Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Topical corticosteroids are utilized in the treatment of a wide variety of skin diseases, primarily those involving an inflammatory component. Recent investigations have revealed that one of the effects of long-term usage of steroids is the depletion of skin mast cells. This led to the treatment of patients with urticaria pigmentosa with topical high potency corticosteroids for 6 weeks. At the end of treatment there was a marked reduction in tissue histamine and an absence of mast cells as well as a disappearance of pruritus and Darier's sign. Treated areas remained clinically improved for at least 9-12 months. Observations that corticosteroids profoundly affect mast cells in vivo provides a rationale to devise new treatment regimens for mast-cell-related diseases.
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PMID:Aggressive topical corticosteroid therapy: a novel approach to mast-cell-dependent cutaneous disorders. 367 48

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.
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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.
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PMID:Cutaneous mastocytosis in Singapore. 815 91

We report a case of solitary mastocytoma in a child which was successfully treated with a topical steroid of moderate potency. The patient was an 18-month-old girl who presented with localized oval shape yellowish to hyperpigmented lesion on the medial aspect of her right forearm noticed accidentally by the parents since the age of 6 months. The lesion was observed to get urticated mainly after bathing, toweling, and scratching of the area, associated with reddening and itching confined to the lesion (Figure 1). No other area of the body was affected with any similar lesion. Examination of the skin revealed a yellow-tan oval shape patch 1 x 3 cm in diameter which was firm to the touch with intact overlying skin. The lesion became swollen and itchy when it was rubbed vigorously (positive Darier's sign). Systemic examination was unremarkable. The patient investigations including complete blood count, routine biochemical data, plasma histamine level, and urinalysis were within normal levels. Skin biopsy was cancelled because the parents refused, so our clinical diagnosis was solitary mastocytoma even though it was not confirmed histologically. We started the patient on a moderate potency corticosteroid (betamethasone valerate 0.1% cream) twice a day for six weeks after which the lesion became softer with a weak Darier's sign. This treatment was continued for another four months which led to resolution of the lesion with residual hyperpigmentation, negative Darier's sign, and no signs of atrophy (Figure 2). Follow up of the patient for another 8 weeks without treatment did not reveal any recurrence of the lesion.
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PMID:Solitary mastocytoma successfully treated with a moderate potency topical steroid. 1517 67

Cutaneous mastocytosis (CM) or urticaria pigmentosa is characterized by abnormal proliferation and accumulation of mast cells. Clinically, CM usually presents as symmetrically distributed red-brown macules or papules that develop weals, erythema and often pruritus on stroking (Darier's sign). The histological hallmark of the disease is an increase in oval to spindle-shaped mast cells in the dermis located around blood vessels and skin appendages. We describe three patients with a new clinicopathological type of CM, which clinically mimics a histiocytic disorder and histologically mimics leucocytoclastic vasculitis (LV). Three infants (two boys and one girl) developed generalized reddish-yellow-brown macules of 3-10 cm with occasional scaling and crusting on the trunk and extremities without further symptoms or organ involvement except variable itching. Histology revealed diffuse and dense dermal infiltrates of eosinophils, neutrophils and nuclear debris with perivascular accentuation, imitating LV. This infiltrate masked large epithelioid cells, positive for macrophage markers, which by special histochemical stains for metachromatic granules turned out to be mast cells. This is the first report of this new variant of CM, which may cause considerable diagnostic difficulties both clinically and histopathologically.
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PMID:A new variant of mastocytosis: report of three cases clinicopathologically mimicking histiocytic and vasculitic disorders. 1612 Jan 57

A 41-year-old woman presented with a 2-month history of pruritus and a generalized dermatitis that developed initially on the head and spread to the trunk, legs, and buttocks. The pruritus caused extreme discomfort and was not relieved by antihistamines or topical steroid treatment. The patient denied flushing, syncope, and vomiting. Her medical history included asthma treated with salmeterol/fluticasone propionate inhaler, and status post silicone breast augmentation. Physical examination revealed a papular dermatitis on the trunk and extremities composed of lesions up to 0.5 cm in diameter, surrounded by excoriation marks (Figure 1). There was no hepatosplenomegaly or lymphadenopathy. Darier's sign was negative. Results of complete blood count, peripheral blood film examination, and liver function tests were all with normal limits. A biopsy specimen taken from a lesion and stained with hematoxylin-eosin showed telangiectasias, with an increased number of mast cells around blood vessels (Figure 2). Positive Giemsa (Figure 3) and c-kit stain (Figure 4) indicated an increased number of mast cells. Bone marrow aspiration and total body CT performed to rule out systemic involvement showed no pathology. Protein electrophoresis was normal. Serum tryptase and histamine were within normal limits, and 24-hour urine collection for histamine was normal. Narrow-band UV-B treatment was begun 3 times weekly, reduced to twice weekly after 2 months, and then stopped. The first few treatments resulted in significant relief of the pruritus and regression of lesions. After 3 months without treatment, the patient remained free of pruritus and lesions.
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PMID:Telangiectasia macularis eruptiva perstans: unusual presentation and treatment. 1708

The term mastocytosis is referred to as an array of uncommon, usually sporadic, heterogeneous clinical illnesses that result from the hyperplasia of tissue mast cells. It comprises many different clinical manifestations varying from indolent cutaneous forms to systemic and malignant conditions. The characteristic presentation of mastocytosis consists of cutaneous manifestations: either a solitary mastocytoma, urticaria pigmentosa, or less commonly, diffuse cutaneous mastocytosis. Urticaria pigmentosa is the most common manifestation of cutaneous mastocytosis that manifests as a generalized eruption of round or oval erythematous macules, papules and plaques with variable amounts of brown pigment, usually on the trunk, but may also occur in all regions of the body including face and mucous membranes. Pruritus, dermographism and Darier's sign are additional features of these eruptions. Mastocytosis may also be manifested as mastocytoma, a rare, benign, pediatric tumor that results from hyperplasia of mast cells in papillary dermis in the first few weeks of life. The clinical course of mastocytosis is variable. The prognosis for the majority of pediatric patients with urticaria pigmentosa is extremely good, and over half of cases clear completely by adolescence, while those with aggressive systemic mastocytosis or mast cell leukemia show a progressive course, usually with a fatal outcome.
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PMID:Most common clinical presentations of cutaneous mastocytosis. 1962 75

A 24-year old female developed late onset solitary mastocytoma on the left forearm. She complained of intense pruritus off and on which was not associated with flushing of face and blister formation over the nodule. Darier's sign was positive. Excisional biopsy was done and histopathology confirmed the diagnosis.
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PMID:Solitary mastocytoma in an adult. 2094 63

A 10-month-old boy presented with a history of a generalized cutaneous bullous eruption since 3 months of age. Emesis, flush, pruritus, and fatigue had accompanied relapsing episodes of sometimes extensive blistering. Histopathology showed dense dermal infiltrates of mast cells on hematoxylin and eosin and corroborating immunohistochemical staining. Laboratory examination revealed a markedly high level of serum tryptase. Based on these results and after consecutive staging, the patient was diagnosed with diffuse cutaneous bullous mastocytosis (BM). Mutation analysis detected a deletion mutation (del419) in C-Kit by direct exon sequencing. This rare entity must be considered in the differential diagnosis whenever a child presents with bullae and erosions. A crucial diagnostic hint is that rubbing of affected skin areas results in whealing (Darier's sign). A comprehensive diagnostic approach, advanced therapeutic strategies, regular follow-ups, and instruction of patients and relatives on prevention and prophylaxis are highly indicated.
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PMID:Diffuse cutaneous mastocytosis masquerading as epidermolysis bullosa. 2185 15

A few case reports have only been published to date, which show the possible link between mastocytosis and gastroesophageal reflux disease (GERD). Here, we present a 17-month-old boy with urticaria pigmentosa who also suffered from GERD. First skin lesions accompanied by gastrointestinal symptoms (regurgitations, appetite worsening) were noticed at the age of four months. At that time, they were misdiagnosed as symptoms of cow's milk allergy. Despite the cow's milk-free diet, the skin and gastrointestinal symptoms slowly worsened and the patient was referred to us for further diagnostics several months later. Based on the clinical presentation of skin lesions, presence of pruritus and positive Darier's sign, cutaneous mastocytosis was diagnosed; 24-hour pH-metry revealed an increased number of acidic refluxes and biopsy of duodenal mucosa showed numerous mast cells. Taking into account these findings, the gastrointestinal symptoms were supposed to be a manifestation of mastocytosis. Treatment with sodium cromoglycate, cetirizine, ranitidine and probiotics resulted in pruritus alleviation, improvement of appetite and sleeping as well as increase of body weight.
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PMID:Gastroesophageal reflux disease accompanying urticaria pigmentosa. 2218 27


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