Urticaria pigmentosa

BACKGROUND: Mastocytosis is characterized by clonal proliferation of mast cells in various organs and can have isolated cutaneous or systemic involvement. Childhood-onset mastocytosis (COM) is usually cutaneous and regresses spontaneously, while adult-onset mastocytosis (AOM) is often persistent with systemic involvement. There is limited data on COM from India. OBJECTIVE: To elucidate the clinicopathological profile of COM. METHODS: We conducted a retrospective chart review of all the patients with histologically proven COM (≤16 years), presenting over 11 years (January 2009 to December 2019) to the Dermatology Department...

Indolent systemic mastocytosis (ISM) is a group of heterogenous diseases characterized by abnormal accumulation of mast cells in at least one organ. ISM can be a cause of osteoporosis. The aim of this study is to determine the prevalence, and the prognosis of ISM in a cohort of patients with osteoporosis. In this monocentric and retrospective study, patients with osteoporosis who did not receive a bone biopsy (cohort 1) and patients that subsequently received a diagnostic bone biopsy for differential diagnosis (cohort 2) are compared with patients who are diagnosed with ISM (cohort 3)...

INTRODUCTION: Mastocytosis is a rare and heterogenous disease, and in children it is generally limited to the skin and tends to regress spontaneously in adolescence. AIM: In this study, demographic, clinical, and laboratory characteristics of pediatric patients with mastocytosis, and also coexisting diseases were investigated. RESULTS: A total of 61 pediatric patients were included in the study. The male-to-female ratio was 2.2, the median age was 2 years (range, 0...

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Mastocytosis (M) represents a systemic pathology characterized by increased accumulation and clonal proliferation of mast cells in the skin and/or different organs. Broadly, M is classified into two categories: Cutaneous mastocytosis (CM) and systemic mastocytosis (SM). In children, CM is the most frequent form. Unfortunately, pathogenesis is still unclear. It is thought that genetic factors are involved, but further studies are necessary. As for features of CM, the lesions differ in clinical forms. The most important fact is evaluating a pediatric patient with CM...

Mastocytosis is a heterogeneous disease of bone marrow origin, characterized by local or diffuse increased growth and accumulation of clonal mast cells in the skin and/or in internal organs. The skin is the organ most frequently involved, but others may be affected as well. The purpose of this article is to present the oral management of a child with urticaria pigmentosa/maculopapulous cutaneous mastocytosis requiring dental treatment under general anesthesia. The multidisciplinary team approach involving the relevant medical specialists is discussed to emphasize the significance of coordinated patient management...

Systemic mastocytosis (SM) is a heterogeneous disease of the bone marrow, which is characterized by the abnormal proliferation and infiltration of mast cells in one or more organs, such as the skin, bone marrow, digestive tract, liver and spleen. Urticaria pigmentosa is a typical but infrequent manifestation of SM. Other clinical presentations are non-specific, varying from pruritus and hypotension to multiple organ dysfunction, which may be lethal when hemodynamic changes occur, such as the sharp decline in blood pressure observed in the present case...

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Immunization compliance in the United States is declining, in part due to misinformation and fear surrounding adverse vaccination reactions. Recently, there have been data published in the allergy and immunology literature to show that there may be a relationship between routine vaccinations and induction of symptoms in cutaneous mastocytosis patients; however, this has not yet been explored in the dermatology literature. We sought to uncover the prevalence of vaccine reactions due to mast cell activation within our cohort of maculopapular cutaneous mastocytosis (MPCM) patients in order to contribute to ensuring administration safety, managing familial expectations, and encouraging continued adherence...

Urticaria pigmentosa (UP), the most common variant of cutaneous mastocytosis (CM), is characterized by hyperpigmented, brownish macules and patches, with positive Darier's sign. Diffuse cutaneous mastocytosis (DCM), is a rare variant of CM, characterized by diffuse infiltration of the skin by mast cells (MCs), resulting in a typical grain-leather appearance. Blistering is commonly seen in infants with CM but it is not specific for any variant of CM. Herein, we report a case of a 5-month-old male infant who presented with recurrent bullous lesions and hyperpigmented, brownish patches with intervening normal skin suggestive of UP, as well as a single, localized plaque with grain-leather appearance, thereby exhibiting an interesting dual morphology...

Mastocytosis is an accumulation of clonal mast cells within tissues and it is most commonly caused by an activating mutation in the KIT gene. In this study, we report a neonatal case who presented with diffuse cutaneous mastocytosis (CM) at birth. In China, nine other cases of neonatal-onset CM have been reported in the literature since 2006. In those cases, diffuse CM and urticaria pigmentosa were the main symptoms, and mutations in exon 17 at codon 816 in KIT were identified.

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Cutaneous mastocytosis is characterized by the abnormal accumulation of mast cells in the skin. However, mast cell counting is not always easy and reproducible with classical methods. This work aims to demonstrate the reliability, usability, and virtues of a new software used on digital tablets for counting mast cells in cutaneous specific lesions of mastocytosis, to assess differences in mast cell counts between clinical subtypes of mastocytosis in the skin, and to consider the feasibility of applying a diagnostic mast cell count cutoff to urticaria pigmentosa, which is the most frequent form of cutaneous mastocytosis...

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Urticaria pigmentosa is the most common form of mastocytosis that often develops in infancy or early childhood. We report two male children- first, a 7-month-old child with a history of asymptomatic multiple dark colored skin lesions macules with wheals on gentle rubbing (Darier's sign) and second, a 2-year-old child with similar clinical presentation without Darier's sign. Dermoscopy showed dark brown lines in a reticulate pattern which is an exaggeration of the pigment network seen in the normal skin. The reticulate pigment network was darker and thicker in the child with positive Darier's sign...

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Urticaria, also known as hives among people, is a very common disease characterized by erythematous, edematous, itchy, and transient plaques that involve skin and mucous membranes. It is classified as acute spontaneous urticaria, chronic spontaneous urticaria, chronic inducible urticaria, and episodic chronic urticaria. Many factors such as infections, medicines, food, psychogenic factors, and respiratory allergens are accused of etiology, but sometimes, it is idiopathic. Clinical presentation involves red, swelling, and itchy plaques...

Mastocytosis is a disease characterized by abnormal and pathologic increase in mast cells in the cutaneous tissue and extracutaneous organs such as the bone marrow, liver, spleen, lymph node and gastrointestinal tract. Cutaneous mastocytosis comprises of four major clinical variants: solitary and multiple mastocytomas, urticaria pigmentosa, diffuse cutaneous mastocytosis and telangiectasia macularis eruptiva perstans. Cutaneous mastocytosis of the xanthelasmoid type is a rare variant of diffuse mastocytosis...

Case summary: A 14-month-old castrated male domestic shorthair cat presented with an 8 month history of severe pruritus, alopecia, papules and excoriations. Initial evaluation and treatment prior to referral included skin scrape, cytology, two strict food trials, dermatophyte culture, and bacterial culture and sensitivity, as well as antibiotic therapy, empiric treatment for mites, steroids and ciclosporin A (Atopica; Elanco). The cat was referred to the Dermatology and Otology Clinic at the University of Illinois Veterinary Teaching Hospital for further diagnostics and treatment...

Gastrointestinal stromal tumours (GISTs) are mesenchymal tumours arising in the gastrointestinal tract. Early detection, before metastasis occurs, is important as complete surgical excision achieves cure. Approximately 85% of GISTs are associated with mutations in the KIT gene, and although the majority of GISTs are sporadic, familial GISTs have been identified. Several families with multiple GIST tumours have also been described with various cutaneous findings including hyperpigmentation, multiple lentigines, vitiligo and urticaria pigmentosa...