1/1108. Photoinduced dermal pigmentation in patients taking tricyclic antidepressants: histology, electron microscopy, and energy dispersive spectroscopy. Two patients had been taking long-term tricyclic antidepressant therapy. Each developed a blue to slate-gray hyperpigmentation in sun-exposed areas. On histologic examination there were refractile golden brown granules free in the dermis along collagen bundles. Similar pigment was present in macrophages and along the basement membrane zone. The granules stained for melanin, but not for iron, and were bleached by the permanganate method. Electron microscopy showed varying size and shaped electron-dense granules within lysosomes and free in the dermis, which, in unstained sections, showed a less dense peripheral halo. This peripheral halo was also evident on light microscopy. Energy dispersive spectroscopy showed these granules to be rich in copper and sulfur (elements present in tyrosinase and pheomelanin, respectively). We believe that this represents a drug-melanosome complex, which is most likely caused by chronic photoactivation. ( info) |
2/1108. Phakomatosis pigmentovascularis: A new case with renal angiomas and some considerations about the classification. We report phakomatosis pigmentovascularis detected in a Caucasian child characterized by the presence of a nevus flammeus and nevus anemicus on the face, a telangiectatic linear nevus of the right leg, and a very extensive blue spot covering 60% of the body surface, with ocular melanosis. Multiple angiomatous lesions of the kidney are associated without alterations of the central nervous system (CNS). This association has not been reported before; it could be a further expression of the complex of developmental defects. Our case corresponds exactly to type IIb in the classification of phakomatosis pigmentovascularis proposed by Hasegawa. As this classification seems very extensive, the higher incidence of cases corresponding to the second subtype suggests that we should identify it by the term phakomatosis pigmentovascularis, while the others could be considered as only very uncommon variants. ( info) |
3/1108. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease. We diagnosed phakomatosis pigmentovascularis type IIIb in an 11-month-old baby who had a giant nevus spilus, a nevus flammeus, and moyamoya disease. Development of the patient was normal until 6 months of age when he developed a sudden onset of focal seizures and left hemiparesis. This patient represents the sixth case of phakomatosis pigmentovascularis type IIIb, including three cases in the Japanese literature, reported thus far. However, to our knowledge, this is the first case with an association to moyamoya disease. ( info) |
4/1108. E.N.T. manifestations of Von Recklinghausen's disease. Von Recklinghausen's Disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade. ( info) |
| prurigo pigmentosa is a papular pruriginous eruption that leaves a marble-like pigmentation. The majority of cases have been found in japan. Three new female. Sicilian patients with prurigo pigmentosa were studied. All of them had previously been diagnosed as having different types of dermatitis. The administration of minocycline, at a dosage of 100 mg/day for 1 month, induced the disappearance of the papular eruption and pruritus in two patients, with an improvement of the gross reticular pigmentation. The third showed no modifications of the clinical picture after 2 months of minocycline treatment, but her condition significantly improved after 1 month of treatment with diaminodiphenylsulfone, 100 mg/day. These observations allow us to suggest that prurigo pigmentosa might be relatively frequent but misdiagnosed in the Sicilian population. ( info) |
6/1108. Pigmented purpuric dermatosis in a young male. The pigmented purpuric dermatoses (PPD) are a group of disorders that most often occur on the lower extremities of middle-aged adults as asymptomatic, yellow-orange patches with petechiae. The PPD represent a benign, often chronic, capillaritis of unknown cause. We present a case of PPD on the abdomen of a young male and emphasize careful observation in such a patient to reveal possible progression to purpuric mycosis fungoides. ( info) |
7/1108. Erythrosis pigmentosa mediofacialis (Brocq) and erythromelanosis follicularis faciei et colli in the same patient. Erythrosis pigmentosa peribuccalis (Brocq) (or erythrosis pigmentosa mediofacialis) and erythromelanosis follicularis faciei et colli, have been regarded as different disorders, mainly because the first occurs on the mediofacial area and is common in women and the second mostly occurs pre-auricularly in men. Both conditions show histological signs of abnormal follicular keratinization with teleangiectasia and round cell infiltrate. An increase in the level of melanin has been seen in some patients. We describe here a woman in whom lesions started in the middle of the face and later became evident in the pre-auricular area. This suggests that the two conditions are in fact the same disease. As a neutral term for this not uncommon disorder we propose erythrosis pigmentosa faciei et colli. ( info) |
8/1108. erythema dyschromicum perstans in early childhood. erythema dyschromicum perstans (EDP) is a rare disorder characterized by asymptomatic, slowly progressive, ash-gray macular pigmentation of the skin which usually occurs from age 5 through adult life. We have experienced two cases of EDP in children aged 2 and 3, both exceptionally younger than the previously reported cases. We therefore suggest that EDP should be included in the differential diagnosis of pigmentary disorders occurring at an early age. ( info) |
| We describe a woman with dyskeratosis congenita (DKC), microcephaly, and a purple discoloration of the tongue. The latter findings are not commonly described in males with DKC, have been reported in another female patient with this condition, and may represent the phenotype of an autosomal recessive entity of DKC. Results of x chromosome inactivation studies did not support X-linked DKC in our family. The additional findings of an affected brother and parental consanguinity support the hypothesis of autosomal recessive inheritance. ( info) |
10/1108. Painful pigmented prosthesis pressure papules. Two patients who presented with tender hyperkeratotic papules involving their weight-bear.ing amputation stumps were studied. Although these clinical findings are common in the experience of prosthetists, the author has been unable to find any documentation of these findings in the literature. All attempts at relining and reshaping the cups of the original offending prostheses by experienced prosthetists were unsuccessful. The eruption cleared only after entirely new prostheses were designed. It would appear that this condition is due to a poorly fitting prosthesis. ( info) |