Urticaria Pigmentosa

The most common form of cutaneous mastocytosis (MASTOCYTOSIS, CUTANEOUS) that occurs primarily in children. It is characterized by the multiple small reddish-brown pigmented pruritic macules and papules.

Also Known As:

Networked: 180 relevant articles (8 outcomes, 6 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Atopic Dermatitis (Atopic Eczema)
2.	Skin Diseases (Skin Disease)
3.	Granuloma Annulare
4.	Systemic Mastocytosis
5.	Pruritus (Itching)

Experts

1.	Johansen, Maija L: 2 articles (03/2021 - 01/2021)
2.	Lawley, Leslie P: 2 articles (03/2021 - 01/2021)
3.	Carter, Melody C: 2 articles (01/2021 - 12/2015)
4.	Butterfield, Joseph H: 2 articles (08/2012 - 02/2004)
5.	Schwartz, L B: 2 articles (06/2012 - 08/2000)
6.	Bessis, Didier: 2 articles (01/2004 - 07/2003)
7.	Guillot, Bernard: 2 articles (01/2004 - 07/2003)
8.	Basarab, Marina: 1 article (05/2022)
9.	Evans, Terry John: 1 article (05/2022)
10.	Fernando, Samitha: 1 article (05/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Urticaria Pigmentosa:

1.	Adrenal Cortex Hormones (Corticosteroids)IBA 09/01/1994 - "A case of adult-onset urticaria pigmentosa is presented, in which the eruption cleared following the application of potent topical corticosteroids. " 01/01/1987 - "This led to the treatment of patients with urticaria pigmentosa with topical high potency corticosteroids for 6 weeks. " 12/01/1985 - "Treatment of urticaria pigmentosa with corticosteroids." 02/01/1991 - "Nine patients with adult-onset urticaria pigmentosa were studied for the incidence of extracutaneous mast cell involvement and the efficacy of potent topical corticosteroid therapy for cutaneous lesions. " 11/01/1975 - "A patient had systemic mastocytosis of 16 years' duration, with urticaria pigmentosa, osteopetrosis with severe bone pain, mastocytosis of the bone marrow with myeloid metaplasia, gastrointestinal malabsorption, and Cushingold features associated with prolonged usage of corticosteroids. "
2.	Histamine (Histamine Dihydrochloride)FDA Link 01/01/1981 - "These results suggest that this effective new treatment for urticaria pigmentosa reduces the histamine turnover in the skin by inhibiting mast cell proliferation." 05/01/1986 - "The possibility exists that several mediators and mechanisms superimpose during the early histamine-mediated phase of the vascular response in urticaria pigmentosa following activation of mast cells." 05/01/1986 - "Ultrastructure of the immediate, histamine-mediated microvascular response in human skin with urticaria pigmentosa." 07/01/1983 - "The beneficial effect of PUVA in urticaria pigmentosa therefore does not appear to be directly related to a change in mast cell numbers or morphology, or to the histamine concentration in the skin." 01/01/1981 - "Decreased urinary histamine metabolite after successful PUVA treatment of urticaria pigmentosa."
3.	SteroidsIBA 09/01/1994 - "Urticaria pigmentosa--response to topical steroids." 12/01/1985 - "Based on a previous observation that the long-term application of potent topical corticosteroids under occlusion to normal skin resulted in the loss of mast cells, we investigated the effects of intralesional and topical steroids in urticaria pigmentosa (UP). " 11/01/1997 - "Prolonged remission of urticaria pigmentosa following topical steroid therapy under hydrocolloid occlusion." 01/01/1996 - "Twenty-three patients with chronic idiopathic urticaria and 3 patients with urticaria pigmentosa received a 2-week treatment with daily application of potent topical steroid using plastic occlusion. "
4.	CladribineFDA LinkGeneric 02/01/2013 - "Response of urticaria pigmentosa to cladribine in a patient with systemic mastocytosis." 03/01/2015 - "Sustained improvement in urticaria pigmentosa and pruritus in a case of indolent systemic mastocytosis treated with cladribine."
5.	Colloids (Colloid)IBA 11/01/1997 - "Prolonged remission of urticaria pigmentosa following topical steroid therapy under hydrocolloid occlusion."
6.	AntibodiesIBA 01/01/2013 - "Using the antibodies 2D7, BB1, and c-Kit, we performed an immunohistochemical study of skin biopsy specimens from patients with cutaneous mastocytosis (15 urticaria pigmentosa and telangiectatic macularis eruptive perstans) and liver or bone marrow biopsy specimens from patients with systemic mastocytosis. " 01/01/1985 - "Urticaria pigmentosa, steatorrhea, eosinophilia, absence of antireticulin antibodies, and submucosal nodularity seen on radiographic study of the duodenum were the clinical characteristics. " 11/01/1990 - "Specific IgE antibodies to common inhalants were also observed less frequently in urticaria pigmentosa patients than in controls, although this difference was not statistically significant. " 11/01/1990 - "Thirty-three patients with histologically verified urticaria pigmentosa were studied for coexisting atopic disease by means of history, skin prick testing with five common inhalants and serological investigation for total IgE and specific IgE antibodies to five common inhalants. " 01/01/1997 - "These findings suggest that (1) anti-C5a receptor antibodies directed to the N-terminal domain of the receptor are suitable tools for the identification of mast cells in acrylate-embedded sections of human skin, (2) mast cell activation in weal and flare reactions results in C5a receptor downregulation or receptor blockade and (3) mast cells in urticaria pigmentosa lack a typical marker of normal human skin mast cells."
7.	Monoclonal AntibodiesIBA 10/01/1995 - "In order to identify more specific or selective mast cell markers, the reactivity of two monoclonal antibodies, Ki-MC1 and Ki-M1P, was studied by immunohistochemistry in two human cell lines (mast cell line HMC-1, basophilic cell line KU812), in mast cells cultured from blood precursors, in adherent mononuclear cells from peripheral blood, and in mast cells of tissue sections from 13 urticaria pigmentosa lesions, five mastocytomas and five normal skin specimens. " 01/01/1995 - "We utilized a monoclonal antibody to human MGF to further define patterns of immunoreactivity in frozen specimens of neonatal and adult skin from normal individuals and from patients with urticaria pigmentosa. " 06/01/1990 - "Skin mast cells of five patients with mastocytosis/urticaria pigmentosa and from four normal controls were immunophenotyped with a panel of 17 monoclonal antibodies; peripheral blood mononuclear cells from the patients were similarly labelled and analysed by flow cytometry. " 01/01/1990 - "Biopsy specimens from lesional and uninvolved skin of nine patients with delayed pressure urticaria, three patients with acute urticaria, six patients with chronic recurrent urticaria, and four patients with urticaria pigmentosa were analyzed by routine histology and by immunochemistry for their reactivity with monoclonal antibodies to three different subsets of macrophages. " 07/01/1993 - "We have used the monoclonal antibody PC10 to study MCs in reactive or hyperplastic states (chronic non-specific lymphadenitis, n = 10; benign and malignant solid tumours, n = 5) and in the various subtypes of mastocytosis (urticaria pigmentosa, n = 22; solitary mastocytoma of the skin, n = 7; systemic mastocytosis; n = 8; malignant mastocytosis, n = 4). "
8.	Substance PIBA 01/01/1998 - "The aim of our study was to evaluate the sensitivity of skin mast cells from urticaria pigmentosa (UP) patients to substance P (SP), tumor necrosis factor alpha (TNF-alpha) and anti-IgE, and to compare the sensitivity of these cells with that of skin mast cells from healthy human donors. " 01/01/1993 - "Patients with urticaria pigmentosa were investigated during symptom-free interval regarding plasma concentrations and urinary excretion of immunoreactive regulatory peptides: calcitonin gene-related peptide (CGRP), gastrin, neurokinin A (NKA), neuropeptide Y (NPY), somatostatin (SOM), substance P (SP) and vasoactive intestinal peptide (VIP). "
9.	Histidine (L-Histidine)FDA Link 04/01/1961 - "Histidine metabolism in urticaria pigmentosa." 12/01/1961 - "Further studies of histidine and histamine metabolism in urticaria pigmentosa."
10.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 01/01/1998 - "The aim of our study was to evaluate the sensitivity of skin mast cells from urticaria pigmentosa (UP) patients to substance P (SP), tumor necrosis factor alpha (TNF-alpha) and anti-IgE, and to compare the sensitivity of these cells with that of skin mast cells from healthy human donors. "

Therapies and Procedures

1.	Therapeutics 10/01/2003 - "Medium- versus high-dose ultraviolet A1 therapy for urticaria pigmentosa: a pilot study." 07/01/2005 - "Ultraviolet-based therapy has been used to treat various pruritic conditions including pruritus in chronic renal failure, atopic dermatitis, HIV, aquagenic pruritus and urticaria, solar, chronic, and idiopathic urticaria, urticaria pigmentosa, polycythemia vera, pruritic folliculitis of pregnancy, breast carcinoma skin infiltration, Hodgkin's lymphoma, chronic liver disease, and acquired perforating dermatosis, among others. " 02/01/1991 - "Nine patients with adult-onset urticaria pigmentosa were studied for the incidence of extracutaneous mast cell involvement and the efficacy of potent topical corticosteroid therapy for cutaneous lesions. " 09/01/1990 - "Administration of 8-methoxypsoralen followed by ultraviolet A irradiation (PUVA treatment) has been used as a therapy for urticaria pigmentosa. " 10/01/1984 - "A patient with symptomatic urticaria pigmentosa who responded to nifedipine therapy is reported. "
2.	Phototherapy (Light Therapy) 12/01/2000 - "UVA1 phototherapy (340-400 nm) is effective in the treatment of inflammatory skin diseases such as acutely exacerbated atopic dermatitis, localized scleroderma, urticaria pigmentosa and disseminated granuloma annulare. " 07/01/2013 - "Further indications for UVA1 include subacute prurigo, lichen sclerosus, dyshidrotic dermatitis, cutaneous T cell lymphoma, urticaria pigmentosa, and pityriasis rosea; nevertheless, there are some unknowns, uncertainties, and controversies concerning short- and long-term side effects, efficacy and dosage regimens of UVA1 phototherapy in some conditions. " 02/01/2008 - "Investigation of the efficacy of ultraviolet (UV) A1 phototherapy on atopic eczema, scleroderma, granuloma annulare, urticaria pigmentosa, prurigo nodularis, lichen sclerosus et atrophicus, T-cell lymphoma, keratosis lichenoides chronica, chronic urticaria and some rare, sclerosing skin diseases. " 01/01/1985 - "Ten patients with moderate to very severe urticaria pigmentosa were studied for the therapeutic effect of photochemotherapy (PUVA; six adults) and selective ultraviolet phototherapy (SUP; four adolescents). " 01/01/1985 - "Phototherapy of urticaria pigmentosa: clinical response and changes of cutaneous reactivity, histamine and chemotactic leukotrienes."
3.	Solid-State Lasers 03/01/2000 - "We describe a 30-year-old woman with urticaria pigmentosa in whom treatment with the Nd:YAG laser produced a significant improvement in the clinical appearance of the eruption, with some recurrence after 9 months. " 06/01/2005 - "The purpose was to investigate the use of a diode-pumped Nd:YAG laser at 532 nm for the treatment of the cosmetic comorbidity of urticaria pigmentosa lesions. " 06/01/2005 - "The cosmetic treatment of urticaria pigmentosa with Nd:YAG laser at 532 nanometers." 03/01/2000 - "The treatment of urticaria pigmentosa with the frequency-doubled Q-switch Nd:YAG laser."
4.	PUVA Therapy 10/01/2005 - "A 64-year-old man was diagnosed as having urticaria pigmentosa in 1998, and treated with PUVA therapy. " 03/01/1980 - "This suggests that PUVA-therapy may exercise its beneficial effect in urticaria pigmentosa by direct destruction of these cells. " 08/01/2001 - "After 18 months of intensive UVA1- and bath PUVA therapy for urticaria pigmentosa, a female patient developed a malignant melanoma on the right thigh. " 01/01/1997 - "Short- and long-term effectiveness of oral and bath PUVA therapy in urticaria pigmentosa and systemic mastocytosis." 01/01/1981 - "The effect of PUVA therapy on pruritus, the skin mast cell population and histamine metabolism has been studied in 3 patients with urticaria pigmentosa and manifestations of systemic mastocytosis. "
5.	Photochemotherapy (Photodynamic Therapy) 07/01/1983 - "Photochemotherapy (PUVA) in the treatment of urticaria pigmentosa." 09/01/1984 - "In six patients with urticaria pigmentosa, population density and ultrastructure of the cutaneous mast cells and histamine levels of the lesional skin were studied before, immediately after, and again 5-8 months after photochemotherapy (PUVA). " 01/01/1985 - "Ten patients with moderate to very severe urticaria pigmentosa were studied for the therapeutic effect of photochemotherapy (PUVA; six adults) and selective ultraviolet phototherapy (SUP; four adolescents). "