Cutaneous mastocytosis: diagnostic challenges and dietary influences in a prolonged case report


Submitted: 17 September 2024
Accepted: 7 October 2024
Published: 23 October 2024
Abstract Views: 263
PDF: 91
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

Authors

Cutaneous mastocytosis is a rare condition characterized by abnormal mast cell proliferation in the skin. We present the case of a 65-year-old man with recurrent urticaria-like rashes persisting for more than eight years. Despite multiple misdiagnoses as insect bites, examination and biopsy revealed mast cell infiltration. The patient's unique lifestyle, particularly his frequent seafood consumption, adds an intriguing dimension to the case. Treatment with systemic antihistamines and corticosteroids provided symptomatic relief, but long-term follow-up showed the persistence of clinical lesions, indicating that the treatment did not adequately address the underlying cause of the disease. A notable improvement in disease control was observed when the patient's diet was adjusted, suggesting a potential link between food and cutaneous mastocytosis. To our knowledge, this is the first documented case of maculopapular cutaneous mastocytosis in Syria.


Valent P, Akin C, Escribano L, et al. Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest 2007;37:435-53. DOI: https://doi.org/10.1111/j.1365-2362.2007.01807.x

Valent P, Akin C, Hartmann K, et al. Advances in the classification and treatment of mastocytosis: current status and outlook toward the future. Cancer Res 2017;77:1261-70. DOI: https://doi.org/10.1158/0008-5472.CAN-16-2234

Theoharides TC, Valent P, Akin C. Mast cells, mastocytosis, and related risorders. N Engl J Med 2015;373:163-72. Mastocytosis - Symptoms, Causes, Treatment | NORD. Accessed May 1, 2024. available from: https://rarediseases.org/rare-diseases/mastocytosis/ DOI: https://doi.org/10.1056/NEJMra1409760

Hartmann K, Escribano L, Grattan C, et al. Cutaneous manifestations in patients with mastocytosis: consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology. J Allergy Clin Immunol 2016;137:35-45. DOI: https://doi.org/10.1016/j.jaci.2015.08.034

Di Raimondo C, Del Duca E, Silvaggio D, et al. Cutaneous mastocytosis: a dermatological perspective. Australas J Dermatol 2021;62:e1-e7. DOI: https://doi.org/10.1111/ajd.13443

Valent P. Mastocytosis: a paradigmatic example of a rare disease with complex biology and pathology. Am J Cancer Res 2013;3:159-72.

Nirmal B, Krishnaram AS, Muthu Y, et al. Dermatoscopy of urticaria pigmentosa with and without Darier’s sign in skin of color. Indian Dermatol Online J 2019;10:577-79. DOI: https://doi.org/10.4103/idoj.IDOJ_501_18

Maintz L, Novak N. Histamine and histamine intolerance. Am J Clin Nutr 2007;85:1185-96. DOI: https://doi.org/10.1093/ajcn/85.5.1185

Hoffbrand AV, Vyas P, Campo E, et al. Mastocytosis, myeloid/lymphoid neoplasms with eosinophilia and specific cytogenetic rearrangements, myelodysplastic/myeloproliferative neoplasms. In: Hoffbrand AV, Vyas P, Campo E, et al. (eds.) Color Atlas of Clinical Hematology. Wiley; 2018.

Criscuolo M, Fianchi L, Maraglino AME, et al. Mastocytosis: one word for different diseases. Oncol Ther 2018;6:129-40. DOI: https://doi.org/10.1007/s40487-018-0086-2

Doyle LA, Hornick JL. Pathology of extramedullary mastocytosis. Immunol Allergy Clin North Am 2014;34:323-39. DOI: https://doi.org/10.1016/j.iac.2014.01.010

Folkerts J, Stadhouders R, Redegeld FA, et al. Effect of dietary fiber and metabolites on mast cell activation and mast cell-associated diseases. Front Immunol 2018;9:1067. DOI: https://doi.org/10.3389/fimmu.2018.01067

Molderings GJ, Brettner S, Homann J, et al. Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. J Hematol Oncol 2011;4:10. DOI: https://doi.org/10.1186/1756-8722-4-10

Schnedl WJ, Lackner S, Enko D, et al. Evaluation of symptoms and symptom combinations in histamine intolerance. Intest Res 2019;17:427-733. DOI: https://doi.org/10.5217/ir.2018.00152

Gülen T, Hägglund H, Dahlén B, et al Nilsson G. Mastocytosis: the puzzling clinical spectrum and challenging diagnostic aspects of an enigmatic disease. J Intern Med 2016;279:211-28. DOI: https://doi.org/10.1111/joim.12410

Rouet A, Aouba A, Damaj G, et al. Mastocytosis among elderly patients: a multicenter retrospective french study on 53 patients. Medicine (Baltimore). 2016;95:e3901. DOI: https://doi.org/10.1097/MD.0000000000003901

Cavazos A, Subrt P, Tschen JA. Delayed diagnosis of adult-onset mastocytosis. Baylor Univ Med Cent Proc 2022;35:717-18. DOI: https://doi.org/10.1080/08998280.2022.2081914

Al-Soufi, L., Marashli, A., HajBakri, R. C., & Al-Shehabi, Z. (2024). Cutaneous mastocytosis: diagnostic challenges and dietary influences in a prolonged case report. Dermatology Reports. https://doi.org/10.4081/dr.2024.10143

Downloads

Download data is not yet available.

Citations