scholarly journals Cutaneous mastocytosis in an infant: Case report and clinicopathological correlation

Author(s):  
Solanki Prithviraj Kishoresingh ◽  
Bhide Vijayshri ◽  
Gadage Vijaya ◽  
Kulkarni Vinay ◽  
Patki Anil

Mastocytosis is a disorder of clonal proliferation of the mast cells, which can be cutaneous or systemic. Abnormal mast cell infiltration comprising multifocal compact clusters or cohesive aggregates can affect one or more organ systems. Cutaneous mastocytosis is a relatively uncommon condition in the pediatric population. We report a case of 9 month infant presented with multiple papular and vesicular skin rashes since 6 months of age. On clinical examination Darier’s sign was negative. The serum tryptase levels were within normal limits. Clinical differential diagnoses were benign cephalic histiocytosis vs cutaneous mastocytosis. Skin biopsy revealed a mononuclear cell infiltrate in the papillary dermis reaching up to the dermo-epidermal junction. Toluidine blue staining highlighted the metachromatic granules. CD117, CD30 IHC stains were positive which confirmed the diagnosis of cutaneous mastocytosis. This case is presented to highlight the histomorphology and the special stains in cases of mastocytosis.

2017 ◽  
Vol 9 (3) ◽  
pp. 124-128
Author(s):  
Svetlana Popadić ◽  
Mirjana Gajić-Veljić ◽  
Biljana Marenović ◽  
Miloš Nikolić

Abstract Mastocytosis refers to a group of diseases characterized by a clonal proliferation and accumulation of mast cells in one or more tissues/organs with different clinical presentations. In children, limited cutaneous forms of mastocytosis are rather frequent, while systemic mastocytosis is rare. The diagnosis of cutaneous mastocytosis is based on clinical findings and histopathology. We present a patient who developed skin lesions at the age of 18 months. Clinical findings, confirmed by histopathology, were consistent with diffuse cutaneous mastocytosis. The follow-up period was 7 years. The treatment included oral antihistamines in combination with mast cell stabilizers, mild topical steroids and avoidance of friction. During the follow-up period, there were no signs of systemic involvement, and the quality of life was preserved, despite the large surface of affected skin. This case report should increase the awareness and knowledge of clinicians about this rare form of cutaneous mastocytosis in the pediatric population.


2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Tuntas Rayinda ◽  
Dyah Ayu Mira Oktarina ◽  
Retno Danarti

Diffuse cutaneous mastocytosis is a rare form of cutaneous mastocytosis that can appear in heterogeneous clinical presentations, including eruption of papules, erythematous plaques, blisters, and erythroderma. We report a 1.5- year-old boy who presented with itchy wheals and blisters spreading on his body. The patient was initially managed as a linear IgA bullous dermatosis of childhood (LABD) because of the similarity of clinical symptoms and the presenting of linear IgA deposits at the basement membrane. Due to the development of urticarial plaque after the resolution of the blisters, the diagnosis of diffuse cutaneous mastocytosis was made based on clinical, histopathological (hematoxylin-eosin, Giemsa, and toluidine blue staining), and direct immunofluorescent examinations (IgA, IgG, IgM, C3). The symptoms were improved following antihistamines and oral corticosteroid treatment.


2011 ◽  
Vol 23 (6) ◽  
pp. 1222-1225 ◽  
Author(s):  
Jorge Martínez ◽  
Vicente Martínez ◽  
Llorenç Grau-Roma ◽  
Javier López ◽  
Joaquim Segalés

Cutaneous tumors are not usually observed in pigs, and mast cell tumors, in particular, have been rarely described. The current report describes numerous, pink to red, elevated, 1–5-cm nodules in the skin of a 6-month-old slaughter pig. The histopathology of the nodules revealed well-demarcated, but poorly differentiated, populations of round cells that extended from the dermis into subcutaneous tissues. The presence of eosinophils and the observation, at high magnification, of cells with cytoplasmic metachromatic granules, after toluidine blue staining, confirmed the diagnosis of multiple cutaneous mast cell tumors.


2021 ◽  
Vol 10 (7) ◽  
pp. 1474
Author(s):  
Florica Sandru ◽  
Răzvan-Cosmin Petca ◽  
Monica Costescu ◽  
Mihai Cristian Dumitrașcu ◽  
Adelina Popa ◽  
...  

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. It must comprise laboratory exams (with baseline dosing of total serum tryptase), a skin biopsy (with a pathological exam and, if the diagnosis is unclear, immunohistochemical tests), and a complete clinical evaluation. It is also defining to distinguish between CM and other diseases with cutaneous involvement. As for the management of CM in children, the first intervention implies eliminating trigger factors. The available cures are oral H1 and/or H2 antihistamines, oral cromolyn sodium, oral methoxypsoralen therapy with long-wave psoralen plus ultraviolet A radiation, potent dermatocorticoid, and calcineurin inhibitors. In children, the prognosis of CM is excellent, especially if the disease’s onset is in the first or second years of life.


2021 ◽  
Vol 147 (2) ◽  
pp. AB136
Author(s):  
Alba Juarez ◽  
Alicia Dominguez ◽  
Joaquín Navarro ◽  
Maria Luisa Baeza ◽  
Alberto Alvarez-Perea

2021 ◽  
Vol 9 (2) ◽  
pp. 80-81
Author(s):  
Dorothea Wieczorek

<b>Background:</b>Venom-induced anaphylaxis (VIA) is a common, potentially life-threatening hypersensitivity reaction associated with (1) a specific symptom profile, (2) specific cofactors, and (3) specific management. Identifying the differences in phenotypes of anaphylaxis is crucial for future management guidelines and development of a personalized medicine approach. <b>Objective:</b>This study aimed to evaluate the phenotype and risk factors of VIA. <b>Methods:</b>Using data from the European Anaphylaxis Registry (12,874 cases), we identified 3,612 patients with VIA and analyzed their cases in comparison with sex- and age-matched anaphylaxis cases triggered by other elicitors (non-VIA cases [n = 3, 605]). <b>Results:</b>VIA more frequently involved more than 3 organ systems and was associated with cardiovascular symptoms. The absence of skin symptoms during anaphylaxis was correlated with baseline serum tryptase level and was associated with an increased risk of a severe reaction. Intramuscular or intravenous epinephrine was administered significantly less often in VIA, in particular, in patients without a history of anaphylaxis. A baseline serum tryptase level within the upper normal range (8–11.5 ng/mL) was more frequently associated with severe anaphylaxis. <b>Conclusion:</b>Using a large cohort of VIA cases, we have validated that patients with intermediate baseline serum tryptase levels (8–11 ng/mL) and without skin involvement have a higher risk of severe VIA. Patients receiving β-blockers or angiotensin-converting enzyme inhibitors had a higher risk of developing severe cardiovascular symptoms (including cardiac arrest) in VIA and non-VIA cases. Patients experiencing VIA received epinephrine less frequently than did cases with non-VIA.


2020 ◽  
Vol 64 (4) ◽  
pp. 375-377
Author(s):  
Ekkehard Hewer ◽  
Anja M. Schmitt

Rapid on-site evaluation (ROSE) is one of cytopathology’s “unique selling propositions.” The quality, speed, and ease of handling of the staining used is a critical factor for the efficacy of the ROSE procedure. Here, we describe a modification of rapid toluidine blue staining that can be performed within 25 s, provides excellent nuclear morphology, and is compatible with subsequent Papanicolaou staining of the slides. Furthermore, exposure to hazardous chemicals is minimized, as no organic solvents other than the alcohol-based fixative and glycerin for temporary mounting and coverslipping are required. We have used this protocol successfully in our ROSE practice and have not observed any discrepancies between toluidine blue- and permanent Papanicolaou-stained slides.


2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Gabriela Elena Lupusoru ◽  
Ioana-Georgiana Ailincai ◽  
Andreea Gabriella Andronesi ◽  
Mircea Lupusoru ◽  
Lavinia Maria Bernea ◽  
...  

Abstract Background and Aims Hantavirus infection is a zoonosis rare in the Balkan Peninsula but with increasing frequency and geographic spread, causing two major syndromes, depending on the viral serotype: hemorrhagic fever with renal syndrome (HFRS) and cardiopulmonary syndrome (CPS). Because there is no specific treatment or vaccine for this condition, the key for minimizing the progression to chronic kidney disease, secondary hypertension or death is early diagnosis and prompt therapy. This paper presents a case of HFSR in which needle kidney biopsy played a major role in diagnosis and draws attention on this zoonosis that might be highly underdiagnosed in Balkan Peninsula. Method A 26-year-old female with no medical history was admitted in our department with acute kidney injury (AKI), nephritic syndrome with nephrotic range proteinuria, high blood pressure, hepatic cytolysis, severe thrombocytopenia, anemia and leukocytosis, elevated LDH, normal haptoglobin, positive Coombs test (Table 1). Immunological testing (C3, C4, ANA, ANCA, antiGBM), viral infection markers (hepatitis B/C, HIV, Epstein-Barr, Cytomegalovirus), IgA/M/G were all negative and ADAMTS13 activity was normal. Abdominal sonography showed both kidneys of normal size and shape. A kidney biopsy was performed. The biopsy specimen showed macroscopic features of hemorrhage in the renal medulla. In immunofluorescence the staining was negative for IgA, IgG, IgM, C1q, C3c, k and λ chains, albumin and fibrinogen. Light microscopy (LM) revealed normal glomeruli and arterioles, dilated proximal tubules with resorption droplets at the apical pole and erythrocytes in the lumen, important interstitial hemorrhage in the medulla, with no inflammation or interstitial fibrosis. The electron microscopy (EM) showed segmental foot process effacement, endotheliosis of the peritubular capillaries, rare plasmocytes and macrophages in the interstitium (Figure 1). The aspect of hemorrhagic interstitial nephritis suggested Hantavirus infection. Serological testing revealed both IgM and IgG antibodies for the Hantaan serotype (HTNV). The final diagnosis was HTNV hemorrhagic interstitial nephritis with intrinsic AKI and secondary hypertension. MO426   Figure 1: A, B LM, Toluidine Blue staining. Normal glomerulus. Resorption droplets in the proximal tubular cells. C, D LM, Toluidine Blue staining. Extensive interstitial hemorrhage in medulla, tubulitis. E EM. Interstitial extravasation of erythrocytes. F EM. Endothelial swelling, foot process effacement. Results The patient was treated with oral methylprednisolone 16mg/d for 2 weeks, with progressive tampering of the dose and removal after 2 months. She received antihypertensive and antiproteinuric treatment with ramipril. The evolution was good, with creatinine and liver enzymes returning to normal. Conclusion HFRS belongs to a group of rare zoonoses in Balkan Peninsula, the most involved serotypes being Dobrava and Puumala. This case had positive serology for HTNV usually being found in China and Russia, but our patient didn’t travel abroad before she got ill, so we can’t consider the case as being an imported infection. That highlights a possible underdiagnosis of the disease in this region and also the need to re-evaluate geographic distribution of different strains and changes in ecological aspects given that they may pose a major risk to public health. The disease begins with flu-like symptoms and progresses to AKI with severe thrombocytopenia, anemia and coagulation disorders, being easily mistaken for haemolytic uremic syndrome. In a region with sporadic cases, we face diagnosis difficulties related especially to the absence of initial diagnosis suspicion, so we emphasize the need to include this pathology in the differential diagnosis algorithm of diseases evolving with thrombocytopenia, anemia, hepatic cytolysis and renal injury.


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