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Distinctive cutaneous lesions frequently accompany and occasionally precede the intestinal lesions in Gardner's syndrome, Peutz-Jeghers syndrome, and Muir's syndrome. Awareness of the dermatologic manifestations of these entities may facilitate early diagnosis in these potentially life-threatening hereditary disorders.
Incontinentia pigmenti is a familial disorder affecting tissues derived from neuroectoderm. Statistical analysis of reported pedigrees is consistent with transmission of incontinentia pigmenti by an X-linked dominant gene with male hemizygote lethality. This report describes a male infant with the classic clinical features of this condition and a 47,XXY chromosomal constitution. These findings support the concept that incontinentia pigmenti is an X-linked dominant disorder. This case illustrates the importance of a full genetic investigation in all males with physical findings suggestive of an X-linked dominant disorder lethal in males.
Pigmentary disorders are commonly seen in ethnic skin. They are psychologically problematic in darker skin. Treatment of many of these disorders remains difficult.
BACKGROUND: Non-ablative photorejuvenation is characterized by the reduction of intrinsic and extrinsic changes in photodamaged skin. Only short-term improvement has been documented previously. OBJECTIVE: To evaluate quantitatively the short-term and long-term clinical effectiveness of multiple full-face IPL treatments for non-ablative facial photorejuvenation. METHODS: A total of 47 patients with varying degrees of photodamaged skin and rosaceal dermatitis underwent a series of four to five IPL treatments with a Vasculight (Lumenis Corp). Treatments were conducted every 3-4 weeks. Photographs were taken at baseline and after the treatment series was completed at both 6 weeks and 6 months. Adverse effects and clinical improvement were documented. A patient satisfaction questionnaire was completed and reviewed at the 6-month evaluation period. RESULTS: Standardized evaluation of rhytids showed a statistically significant improvement in wrinkles at both evaluation endpoints. Some degradation occurred over time. Facial vascularity, dyschromia, and large pore size progressively improved from the 6-week measurement to the 6-month measurement. CONCLUSION: This clinical study demonstrates that non-ablative facial rejuvenation is associated with long-term clinical improvement of facial rhytids, abnormal vascularity and pigmentary disorders with minimal risks and side effects to the patient.
Familial melanopathy with giant melanocytes is a unique pigmentary disorder that to the best of the author's knowledge has not been described before. The clinical and pathological features of this disorder, herein described in four patients, are very characteristic and can be easily discerned both grossly and histologically. More patients with this disorder will probably now be recognized, particularly in the Far and Middle East.
Pigmentary disorders resulting from the prolonged use of the semisynthetic tetracycline derivative antibiotic, minocycline, are rare but well recognized sequelae of ingestion of this drug. We present two cases of women with bullous pemphigoid who developed blue-black pigmentation in areas of scarring on the legs after oral minocycline therapy. On histologic examination, the deposited pigment was localized within dendritic cells limited to the upper dermis. Immunohistochemical analysis revealed these cells to be factor XIIIa positive dermal dendrocytes which was confirmed by transmission electron microscopy. We believe these cases demonstrate, in-situ, the phagocytic capabilities of this recently described cell population.
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We describe four patients with Waardenburg syndrome and Hirschsprung aganglionic megacolon. In view of pathophysiologic relationships and animal studies, we conclude that the association of these two uncommon disorders is pathophysiologically significant.
A physical examination with detailed dermatological examination was made on 28 Japanese male workers, 22 to 63 years old, who had been exposed to arsenic for one to 24 years, in processes manufacturing lead arsenate, calcium arsenate and so on to be used as insecticides. Many corn-like punctate keratoses were seen on the palms and soles of 7 workers. The histopathological features in 6 specimens of punctate keratoses of palms and other regions of 4 workers were hyperkeratosis, acanthosis and elongation of rete ridge, but no malignant changes, although there were mild disorderly arrangements in part of stratum malpighii. Multiple lesions of sharply demarcated dullred patch with scale were found on the trunk and extremities of 3 workers. These lesions histopathologically showed the typical feature of Bowen's disease. Characteristic bronze pigmentation on the trunk and extremities was seen in 6 workers, and 5 of these 6 workers showed tiny normal or depigmented skin (raindrops) throughout the involved areas. These corn-like punctate keratoses mainly on the palms and soles, multiple Bowen's disease and diffuse bronze pigmentation with raindrop areas of hypopigmentation on the trunk and extremities were manifest almost in the same individuals, and it was concluded that they were characteristic cutaneous symptoms of chronic arsenical poisoning. There was no correlation between the intensity of cutaneous manifestations and the term of exposure to arsenic. Cutaneous manifestations due to arsenic were generally recognized as follows: diffuse pigmentation appears at first in one to two years after exposure to arsenic, then punctate hyperkeratotic lesions appear mainly on the palms and soles, and Bowen's lesions occur after several years or more. The use of arsenic has been abandoned in this factory since January, 1971. Diffuse bronze pigmentation now seems to be considerably lightened, as long time has passed after exposure to arsenic. However, since punctate hyperkeratotic lesions on the palms, soles and others may, though rarely, undergo malignant transformations, new lesions of Bowen's disease may further occur and these lesions may develop to invasive squamous cell carcinoma, physical examinations of the workers exposed to arsenic should be continued and careful observation of them is necessary for the future.
We describe a case of a 28-year-old woman affected by celiac disease (CD) associated with rare multiple disorders of the cutaneous pigmentary system: atypical mole syndrome and congenital giant naevus. Some other rare skin lesions have been reported in association with celiac disease such as cutaneous sarcoidosic granuloma, bullous pemphigoid, ichthyosis, alopecia areata, erythema elevatum diutinum, sclero-atrophic lichen and primary cutaneous amyloidosis. This is the 1(st) report concerning celiac disease and congenital disorders of the pigmentary system.
Case reports of oculodermal melanocytosis (nevus of Ota) and ipsilateral glaucoma often have other ocular disorders which could account for the glaucoma. It has not been clearly shown that the oculodermal melanocytosis alone can cause secondary glaucoma. In the present case report, optic nerve head and visual field changes, suggestive of a long-standing unilateral elevation of intraocular pressure, occurred in an eye with oculodermal melanocytosis and no other apparent ocular disorder to account for the chronic glaucoma.
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