Topical pimecrolimus for paediatric cutaneous mastocytosis

Mashiah, Jacob; Harel, Avikam; Bodemer, Christine; Hadj‐Rabia, S.; Goldberg, Ilan; Sprecher, Eli; Kutz, Ana

doi:10.1111/ced.13391

Cited by 19 publications

(8 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“… 49 Instead of corticosteroids, local application of pimecrolimus is recommended in pediatric CM due to its excellent results and safety profiles. 18 , 50 , 51 Pimecrolimus is a calcineurin inhibitor with significant anti-inflammatory activity by blocking T-cell activation, inhibiting inflammatory cytokine synthesis, and immunomodulatory effects with a low systemic immunosuppressive potential. Disodium cromoglycate at a 1% to 4% concentration in an aqueous solution or mixed with a water-based emollient cream may decrease itching.…”

Section: Treatmentmentioning

confidence: 99%

Pediatric Mastocytosis: An Update

Giona

2021

Mediterr J Hematol Infect Dis

View full text Add to dashboard Cite

Mastocytosis is a rare clonal disorder, characterized by excessive proliferation and accumulation of mast cells (MC) in various organs and tissues. Cutaneous mastocytosis (CM), the most common form in children, is defined when MC infiltration is limited to the skin. Systemic mastocytosis (SM), the most common form in adults, is characterized by MC proliferation and accumulation in organs, such as bone marrow, lymph nodes, liver and spleen (1). Genetic aberrations, mainly the KIT D816V mutation, play a crucial role in the pathogenesis of mastocytosis, resulting in enhancing MC survival and subsequent accumulation in organs and tissues (2,3). CM includes 3 forms: solitary mastocytoma, maculopapular cutaneous mastocytosis (MPCM) and diffuse cutaneous mastocytosis (DCM). In the majority of children with CM, skin lesions regress spontaneously around puberty; unfortunately, in a few cases, it is not a self-limiting disease (4). Even if SM occurs occasionally, all children with mastocytosis require planned follow-up over time. Children with mastocytosis often suffer from MC mediator-related symptoms, the most common of which is itching, often triggered by rubbing the lesions. Management of pediatric mastocytosis is mainly based on strict avoidance of triggers. Treatment with H1 and H2 histamine receptor blockers on demand, and the availability of epinephrine auto-injectors for the patients to use in case of severe anaphylactic reactions are recommended.

show abstract

Section: Treatmentmentioning

confidence: 99%

Pediatric Mastocytosis: An Update

Giona

2021

Mediterr J Hematol Infect Dis

View full text Add to dashboard Cite

show abstract

“…Therapy with topical antibiotics such as mupirocine or fucidic acid is recommended in children with denuded skin areas to prevent skin infections, particularly in those with blistering and DCM (33). Another therapeutic option is pimecrolimus, a calcineurin inhibitor that has shown effects in children with symptomatic CM (79,80). In particular, effective and safe therapy of MPCM and mastocytoma with 1% pimecrolimus cream applied twice daily on skin lesions was reported (79).…”

Section: Topical Therapy Of Skin Symptomsmentioning

confidence: 99%

“…Another therapeutic option is pimecrolimus, a calcineurin inhibitor that has shown effects in children with symptomatic CM (79,80). In particular, effective and safe therapy of MPCM and mastocytoma with 1% pimecrolimus cream applied twice daily on skin lesions was reported (79).…”

Section: Topical Therapy Of Skin Symptomsmentioning

confidence: 99%

Mediator-Related Symptoms and Anaphylaxis in Children with Mastocytosis

Brockow

Plata-Nazar

Lange

et al. 2021

Preprint

View full text Add to dashboard Cite

Mastocytosis is characterized by pathological accumulation of mast cells (MC) in various organs. In these patients, MC may degranulate and thereby contribute to clinical symptoms, especially when a concomitant allergy is present. However, MC activation can not only be induced by high-affinity receptors for IgE, but also by anaphylatoxins, neuropeptides, IgG immune complexes, complement-components, drugs, products of bacteria or parasites, as well as physical factors such as heat, cold, vibration, stress, sun, or physical effort. Symptoms due to mediators released by activated MC may develop in adults suffering from systemic mastocytosis, but also evolve in children who usually have cutaneous mastocytosis (CM). Clinically, CM is otherwise characterized by typical brown, maculopapular skin lesions or mastocytoma associated with a positive Darier’s sign. Pruritus and flushing are common and blistering may also be recorded, especially in diffuse CM (DCM). Pediatric patients with mastocytosis may also have gastrointestinal, respiratory, and neurologic complaints. Although anaphylaxis is not a typical finding, pediatric patients with massive skin involvement and high tryptase levels have a relatively high risk to develop anaphylaxis. This paper reviews MC mediator-related symptoms and anaphylaxis in children with mastocytosis, with special emphasis on risk factors, triggers, and management.

show abstract

“…As some of the lesions on the skin had an uneven surface, it was not always feasible to measure the quantitative changes in vertical dimension and calculate the tumor volume. In an attempt to better reflect these changes, cutaneous response was also assessed via a scoring system adapted and modified from Mashiah et al and Maytin et al [18,19]. In this scoring system, each lesion was scored based on two parameters, change in horizontal dimension (progression > 20% increase = -1, stable = 0, partial regression > 30% decrease = 1, complete regression = 2) and vertical dimension (prominent elevation = -0.5, minor change (decrease or increase) in elevation or no change at all = 0, prominent flattening = 0.5).…”

Section: Assessment Of Study Outcomementioning

confidence: 99%

Efficiency of long-term high-dose intravenous ascorbic acid therapy in locally advanced basal cell carcinoma – a pilot study

Bánvölgyi¹,

Lőrincz²,

Kiss³

et al. 2020

pdia

View full text Add to dashboard Cite

Introduction: The anti-cancer properties of high-dose intravenous ascorbic acid have been demonstrated in various malignancies. In our recent study, we tested topically applied ascorbic acid to treat basal cell carcinoma (BCC), and achieved a good clinical response. Aim: Based on these results, we decided to examine the efficacy and tolerability of high-dose intravenous ascorbic acid (IVA) for locally advanced BCC. Material and methods: In this pilot study, patients diagnosed with locally advanced BCC who were not amenable to radiation, surgical or local therapy (no other treatment option was available at the time) received intravenous ascorbic acid (1-1.8 g/kg), in an outpatient setting, 1-3 times per week for a mean duration of 42 ±23.6 weeks. This therapy was generally well tolerated. Results: Among 4 patients who had a total of 165 (mean: 41 ±51, range: 1-114) skin lesions, 3 patients achieved stable disease and one had progressive disease. There was substantial variability in individual tumor response to therapy. With the aid of two-photon microscopy and second harmonic generation imaging techniques, alterations in collagen structure were observed between tumor nests during IVA therapy. Conclusions: Our results suggest that IVA is well tolerated in a small group of patients with extensive BCCs. However, in the era of smoothened (Smo) receptor inhibitors, it may only be considered as an adjuvant therapy in treatment-resistant cases.

show abstract

Topical pimecrolimus for paediatric cutaneous mastocytosis

Abstract: Topical therapy with pimecrolimus 1% cream should be considered in the treatment of CM.

Cited by 19 publications

References 25 publications

Pediatric Mastocytosis: An Update

Pediatric Mastocytosis: An Update

Mediator-Related Symptoms and Anaphylaxis in Children with Mastocytosis

Efficiency of long-term high-dose intravenous ascorbic acid therapy in locally advanced basal cell carcinoma – a pilot study

Contact Info

Product

Resources

About