Subject(s)
Keratinocytes/immunology , Molecular Biology , Molecular Targeted Therapy/methods , Nevus, Sebaceous of Jadassohn/genetics , Nevus, Sebaceous of Jadassohn/therapy , Biopsy , CARD Signaling Adaptor Proteins/genetics , Case-Control Studies , Child , Child, Preschool , Connexin 43/genetics , Female , Guanylate Cyclase/genetics , Humans , Infant , Inflammation , Keratinocytes/metabolism , Male , Membrane Proteins/genetics , Mosaicism , Mutation, Missense , Treatment OutcomeABSTRACT
We report the case of a 10-month-old girl who presented with a spontaneous ulcer on the left buttock which failed to heal despite antibiotic therapy. Histology showed changes consistent with pyoderma gangrenosum and the ulcer resolved rapidly with super-potent topical steroids under occlusion. Blood tests revealed a persistent neutropenia. Immunoglobulin G (IgG) antineutrophil antibodies were detected in the serum, directed against human neutrophil antigen (HNA)-1a. Bone marrow studies showed normocellular marrow with no evidence of dysplasia. T and B cell subsets and karyotype analysis were normal. Autoimmune neutropenia is an uncommon self-limiting condition in young children. Pyoderma gangrenosum is rare in infants, although the buttocks are a common site of involvement in this age group. Pyoderma gangrenosum in infancy can be associated with systemic disease as in adults, particularly myelodysplasia and leukemia, arthritis and inflammatory bowel disease. However, the association of pyoderma gangrenosum and autoimmune neutropenia of infancy has not previously been reported.
Subject(s)
Autoimmune Diseases/complications , Neutropenia/complications , Pyoderma Gangrenosum/complications , Administration, Topical , Antibody Specificity , Autoantibodies/blood , Autoimmune Diseases/immunology , Buttocks , Clobetasol/administration & dosage , Female , Granulocytes/immunology , Humans , Immunoglobulin G/blood , Infant , Isoantigens/immunology , Neutropenia/immunology , Neutrophils/immunology , Pyoderma Gangrenosum/drug therapy , Recurrence , Steroids/administration & dosageABSTRACT
BACKGROUND: There is wide variation in the objective visual variables used to measure atopic eczema severity in clinical trials, making comparison and interpretation of results difficult. OBJECTIVE: To provide a rationale for simplifying and standardizing objective atopic eczema scoring by investigating which visual variables provide the best measure of disease severity from the patient's perspective. SETTING: The dermatology outpatient department at the Queen's Medical Centre, University Hospital in Nottingham, and 5 local general practices. PATIENTS: One hundred eighty individuals with atopic eczema. INTERVENTIONS: Clinical examination with scoring of 7 clinical signs and disease extent, followed by regression analyses of visual variable scores against a patient-rated measure of current disease severity. RESULTS: Objective measurements account for only a quarter of the variation in patient-rated disease severity. Three clinical signs were independent predictors of patient-rated disease severity: excoriations, erythema, and edema/papulation. Disease extent measurements do not reflect patient-rated disease severity in a linear manner, with mean severity scores increasing little above 30% body surface area involvement. CONCLUSIONS: From the patient's perspective, the measurement of 3 clinical signs-excoriations, erythema, and edema/papulation-provides as much information about current atopic eczema severity as more complex scoring systems that measure multiple clinical signs and disease extent. The simplicity of the Three Item Severity score, a previously published atopic eczema score based on measurement of these 3 clinical signs, makes it a suitable tool for research studies or clinical practice.
Subject(s)
Dermatitis, Atopic/diagnosis , Adolescent , Adult , Aged , Attitude to Health , Child , Child, Preschool , Dermatitis, Atopic/pathology , Female , Humans , Infant , Male , Middle Aged , Quality of Life , Severity of Illness IndexABSTRACT
OBJECTIVE: To develop a simple, valid, repeatable, and readily understandable patient-oriented assessment measure for monitoring disease activity in children and adults with atopic eczema. DESIGN: Qualitative semistructured patient interviews identified a list of symptoms of atopic eczema. These symptoms were quantitatively analyzed in a larger patient population to identify which symptoms were important to patients and amenable to monitoring as part of a scoring system. SETTING: The outpatient Department of Dermatology at the Queen's Medical Centre, University Hospital, Nottingham, England, and 5 local general practices. PATIENTS: Four hundred thirty-five patients with atopic eczema. RESULTS: Seven symptoms were incorporated into the final patient-oriented eczema measure using a simple 5-point scale of frequency of occurrence during the previous week, with a maximum total score of 28. Validity testing against the Dermatology Life Quality Index, Children's Dermatology Life Quality Index, and patients' global severity assessments showed good correlation (r = 0.78, r = 0.73, and r = 0.81, respectively; P<.001). Internal consistency was high (Cronbach alpha = 0.88), and test-retest reliability was good, with 95% of scores falling within 2.6 points on repeat testing (mean score difference, 0.04; SD, 1.32). Individual variables in the measure demonstrated sensitivity to change during a 4-week in-clinic period and an 18-week randomized controlled clinical trial. CONCLUSION: The patient-oriented eczema measure is a practical self-assessed measurement tool for monitoring aspects of atopic eczema that are important to patients in routine clinical practice or in the clinical trial setting.
