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Turk Pediatri Arsivi Jun 2015We aimed to retrospectively evaluate histopathological, demographic and clinical findings of children with mastocytosis diagnosed with mastocytosis in our clinic.
AIM
We aimed to retrospectively evaluate histopathological, demographic and clinical findings of children with mastocytosis diagnosed with mastocytosis in our clinic.
MATERIAL AND METHODS
The files of 21 patients diagnosed with mastocytosis between 2000 and 2014 in our clinic were retrospectively analyzed.
RESULTS
All patients had cutaneous mastocytosis, 19 patients had urticaria pigmentosa and 2 patients had mastocytoma. The male-female ratio was: 1/1.6. The median age for onset of disease was 12.1 months and the disease occured in the newborn period in 3 patients. While all patients had eruption, 10 patients had pruritis, 1 patient had a bullous formation, 1 patient had abdominal pain and 1 patient had attacks of redness throughout the body and a sense of burning in the chest. Two patients had a positive familial history. The diagnosis was confirmed with skin biopsy in all patients. The median follow up time of the patients were 5 years. The patients were treated with H1, H2 antihistaminics, local moisturizing creams and topical corticosteroid drugs. The lesions resolved completely in 4 patients who reached to puberty and 7 patients had marked improvement in a 5.5 year-follow-up period. Ten patients had stabile lesions in a 3.6 year-follow-up period.
CONCLUSIONS
Most cases of childhood mastocytosis are observed in the form of cutaneous mastocytosis. The prognosis is good; the disease limits itself and is prone to regress in the adolescent period.
PubMed: 26265895
DOI: 10.5152/tpa.2015.2332 -
Cureus Dec 2023Congenital cutaneous mastocytoma is an uncommon disorder characterized by abnormal proliferation of mast cells. It typically presents as a single, small,...
Congenital cutaneous mastocytoma is an uncommon disorder characterized by abnormal proliferation of mast cells. It typically presents as a single, small, yellowish-brown plaque, and its diagnosis is generally facilitated by distinctive clinical features, including a positive Darrier's sign. This report presents a case of an unusually large, solitary congenital mastocytoma encompassing nearly the entire circumference of the calf, observed in a newborn boy of Bangladeshi origin. Measuring 13x6 cm, the lesion formed large bullae and subsequent erosions. The perplexing clinical appearance prompted a skin biopsy, revealing monomorphic CD117 (c-KIT) positive infiltration without significant cell pleomorphism, confirming the diagnosis of cutaneous mastocytoma. The patient underwent management with potent and very potent topical steroids, oral antihistamines, and non-adhesive dressings, remaining under long-term follow-up with secondary care dermatology. In reporting this case, our objective is to augment the existing scientific literature by providing additional evidence that cutaneous mastocytomas can display a spectrum of clinical presentations, as illustrated in this case.
PubMed: 38205464
DOI: 10.7759/cureus.50306 -
Indian Dermatology Online Journal 2021Mastocytosis is characterized by clonal proliferation of mast cells in various organs and can have isolated cutaneous or systemic involvement. Childhood-onset...
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. We compiled the demographic data, clinical characteristics (morphology, extent, distribution), laboratory investigations, histopathology findings, imaging (ultrasound abdomen), mutation results, where available, and other associated abnormalities, and grouped them according to the WHO classification for mastocytosis.
RESULTS
Among the 66 patients with COM (M: F-1.6:1), 89.4% had onset before 2 years of age. The subtypes were: maculopapular cutaneous mastocytosis (MPCM: 44, 66.7%); mastocytoma of the skin (MOS: 19, 28.8%); diffuse cutaneous mastocytosis (DCM: 2, 3%) and indolent systemic mastocytosis (ISM: 1, 1.5%). Blistering was observed in 29 (43.9%) and Darier sign was elicited in 47 (71.2%) patients. Serum tryptase was elevated in 9/21 (42.9%) patients, but none had systemic mastocytosis. Three patients had mutations (two in exon 8 and one in exon 17). Most patients were managed symptomatically and the patient with ISM improved with imatinib.
CONCLUSION
MPCM is the most common variant of COM and most patients had a disease onset before 2 years. Overall, COM had a good prognosis with rare systemic involvement, mitigating the need for extensive evaluation routinely in children.
PubMed: 34667757
DOI: 10.4103/idoj.IDOJ_924_20 -
Dermatology Online Journal Sep 2010Cutaneous Mastocytosis is not an uncommon condition in the pediatric setting. The eruption can have multiple clinical presentations. We present a case of a 3-month-old...
Cutaneous Mastocytosis is not an uncommon condition in the pediatric setting. The eruption can have multiple clinical presentations. We present a case of a 3-month-old child with a solitary mastocytoma who was initially diagnosed with recurrent bullous impetigo. Solitary mastocytoma can present as a blister. Although bullous impetigo is a common diagnosis in children and it would be tempting to make that diagnosis in the presence of a positive skin swab culture, clinicians always have to be mindful of secondary impetiginization of another primary skin disease process.
Topics: Blister; Diagnosis, Differential; Erythema; Female; Humans; Impetigo; Infant; Leg; Mastocytoma, Skin
PubMed: 20875331
DOI: No ID Found -
Experimental Hematology Oct 2011The therapeutic potential of allogeneic hematopoietic stem cell transplantation relies on the graft-vs.-leukemia (GVL) effect to eradicate residual tumor cells... (Comparative Study)
Comparative Study
OBJECTIVE
The therapeutic potential of allogeneic hematopoietic stem cell transplantation relies on the graft-vs.-leukemia (GVL) effect to eradicate residual tumor cells immunologically. The relationship of conditioning intensity to GVL effect was investigated.
MATERIALS AND METHODS
Lethally irradiated (either 900 or 1300 cGy) B6D2F1 (H-2(b/d)) recipients were transplanted from B6 (H-2(b)) donors. P815 or L1214 (H-2(d)) tumor cells were injected intravenously or subcutaneously on day 1 post-transplantation to generate a GVL model.
RESULTS
Tumors in allogeneic mice treated with 1300 cGy exhibited markedly delayed subcutaneous tumor growth in vivo as compared with mice treated with 900 cGy, while intravenous tumor growths were comparable between the two radiation doses. Serum levels of tumor necrosis factor-α or interferon-γ were similar and the percentages of donor T-cell proliferation or apoptosis early after hematopoietic stem cell transplantation were comparable. The numbers of CD8(+) T cells from recipients that received 1300 cGy were significantly elevated in skin and tumor tissues. CD62L(low) and CD44(high) CD8(+) T-cell fractions were also elevated in 1300 cGy. After the higher radiation dose, P815-specific interferon-γ responses of splenic CD8(+) T cells were markedly enhanced and the fractions of T cells of interferon-γ-producing T cells in tumor tissues but not in spleen were increased. The protein concentrations of chemokines in skin and tumor tissues were substantially elevated in 1300 cGy compared to 900 cGy.
CONCLUSIONS
The higher radiation dose (1300 vs. 900 cGy) resulted in significantly enhanced GVL effect against an extramedullary tumor and the alterations in effector T-cell trafficking into tumor tissue are the most likely mechanism.
Topics: Animals; CD8-Positive T-Lymphocytes; Female; Graft vs Host Disease; Graft vs Leukemia Effect; Interferon-gamma; Leukemia, Experimental; Leukocyte Count; Lymphocytes, Tumor-Infiltrating; Mastocytoma; Mice; Mice, Inbred C57BL; Neoplasm Transplantation; Radiation Chimera; Radiotherapy Dosage; T-Lymphocyte Subsets; Transplantation Conditioning; Transplantation, Homologous; Tumor Necrosis Factor-alpha; Whole-Body Irradiation
PubMed: 21767512
DOI: 10.1016/j.exphem.2011.07.003 -
Archives of Dermatology Aug 2011To evaluate dermoscopic features in a group of 127 patients with mastocytosis in the skin and to investigate the relationship between different dermoscopic patterns and...
OBJECTIVES
To evaluate dermoscopic features in a group of 127 patients with mastocytosis in the skin and to investigate the relationship between different dermoscopic patterns and other clinical and biological characteristics of the disease.
DESIGN
Clinical and laboratory data were compared among patients with mastocytosis grouped according to the different dermoscopic patterns.
SETTING
Patients were selected from the Instituto de Estudios de Mastocitosis de Castilla La Mancha and the Department of Dermatology of Hospital Universitario Ramón y Cajal from April 1 through September 30, 2009. Patients Overall, 127 consecutive patients (70 females [55.1%] and 57 males [44.9%]; median age, 17 years; range, 0-81 years) with mastocytosis in the skin were included in the study.
MAIN OUTCOME MEASURES
Evaluation of dermoscopic patterns and investigation of potential predictive factors for more symptomatic forms of the disease according to the need for daily antimediator therapy.
RESULTS
Four distinct dermoscopic patterns were observed: yellow-orange blot, pigment network, reticular vascular pattern, and (most frequently) light-brown blot. A reticular vascular pattern was identified in all telangiectasia macular eruptiva and some maculopapular mastocytosis. In turn, all patients with mastocytoma displayed the yellow-orange blot pattern. The reticular vascular dermoscopic pattern was associated with the need for daily antimediator therapy; this pattern, together with serum tryptase levels and plaque-type mastocytosis, represented the best combination of independent factors to predict the need for maintained antimediator therapy.
CONCLUSIONS
Dermoscopy is a feasible method for the subclassification of mastocytosis. Of note, a reticular vascular pattern is more frequently associated with the need for antimediator therapy.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Dermoscopy; Female; Humans; Infant; Male; Mastocytosis, Cutaneous; Middle Aged; Young Adult
PubMed: 21844452
DOI: 10.1001/archdermatol.2011.190 -
Journal of Veterinary Diagnostic... May 2018Although quantitative assessment of margins is recommended for describing excision of cutaneous malignancies, there is poor understanding of limitations associated with...
Although quantitative assessment of margins is recommended for describing excision of cutaneous malignancies, there is poor understanding of limitations associated with this technique. We described and quantified histologic artifacts in inked margins and determined the association between artifacts and variance in histologic tumor-free margin (HTFM) measurements based on a novel grading scheme applied to 50 sections of normal canine skin and 56 radial margins taken from 15 different canine mast cell tumors (MCTs). Three broad categories of artifact were 1) tissue deformation at inked edges, 2) ink-associated artifacts, and 3) sectioning-associated artifacts. The most common artifacts in MCT margins were ink-associated artifacts, specifically ink absent from an edge (mean prevalence: 50%) and inappropriate ink coloring (mean: 45%). The prevalence of other artifacts in MCT skin was 4-50%. In MCT margins, frequency-adjusted kappa statistics found fair or better inter-rater reliability for 9 of 10 artifacts; intra-rater reliability was moderate or better in 9 of 10 artifacts. Digital HTFM measurements by 5 blinded pathologists had a median standard deviation (SD) of 1.9 mm (interquartile range: 0.8-3.6 mm; range: 0-6.2 mm). Intraclass correlation coefficients demonstrated good inter-pathologist reliability in HTFM measurement (κ = 0.81). Spearman rank correlation coefficients found negligible correlation between artifacts and HTFM SDs ( r ≤ 0.3). These data confirm that although histologic artifacts commonly occur in inked margin specimens, artifacts are not meaningfully associated with variation in HTFM measurements. Investigators can use the grading scheme presented herein to identify artifacts associated with tissue processing.
Topics: Animals; Artifacts; Biopsy; Dog Diseases; Dogs; Margins of Excision; Mastocytoma, Skin; Observer Variation; Reproducibility of Results; Skin Neoplasms
PubMed: 29429400
DOI: 10.1177/1040638718757582 -
Vaccine Jun 2015We investigated the influence of the site of administration of DNA vaccine on the induced immune response. DNA vaccines were administered by electroporation at three...
We investigated the influence of the site of administration of DNA vaccine on the induced immune response. DNA vaccines were administered by electroporation at three different sites: tibial cranial muscle, abdominal skin and ear pinna. Aiming to draw general conclusions about DNA vaccine delivery, we successively used several plasmids encoding either luciferase and ovalbumin as models or gp160 and P1A as vaccines against HIV and P815 mastocytoma, respectively. Low levels and duration of luciferase transgene expression were observed after electroporation of the abdominal skin, partly explaining its lower immunogenic performance as compared to the other sites of administration. Analyses of OT-I CD8+ and OT-II CD4+ T cell responses highlighted the differential impact of the delivery site on the elicited immune response. Muscle electroporation induced the strongest humoral immune response and both muscle and ear pinna sites induced cellular immunity against gp160. Ear pinna delivery generated the highest level of CTL responses against P1A but electroporation of muscle and ear pinna were equally efficient in delaying P815 growth and improving mice survival. The present study demonstrated that the site of administration is a key factor to be tested in the development of DNA vaccine.
Topics: AIDS Vaccines; Abdomen; Administration, Cutaneous; Animals; Antigens, Neoplasm; CD4-Positive T-Lymphocytes; CD8-Positive T-Lymphocytes; Cancer Vaccines; Ear Auricle; Electroporation; Female; HIV Antibodies; HIV Envelope Protein gp160; HIV-1; Hindlimb; Immunity, Cellular; Immunity, Humoral; Luciferases; Mice, Inbred C57BL; Muscle, Skeletal; Plasmids; Skin; Vaccination; Vaccines, DNA
PubMed: 25980430
DOI: 10.1016/j.vaccine.2015.05.005 -
Indian Dermatology Online Journal 2016
PubMed: 27057508
DOI: 10.4103/2229-5178.178091 -
Experimental Animals Apr 2000Spontaneous cutaneous mastocytomas in Djungarian hamsters (D-hamster) were pathologically studied and compared with those in canine and feline cases. Eight (9.3%) of 86... (Comparative Study)
Comparative Study
Spontaneous cutaneous mastocytomas in Djungarian hamsters (D-hamster) were pathologically studied and compared with those in canine and feline cases. Eight (9.3%) of 86 cutaneous biopsy cases in D-hamsters were diagnosed as mastocytomas, being slightly higher in incidence than in canine and feline species. In 4 of 8 D-hamster cases, the tumor lesions were in the head and neck in contrast to most canine lesions in the extremities. The histopathology of the D-hamster mastocytoma was characterized by diffuse or massive proliferation of well-differentiated tumor cells with severe degeneration of collagen fibers and slight eosinophil infiltration in most cases.
Topics: Animals; Cats; Chymases; Cricetinae; Dogs; Female; Immunoenzyme Techniques; Male; Mast Cells; Mast-Cell Sarcoma; Phodopus; Proliferating Cell Nuclear Antigen; Serine Endopeptidases; Skin Neoplasms; Species Specificity; Tryptases
PubMed: 10889951
DOI: 10.1538/expanim.49.127