ABSTRACT
Cutaneous granulomas without detectable infectious etiology rarely occur in children and adults with primary immunodeficiency disorders. These cutaneous granulomas are primarily seen in combined variable immunodeficiency, ataxia-telangiectasia, and severe combined immunodeficiency (SCID) and can emulate the reaction patterns seen in sarcoidosis and granuloma annulare. To date, the literature has described only six cases of non-infectious cutaneous granulomas in SCID. We report an unusual case of cutaneous granuloma, mimicking a sarcoma, in a 40-year old male with recombinase activating gene 1-deficient SCID, who presented with a slow-growing globus mass over the lateral aspect of the right elbow. There was heterogeneous enhancement on MRI, which was concerning for neoplasm but no malignancy was found on frozen or permanent sections. GMS, PAS with diastase, and AFB stains, as well as microbiology cultures, were negative. An AE1/AE3 stain was negative and a CD163 stain highlighted histiocytes. No infectious etiology was identified and histopathology revealed palisaded granulomatous dermatitis, most closely resembling a rheumatoid nodule. Although cutaneous manifestations have been reported in nearly half of primary immunodeficiency disorder cases, non-infectious cutaneous granulomas are exceedingly rare in SCID. To our knowledge, this is the first case report of cutaneous palisaded granulomatous dermatitis mimicking a rheumatoid nodule in a major joint.
Subject(s)
Dermatitis , Granuloma , Homeodomain Proteins/genetics , Rheumatoid Nodule , Severe Combined Immunodeficiency , Adult , Dermatitis/genetics , Dermatitis/metabolism , Dermatitis/pathology , Granuloma/genetics , Granuloma/metabolism , Granuloma/pathology , Humans , Male , Rheumatoid Nodule/genetics , Rheumatoid Nodule/metabolism , Rheumatoid Nodule/pathology , Severe Combined Immunodeficiency/genetics , Severe Combined Immunodeficiency/metabolism , Severe Combined Immunodeficiency/pathologyABSTRACT
Chest physiotherapy (CP) is seen as a cornerstone of Cystic Fibrosis (CF) treatment. However, previous studies have suggested that adherence to CP is low. This study of adults with CF (N = 563) investigated CP adherence and associated factors. Only 29.5 per cent reported undertaking daily CP. Predictors of adherence included problems with fitting CP into lifestyle, a perception that CP does not help, physical consequences of CP, doing exercises instead and doing CP as and when necessary. These variables accounted for 45 per cent of the variance in adherence. Content analysis revealed a number of themes related to adherence to CP. Future studies should explore the benefits of daily CP and attempt to devise simple interventions to maximize adherence to CP.
