Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 21 cases of urticaria pigmentosa (UP), clinical and histological observations and evaluation of mast cell (MC) volume density in the lesions using a morphometric point counting method were performed. The mutual correlations between clinical and histological findings were statistically assessed by a method of multiple regression analysis. Clinical items employed in the analyses were as follows: sex, the age of onset, the age of biopsy, the biopsy, the duration of lesions, the type of skin lesions, sites involved, the presence or absence of Darier's sign of Darier's sign and symptoms, and serum histamine level. Histological items included the localization and infiltration pattern of MC, the level of basal melanosis, the presence or absence of inflammatory cell infiltration, and the MC volume density in the lesions. Statistical significance of the partial regression coefficients was obtained for 6 pairs of the criteria (p = 0.05), including the age of onset and the age of biopsy, the age of onset and the level of basal melanosis, the duration of lesions and the level of basal melanosis, and the type of skin lesions and the level of basal melanosis. No significant correlations were observed between the MC volume density in the lesions and any of the other items. These results suggest that the basal melanosis in a UP lesion may not be a direct reaction to the transitory massive infiltration of MC, but rather be due to a relatively long-term effect of MC infiltration. Furthermore, the MC volume density in the lesion is not likely to be an important factor in determining the clinical manifestations of a UP lesion.
 ...
PMID:A clinical and histological study of urticaria pigmentosa: relationships between mast cell proliferation and the clinical and histological manifestations. 238 37

Histiocytosis X and mastocytosis are proliferative processes that may have similar cutaneous manifestations. However, a positive Darier's sign (urtication on stroking of the lesion) is thought to reliably distinguish between these two diseases. We recently studied a 13-year-old girl with a 2-year history of extensive skin lesions and a positive Darier's sign. Routine histopathologic studies revealed a polymorphous cutaneous infiltrate composed of histiocytes, mast cells, eosinophils, and lymphoid cells. Electron microscopic studies demonstrated Langerhans granules in some of the histiocytes, and immunologic studies of frozen tissue showed that a significant subpopulation of the histiocytes marked as Langerhans cells. Giemsa staining of specimens from eight other cases of cutaneous histiocytosis X from our files revealed mast cells in all of the lesions, although none showed the abundance of mast cells present in the case with urtication. Our studies emphasize the often polymorphous nature of the cell population in cutaneous histiocytosis X and demonstrate that confusing clinical findings can result when the mast cell population in histiocytosis X produces urtication.
 ...
PMID:Urticating histiocytosis: a mast cell-rich variant of histiocytosis X. 242 66

The authors present three cases of urticaria pigmentosa (UP), one with a very early beginning and two with a late beginning. The clinical symptoms orient the diagnosis of the three cases in relation to maculopapular exanthema of a dark pink color, brown macules and, in all cases, the presence of Darier's sign. As normal, the clinical diagnosis was confirmed by the histopathological examination of the skin. The evolution of the three patients was favorable in all cases, with the disappearance of symptoms in one case. In the other two cases the process is stabilized, and at no time have systemic manifestations been found. The relative rareness of UP in infancy, an expression of a proliferation of the reticuloendothelial system (RES) on a mast cell level with different evolutional possibilities, has been the justifying factor in the presentation of these new cases.
 ...
PMID:[Urticaria pigmentosa]. 319 59

One patient with urticaria pigmentosa was treated with PUVA. This treatment resulted in generalized tanning, decrease of Darier's sign and improvement of pruritus. However, the hyperpigmented macules overlying the mast cell infiltrates showed some persistence. This patient was subjected to biopsy of lesional skin before, during and at the end of oral photochemotherapy. During and after PUVA-therapy, histological examination revealed an increased number of free granules and a progressive decrease in the number of dermal mastocytes. Ultrastructurally necrotic cellular changes in the dermal mastocytes were observed. This suggests that PUVA-therapy may exercise its beneficial effect in urticaria pigmentosa by direct destruction of these cells. In addition, PUVA stimulates mast cell degranulation. Histaminaemia was estimated in this patient, before and after several irradiations. No significant changes were observed. Furthermore, histaminaemia was assayed in 15 patients with various dermatoses undergoing PUVA-therapy. No significant changes were observed. It is concluded that histaminaemia assayed using the fluorometric method of Shore et al. remains unchanged during oral photochemotherapy.
 ...
PMID:[Dermal mastocytes, histaminaemia, and oral photochemotherapy (author's transl)]. 725 77

Mastocytosis is the collective name for a group of clinical syndromes whose signs and symptoms are due to the infiltration of various tissues by mast cells and to the release of chemical mediators by these cells. The skin is the most frequently affected organ. Skin manifestations include urticaria pigmentosa, mastocytoma, diffuse cutaneous mastocytosis and telangiectasia macularis eruptiva perstans. Seven cases of mastocytosis were seen over a 3-year period at the National Skin Centre from 1989 to 1992. All our patients were in the paediatric age group. There were four boys and three girls ranging in age from one year to five years. The mean age of onset of the disease was 2.3 months. Six patients presented with cutaneous signs and symptoms of urticaria pigmentosa and one patient had diffuse cutaneous mastocytosis. Itch was the most prominent symptom seen in all the patients. All the patients had a positive Darier's sign, pathognomonic for mastocytosis. None of the patients had a positive family history. Treatment was conservative and symptomatic, with the use of H1 antihistamines to control itching. A particularly important aspect of management is the avoidance of triggering factors. All our patients have remained well with only skin involvement. The prognosis for children with mast cell disease is good, with at least half of the children with urticaria pigmentosa experiencing reduction of symptoms and lesions by adolescence.
 ...
PMID:Cutaneous mastocytosis in Singapore. 815 91

On the first day of life, a healthy infant was given a recombinant hepatitis B vaccine. Over the following year, a 3 by 4.5 cm, well-defined, erythematous patch with an overlying white, reticulated, smaller plaque gradually appeared on her thigh at the vaccination site. Darier's sign was elicited at the site. Examination of a biopsy specimen showed an upper dermal mast cell infiltrate. This is the first reported case of a solitary mastocytoma appearing in a vaccination site.
 ...
PMID:Solitary mastocytoma arising at a hepatitis B vaccination site. 995 94

A markedly elevated serum level of mast cell tryptase (77.6 microg/L; 95th percentile in normals 13.5 microg/L) was detected in a patient treated for 5 years with wasp venom immunotherapy because of severe anaphylaxis following a wasp sting. Retrospective analysis of stored serum samples taken during the course of immunotherapy revealed that the tryptase level had been elevated for at least 3 years. Despite several dermatological examinations, skin changes of mastocytosis had been missed. Re-examination of the patient revealed sparse macules on the thorax and thighs; Darier's sign was negative. Histologically, mast cell accumulation in these lesions was demonstrable. No signs of systemic mastocytosis were detected. The most appropriate diagnosis was telangiectasia macularis eruptiva perstans. Even in patients with highly elevated tryptase levels, mastocytosis may go undiagnosed. As mastocytosis predisposes to severe anaphylaxis, the condition should be looked for in patients with such reactions by clinical examination and measurement of serum tryptase levels.
 ...
PMID:Mastocytosis associated with severe wasp sting anaphylaxis detected by elevated serum mast cell tryptase levels. 1060 62

Varieties of the clinical features of mastocytoses, also called mastocytosis syndrome, are presented. The disease is characterized by excessive accumulation of mast cells, their proliferation and action in the skin and other organs, even in the central nervous system. The mastocytosis syndrome was known as early as the second half of the 19th century under the term urticaria pigmentosa, and was histologically confirmed by the presence in the dermis of metachromatic cells, i.e. Ehrlich mast cells with red-purple cytoplasmic granules visible with Giemsa or toluidine blue stains. The mastocytosis syndrome was then supposed to be a benign chronic dermatosis of childhood with spontaneous regression by adolescence. The clinically pathognomonic symptoms of Darier's sign (urtication of primary skin lesion upon rubbing) and flushing help in the diagnosis of mastocytosis syndrome. In the 1950s, there was a progression in the diagnosis of systemic mastocytosis achieved by scientists and clinicians of various specialties. Upon the discovery of many mast cell released mediators (heparin, histamine, leukotrienes, prostaglandins, proteases, cytokines), receptor functions, relationship to IgE, anaphylatoxin, etc., they were recognized as triggers of various clinical features of the mastocytosis syndrome. In this paper, different forms of cutaneous and systemic mastocytosis are described, with special reference to 'mastocytosis mucocutanea haemorrhagica' observed by one of the authors in a female infant and followed from 6 months till 2.5 years of age. The patient showed practically all the diverse forms of cutaneous mastocytosis: urticaria pigmentosa, papular, nodular, tumorous-like melanoma, vesiculobullous, erythrodermic, telangiectasia eruptiva maculosa perstans. She also suffered from nasal and rectal hemorrhage, conjunctival suggillations, plaque-like infiltrations of the glossal, oropharyngeal and laryngotracheal mucosa, episodes of flushing, and transitory apnea. It is emphasized that the diagnosis of mastocytosis syndrome may be difficult for its mimicking various other diseases. The occurence of mastocytosis syndrome from the neonatal period through adult and old age, and possibilities of symptomatic treatment and prevention of sudden death or fatalities are discussed. Familial occurrence of mastocytosis syndrome and new genetic studies that may prove highly useful for understanding the etiopathogenesis of mastocytosis syndrome are described.
 ...
PMID:Clinical varieties of mastocytoses. 1150 29

We report the case of solitary mastocytoma, which appeared under the identification bracelet on the left wrist of a 2-day-old newborn infant. Stroking the lesion elicited Darier's sign. Histopathologic examination of a punch biopsy specimen revealed a dense mast cell infiltrate of the dermis, which was confirmed by Giemsa and toluidine blue stains. We believe that this case represents and unusual reactive process.
 ...
PMID:[Solitary mastocytoma associated with newborn infants' identification bracelets]. 1152 2