1/184. Juvenile chronic myelogenous leukemia, neurofibromatosis 1, and xanthoma.The triple association of leukemia, xanthomatous skin lesions, and neurofibromatosis 1 (NF) was first described by Royer et al. in 1958. Most of the leukemias were of the juvenile chronic myelogenous type (JCML). We describe a 7-year-old male child with xanthoma, neurofibromatosis 1, and juvenile chronic myelogenous leukemia. His mother also had NF1. We suggest that the presence of xanthomas and NF1 in a young child should raise awareness of the possible development of JCML, especially in patients with a family history of NF1.- - - - - - - - - - ranking = 1keywords = xanthoma (Clic here for more details about this article) |
2/184. Late onset focal dermal elastosis: a distinct clinicopathologic entity?Two patients, aged 72 and 69 years, had skin lesions clinically resembling pseudoxanthoma elasticum (PXE). On histologic examination, the lesions showed an increased accumulation of normal-appearing elastic fibers lacking the abnormalities that characterize PXE. Similar lesions have been described previously in two elderly patients by Tojima et al. (1995). The clinical and histologic differential diagnosis of these lesions is discussed, and it is concluded that they represent a clinicopathologic entity with undetermined prevalence and etiopathogenesis.- - - - - - - - - - ranking = 0.14285714285714keywords = xanthoma (Clic here for more details about this article) |
3/184. Treatment of cutaneous lesions of xanthoma disseminatum with a CO2 laser.We describe a case of a 15-year-old African American girl with widespread papulonodular lesions of xanthoma disseminatum especially in the periorbital area and on the flexural surfaces of the neck, axillae, arms, and legs. There were no mucosal lesions. An initial trial in the distant past of liquid nitrogen cryotherapy resulted in partial shrinking of cutaneous lesions but was too painful for the patient. She then underwent surgical excision of bilateral eyelid lesions with improvement, but additional procedures were deemed impractical when considering the great number and size of the lesions. Consequently we treated the patient with a carbon dioxide (CO2) laser with excellent results. The relatively great speed at which the CO2 laser procedure can be performed has made the removal of multiple lesions in each session possible. Additional advantages included precise vaporization of lesions, hemostasis during the operative procedure, and minimal postoperative pain and edema.- - - - - - - - - - ranking = 40.980916714636keywords = xanthoma disseminatum, disseminatum, xanthoma (Clic here for more details about this article) |
4/184. A sporadic case of progressive mucinous histiocytosis.Hereditary progressive mucinous histiocytosis is a rare autosomal dominant non-Langerhans cell histiocytosis. We describe a sporadic case of this syndrome in a 64-year-old woman who had multiple dark-red dome-shaped papulonodules located mainly on the back of her hands, forearms and thighs. light microscopy revealed a circumscribed upper dermal aggregate of ovoid or spindle-shaped histiocytes with abundant mucin deposition. iron deposits and numerous mast cells were scattered throughout the tumour but giant cells were rare. Electron microscopy revealed a high number of zebra bodies and myeloid bodies in the cytoplasm of the histiocytes. immunohistochemistry showed positive labelling with alpha-1 antitrypsin, factor xiiia and CD68, while CD1a, CD34 and S100 protein were negative. The differential diagnosis of histiocytic syndromes is discussed.- - - - - - - - - - ranking = 1.4529600695615keywords = histiocytosis (Clic here for more details about this article) |
5/184. pseudoxanthoma elasticum and calcinosis cutis.A 42-year-old white woman presented with clinical and histologic manifestations of both calcinosis cutis and pseudoxanthoma elasticum: discrete milia-like calcifications at the anterior aspect of the neck, a funduscopic examination with classic eye findings, peripheral vascular disease, and a mottled appearance of the skin at the axillae, groin, and lateral aspects of the neck. A younger sibling had similar skin lesions and deteriorating visual acuity. The patient was normocalcemic and normophosphatemic. This case may represent the coincidental occurrence of two rare entities in the same person or may be suggestive of a pattern of dystrophic calcification associated with pseudoxanthoma elasticum.- - - - - - - - - - ranking = 0.85714285714286keywords = xanthoma (Clic here for more details about this article) |
6/184. Benign cephalic histiocytosis with diabetes insipidus.Benign cephalic histiocytosis is a rare skin condition consisting of small tan papules on the face and upper trunk that is believed not to be associated with internal organ involvement. The infiltrating histiocytes are not Langerhans' cells (LCs). We report a 5-year-old girl who presented with diabetes insipidus 1 year after developing multiple small brown asymptomatic skin papules. Histologic examination revealed a non-LC histiocytic proliferation in the dermis without epidermal invasion. She had infiltration of the pituitary stalk on brain imaging. diabetes insipidus has heretofore been associated with LC histiocytosis and xanthoma disseminatum but not benign cephalic histiocytosis.- - - - - - - - - - ranking = 9.8913034240823keywords = xanthoma disseminatum, disseminatum, histiocytosis, xanthoma (Clic here for more details about this article) |
7/184. scalp pain and hyperlipidaemia.Acute pancreatitis and eruptive xanthomata are the only recognised direct complications of severe hypertriglyceridaemia, although peripheral neuropathy has been described in patients with hyperlipidaemia. We describe a patient with mixed hyperlipidaemia presenting with severe scalp pain and eruptive xanthomata. Both resolved with treatment. We suspect that high triglyceride concentration can affect the function of sensory nerve fibres.- - - - - - - - - - ranking = 0.28571428571429keywords = xanthoma (Clic here for more details about this article) |
8/184. Juvenile chronic myelogenous leukemia and dermal histiocytosis. In Von Recklinghausen's disease.A 4-year-old boy with multiple cafe-au-lait spots and a family history of neurofibromatosis had generalized edema, histiocytic rash characterized by benign histiocytic infiltration, hepatomegaly, and life-threatening infection. Six months later, progessive splenomegaly led to juvenile chronic myelocytic leukemia that eventually proved fata. The case represents an important association of diseases.- - - - - - - - - - ranking = 0.96864004637432keywords = histiocytosis (Clic here for more details about this article) |
9/184. Multicentric reticulohistiocytosis with neurofibroma-like nodules.We describe a patient with multicentric reticulohistiocytosis (MR), who presented with 2 different clinical types of lesions. Some were characteristic for MR in that they were deeply set, small, firm papules. Many others were larger, soft, and even sessile, like neurofibromas. Histopathologic features of both types of lesions revealed MR. To our knowledge, this is the first report of lesions of MR mimicking neurofibromas.- - - - - - - - - - ranking = 1.2108000579679keywords = histiocytosis (Clic here for more details about this article) |
10/184. pseudoxanthoma elasticum: significance of limited phenotypic expression in parents of affected offspring.The inheritance pattern of pseudoxanthoma elasticum (PXE) is controversial. inheritance patterns are confounded by delayed diagnosis and mild or limited phenotypic expression among certain family members. Because testing for the genetic mutation(s) responsible for PXE is not routine, genetic counseling must be done with caution. We describe 4 families in which one or more children were diagnosed with PXE. Detailed examination of the parents was carried out, including skin biopsy and ophthalmologic examination. In 3 of the 4 families, one parent had limited phenotypic expression, such as ocular findings without skin lesions or very mild skin lesions with no ocular findings. In the other family, one parent had very mild skin and ocular disease. All 4 affected parents had diagnostic skin biopsy findings. In none of the 4 families was the inheritance pattern clear-cut. Although the inheritance pattern of PXE has been debated, clinically significant stigmata of PXE, which are not always readily apparent, can occur in successive generations. Therefore all first-degree relatives of affected patients should receive a full dermatologic examination as well as a funduscopic examination. If even mild typical skin or eye findings are present, then skin biopsy should be performed.- - - - - - - - - - ranking = 0.71428571428571keywords = xanthoma (Clic here for more details about this article) |
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