ABSTRACT
BACKGROUND/OBJECTIVES: Though maculopapular cutaneous mastocytosis is the most common form of pediatric mastocytosis, it remains unclear which patients will experience severe symptoms. We sought to better define the presentation and the cutaneous and systemic signs and symptoms in patients with maculopapular cutaneous mastocytosis. METHODS: We analyzed retrospective data on 227 patients diagnosed with maculopapular cutaneous mastocytosis prior to age 15 years from five US clinical sites. We collected data on signs, symptoms, age of onset, and laboratory testing. RESULTS: Median age of onset of maculopapular cutaneous mastocytosis was 3 months, with 94% of patients presenting prior to age 2 (range 0-15 years). Patients presenting before age 2 had significantly lower serum tryptase level (P = .019). Greater number of skin lesions (P = .006), number of reported skin signs and symptoms (P < .001), and higher tryptase levels (P < .001) were associated with more systemic symptoms. CONCLUSION: Children with maculopapular cutaneous mastocytosis, who have greater skin involvement, higher serum tryptase level, and more skin signs and symptoms, are more likely to have systemic symptoms.
Subject(s)
Mastocytosis, Cutaneous , Mastocytosis , Urticaria Pigmentosa , Adolescent , Child , Child, Preschool , Humans , Infant , Infant, Newborn , Mastocytosis, Cutaneous/diagnosis , Mastocytosis, Cutaneous/epidemiology , Retrospective Studies , Skin , Tryptases , Urticaria Pigmentosa/diagnosis , Urticaria Pigmentosa/epidemiologyABSTRACT
Angioimmunoblastic T-cell lymphoma (AITL) is a rare form of non-Hodgkin lymphoma often accompanied by autoimmune and paraneoplastic phenomena. Up to 50% of patients with AITL present with skin manifestations. This case series highlights two cases of AITL presenting with unusual cutaneous findings: one with a medium-vessel vasculitis and another with a chronic urticarial eruption. Clinicians should consider AITL in the differential diagnosis of vasculitis or urticaria refractory to standard treatment.
Subject(s)
Lymphoma, T-Cell, Cutaneous/complications , Paraneoplastic Syndromes/pathology , Skin Neoplasms/complications , Skin/pathology , Urticaria/etiology , Vasculitis/etiology , Fatal Outcome , Female , Humans , Immunoblastic Lymphadenopathy/pathology , Lymphoma, T-Cell, Cutaneous/pathology , Middle Aged , Skin Neoplasms/pathology , Urticaria/pathology , Vasculitis/pathologyABSTRACT
Delusional infestation is a psychiatric condition defined by a fixed belief of infestation despite contrary evidence. Diagnosis includes exclusion of organic etiologies. Treatment with antipsychotics is effective and safe in the majority of patients. Patients are characteristically reluctant to pursue psychiatric evaluation and may resist discussing their disease in psychiatric terms. Strategies to strengthen the provider-patient therapeutic alliance facilitate communication around appropriate treatment. Without antipsychotic medications, patients can become heavy utilizers of care and practice self-destructive behaviors in attempts to clear their perceived infestation.