ABSTRACT
OBJECTIVE: This paper describes two different clinical presentations of diffuse cutaneous mastocytosis (DCM), based on the largest series published to date. As far as we are aware, these two variants of clinical presentations have not yet been reported. DESIGN: We undertook a case controlled analysis of 8 children with DCM. Results of laboratory testing including mast cell mediator levels, and clinical symptoms on presentation and during follow-up were analyzed. RESULTS: The levels of relevant mast cell mediators were initially high in all cases but declined sharply later on. There was a reduction of 20% in 2 of the 7 cases, whereas there was a reduction of 80% in the remaining 5. No reduction occurred in 1 case. Clinical improvement followed the same pattern. CONCLUSIONS: DCM is a rare variant of cutaneous childhood onset mastocytosis. Various forms show the same or overlapping features at various times. It appears to follow a course similar to that in other types of childhood onset mastocytosis, taking into account the decreased symptoms and the levels of mast cell mediators during follow-up. Obtaining a bone marrow biopsy should be considered only in those cases where there is no improvement or even worsening of signs or symptoms and persistent elevated levels of mast cell mediators.
Subject(s)
Mastocytosis, Cutaneous/classification , Mastocytosis, Cutaneous/pathology , Adolescent , Adult , Age of Onset , Belgium , Biomarkers/blood , Biopsy , Case-Control Studies , Child , Child, Preschool , Diagnosis, Differential , Greece , Humans , Infant , Male , Mast Cells/pathology , Mastocytosis, Cutaneous/diagnosis , Mastocytosis, Cutaneous/therapy , Severity of Illness Index , Shoulder/pathology , Thoracic Wall/pathology , Tryptases/bloodABSTRACT
Mastocytosis is characterized by an increased number of mast cells with an abnormal growth and accumulation in one or more organs. In most children mastocytosis is limited to the skin (cutaneous mastocytosis) and often transient as compared with that in adults in whom mastocytosis is usually progressive and systemic. Generally, we recognize three more common forms of cutaneous mastocytosis: maculopapulous mastocytosis (formerly urticaria pigmentosa), mastocytoma of skin, and diffuse cutaneous mastocytosis. Childhood mastocytosis can further be divided into cutaneous mastocytosis (nonpersisting and persisting) and systemic mastocytosis (extremely rare). An approach to management using a set protocol is described in table form. In most cases of mastocytosis, only yearly checkups are necessary and no treatment is required; preventive recommendations are warranted in those individuals with systemic disease and constitutional symptoms. Symptomatic therapy is advised in only a minority of cases. This article is meant as a guideline for physicians involved in the care of children with mastocytosis and their parents.
Subject(s)
Mastocytosis , Child , Humans , Mastocytosis/diagnosis , Mastocytosis/therapy , Mastocytosis, Cutaneous/diagnosis , Mastocytosis, Cutaneous/therapyABSTRACT
BACKGROUND: Mastocytosis is a disorder that can be subdivided into two forms: cutaneous and systemic. Patients with cutaneous mastocytosis only may suffer from cosmetic problems. Topical steroid application has been shown to be effective in cases of limited skin lesions. METHODS: A case-controlled pilot study was conducted during a 6-weeks treatment using diluted 25% fluticasone propionate 0.05% cream under wet-wrap occlusion in 5 adults and 6 children. Improvement was measured up to the 24th week after treatment using the SCORMA Index. RESULTS: The results of this pilot study showed a partial but clear cosmetic improvement in 9 of the 11 patients. The mean SCORMA Index decreased after treatment from 38 to 26. CONCLUSION: 25% dilution of fluticasone propionate 0.05% cream under wet-wrap occlusion is an alternative treatment modality for alleviating the symptoms of cutaneous mastocytosis, but the improvement may be moderate and fall short of the patient's expectations.
