inflammation with little or no fibrosis, suggestive of mild or likely reversible parenchymal changes, characterize the liver affection in these patients. A similar study of 18 patients with hyperpigmentation of the face and hands but without blisters, an unexplained but common feature of liver disease in Ethiopia, revealed that serum iron and urinary uroporphyrin levels were normal in eight but significantly elevated in ten (56%). Neither elevated serum iron and hepatic siderosis nor increased urinary uroporphyrin completely explains the hyperpigmentation in this group of patients.

Adult↗

Corticotroph cell hyperplasia in a patient with Addison disease: case report.

A woman with Addison disease developed hyperpigmentation, headache, and nausea despite conventional replacement therapy with cortisone. Excessively elevated plasma adrenocorticotropic hormone (ACTH) with absence of response to administration of corticotropin-releasing factor (CRF), and roentgenological evidence of enlargement of the sella turcica, as well as detection of enlarged pituitary gland on magnetic resonance images, led to a diagnosis of ACTH-producing microadenoma, which was removed by transsphenoidal microsurgery. The specimen obtained at surgery evidenced corticotroph hyperplasia, as demonstrated by immunohistochemical staining for ACTH. Fine structure exhibited densely granulated cells with a few bundles of microfilaments and an abundance of large lysosomal bodies. Surgical removal of the hyperplasia alleviated the patient's symptoms, and hyperpigmentation faded remarkably. Her plasma ACTH level returned to normal, has remained normal for more than 3 years, and responds adequately to CRF administration.

Addison Disease↗