Derya Ufuk Altintaş

Congenital Mastocytosis Associated with Neurosensory Deafness

denitis. As mentioned earlier, few reports have provided photographs with the prototypic skin rash in KD (4–7). Cutaneous manifestations cannot replace lymph node biopsy for diagnosis because skin findings are delayed and lack specificity. However, identifying the rash as an extranodal feature of KD will prevent many from undergoing an unnecessary skin biopsy. We believe that our patient had the typical rash of KD, which resembles both urticaria and a generalized morbilliform eruption, and hope that his clinical photographs will function as a visual reference.

The Influence of Concomitant Disorders on Disease Severity of Familial Mediterranean Fever in Children

Objectives This study aims to describe the effects of concomitant disorders on the course of familial Mediterranean fever (FMF) and the relevance of genotype on these associations. Patients and methods Files of 494 FMF patients (257 males, 237 females; mean age 12.8±1.94 years; range 1.6 to 23 years) were retrospectively examined. Age of diagnosis, sex, MEditerrenean FeVer mutations, colchicine dosage, disease severity score and concomitant diseases in FMF course were recorded. FMF diagnoses were based on Tel-Hashomer criteria and disease severity was determined by international severity scoring system for FMF. Patients were divided into two groups as M694V positives and M694V negatives. We compared the groups in terms of accompanying illnesses, MEditerrenean FeVer mutations, and disease severity scores among five concomitant diseases: juvenile idiopathic arthritis (JIA), asthma, Henoch- Schonlein purpura, periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome, and others. Results The mean age at diagnosis was 8.7±1.9 years. Eighty-five patients (17.2%) had accompanying diseases including JIA, asthma, periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome, and Henoch-Schonlein purpura. Mean disease severity scores were 2.4±1.1 in patients with only FMF and 3.0±1.5 in patients with concomitant disorders (p=0.001). Patients with concomitant JIA showed the highest severity scores (4.3±1.6). A statistically significant difference was found with one-way analysis of variance. Conclusion Our findings indicate that concomitant diseases, particularly JIA, influence FMF severity. Therefore, it may be beneficial to focus on diagnosis and treatment of comorbid inflammatory diseases, which may worsen the course of FMF.