A Cutaneous mastocytosis: A self-limiting benign disease in a series of 45 cases

Abstract

Background  Cutaneous mastocytosis is a rare disease. It results from a hyperplastic response of mast cells to abnormal stimuli due of activating c-KIT mutations.   Objective To collect all cases of cutaneous mastocytosis and evaluate them clinically and histopathologically.   Methods This is a case series descriptive study where cutaneous mastocytosis cases were collected during the period from 2017-2021. Full history and clinical evaluation were carried out. Skin biopsies were processed and stained with Giemsa stain and CD117 IHC marker to evaluate histopathological changes. Therapeutic trial using antihistamine and corticosteroids were conducted.   Results Analysis of forty five cases with the classical cutaneous mastocytosis was carried out involving 26 males (57.77%) and19 females (42.22%). Their ages ranged from 1-15 years with a mean±SD of 3.5±3.7 years while the age of onset was mostly after the age of one year. All patients had itching with variable severity. Forty-two (93.3%) patients had a generalized rash while 3(6.7%) cases had localized lesions. The rash was dark brown maculopapular rash in 35 (77.77%), bullous in 4(8.88%), plaque in 3 (6.66%), nodular in 2 (4.44%), and xanthogranuloma like in 1(2.22%) case. The Disease was slow and regressing apart from bullous variant which run aggressive course. Histopathology showed mast cells proliferation in the superficial and deep dermis and sub-epidermal blisters in four patients. All patients fulfilled the diagnostic criteria of cutaneous mastocytosis. All patients responded to therapy and their rash and symptoms were relieved.   Conclusion Cutaneous mastocytosis is a disease of children that runs a benign course and then regresses spontaneously. They all needed symptomatic treatment until they had a full remission.