Cutaneous Mastocytosis with Persistent Blistering: Successful Treatment with Methylprednisolone and 3-Year Follow-Up Management

Abstract

BackgroundMastocytosis is a rare disorder with diverse clinical manifestations. In cutaneous mastocytosis the mast cell infiltration is limited to the skin, but is often associated with systemic symptoms due to the release of mast cell mediators.Case ReportWe report a 6-month-old male infant who had skin lesions of various morphologies (macules, papules, plaques, and nodules) and sizes, persistent blistering and frequent flushing episodes for half a year. Vital signs and physical examinations were unremarkable. No abnormalities in the laboratory tests were found except for a serum tryptase level (STL) of 11.8 ng/ml. The histological and immunohistochemical examinations confirmed the diagnosis of cutaneous mastocytosis. The patient was first treated with methylprednisolone, oral levocetirizine, and topical fusidic acid/betamethasone cream. Subsequently the treatment was tapered and stopped within 9 weeks. The child’s symptoms improved and were successfully controlled with intermittent courses of ketotifen and topical hydrocortisone over 3 years.ConclusionChildhood cutaneous mastocytosis usually has a favorable prognosis, but in some cases the disease can progress with skin manifestations necessitating a more active systemic and topical treatment.Electronic supplementary materialThe online version of this article (doi:10.1007/s13555-015-0073-6) contains supplementary material, which is available to authorized users.

Cutaneous mastocytosis treatment: strategies, limitations and perspectives

“…Systemic GCs are not recommended in CM because of their systemic, serious side effects, particularly diabetes, high blood pressure, osteoporosis, infections, cardiovascular disease and Cushing syndrome. However, systemic GCs can be ordered in short-time therapy in extremely severe CM with mediator-related symptoms or bullous lesions [10, 11]. Taking into account that pediatric CM is mostly benign disease, only non-sedating H1 antihistamines are required in the majority of cases.…”

Mast cells: Therapeutic targets for COVID‐19 and beyond

“…For example, montelukast and zafirlukast are leukotriene receptor antagonists widely used in the management of chronic asthma 109 and might be used to prevent the actions of leukotrienes in bronchoconstriction and lung inflammation during SARS‐CoV‐2 infection. Moreover, montelukast might also possess anti‐viral effect by targeting the 3CL protease of SARS‐CoV‐2 110 ; Methylprednisolone, which is a corticosteroid that has been used to treat cutaneous mastocytosis, 24 , 111 , 112 might be able to alleviate the hyperinflammation in COVID‐19 patients caused by uncontrolled MC activation; Ketotifen and azelastine that are commonly used in asthma and allergic rhinitis, respectively, act as histamine 1 (H1) receptor antagonists to inhibit airway inflammation and bronchoconstriction 24 , 109 ; Sodium cromoglicate which is generally considered as a mast cell stabilizer and is used in asthmatic and allergic rhinitis patients, may also be applied to inhibit MC activation to alleviate bronchoconstriction and control the cytokine storm during COVID‐19. 43 , 113 However, only <5% of sodium cromoglicate can be absorbed orally and rapid tachyphylaxis develops against sodium cromoglicate.…”

Епидемиология На Системната Мастоцитоза

“…Прави впечатление изключително малкият брой научни публикации от български автори за тази група заболявания. При търсене по комбинация от ключови думи "mastocytosis" и "Bulgaria" в PubMed са открити само две публикации [23][24]. Докато втората описва случай на кожна мастоцитоза, първата представя оценка на заболеваемостта от вторични солидни тумори при пациенти с мастоцитоза.…”