Risk factors for morphoea disease severity: a retrospective review of 114 paediatric patients.

BACKGROUND: Morphoea is a rare fibrosing disease of the skin and subcutaneous tissue with an unpredictable disease course, running the spectrum from mild skin involvement to severe disfigurement or extracutaneous complications. OBJECTIVES: Our objective was to describe the natural history of paediatric morphoea and determine patient variables that were associated with severe disease. PATIENTS AND METHODS: We conducted a retrospective chart review of patients with morphoea seen in one paediatric hospital system. Information about demographics, clinical characteristics, disease course and treatment were collected. Statistical analysis was performed using appropriate univariate tests and a multivariable model. RESULTS: One hundred and fourteen patients met study inclusion criteria. The female : male ratio was 2·6 : 1, and the median age of onset was 7 years old. There were 55 patients (48%) with linear morphoea, 38 patients (33%) with circumscribed morphoea, 12 patients (11%) with generalized morphoea, and nine patients (8%) with mixed morphoea. Neurological symptoms and joint involvement were present in 27 subjects (24%). Extracutaneous manifestations occurred in 38% of subjects with linear morphoea, compared with 15% with generalized morphoea and 3% with circumscribed morphoea (P = 0·0001). Thirty-six per cent of children with disease onset prior to 10 years of age and 5% of children with disease onset after 10 years of age had extracutaneous manifestations (P = 0·0002). Both linear morphoea and early-onset disease were significantly associated with extracutaneous involvement in a multivariable model. CONCLUSIONS: Children with linear morphoea and disease onset before 10 years of age should be monitored closely for extracutaneous manifestations and need early treatment with systemic medications to prevent disease complications.

Demographic and clinical characteristics of cutaneous lupus erythematosus at a paediatric dermatology referral centre.

BACKGROUND: Paediatric cutaneous lupus erythematosus (CLE) is uncommon and inadequately described in the literature. Similar to adults, children with CLE develop LE-specific and/or LE-nonspecific skin findings. Similarities and differences in demographics and clinical course between paediatric and adult CLE have not been sufficiently described. OBJECTIVES: To detail the demographic and clinical features of paediatric CLE and compare these findings with those reported in the adult literature. METHODS: A retrospective chart review was performed of 53 children seen in a paediatric dermatology clinic with cutaneous manifestations of LE. RESULTS: Patients presented with all five major subtypes of CLE, with some notable differences from adult CLE and previously published reports of paediatric CLE. Progression from discoid LE to systemic LE (SLE) did not occur in our cohort. Patients with subacute CLE were more likely than adults to have lesions below the waist as well as concomitant SLE. Sex distribution for CLE in our study was equal prior to puberty and female predominant in post-pubertal patients. CONCLUSIONS: Children with CLE have variable clinical presentations and progression to SLE that may be different from adult disease. Specifically, children with acute and subacute CLE may be more likely than adults to have systemic disease; therefore, patients with these subtypes should be monitored closely for evidence of SLE. Study limitations included small patient numbers that may limit the ability to generalize these data and relatively short follow-up intervals.

Cutaneous Crohn's disease mimicking Melkersson-Rosenthal syndrome: treatment with methotrexate.

A woman with a 5-year history of unilateral orofacial granulomatosis required repeated evaluations (including sequential colonoscopies) to establish the diagnosis of cutaneous Crohn's disease, a condition that proved responsive to low doses of oral methotrexate administered weekly. To our knowledge this is the first report describing the use of methotrexate for treatment of orofacial granulomatosis caused by underlying Crohn's disease.

Surgical resection of metastatic renal cell carcinoma: The University of Iowa experience.

The utility of surgical resection of solitary metastatic sites in renal cell carcinoma remains controversial. Additionally, the small literature detailing the role of surgical management suggests that patients who have surgical resection of metachronous metastases have a better outcome than those presenting synchronously. We reviewed the medical records of all patients with metastatic renal cell carcinoma who underwent nephrectomy at the University of Iowa Hospitals and Clinics between 1980 and 1993. Patients who had undergone surgical resection of metastatic disease, either at presentation or subsequent to their nephrectomy, were identified. Clinical parameters, time to treatment failure, and survival was evaluated. Eighteen patients underwent surgical resection of metastases, 7 were synchronous to their nephrectomy, and 11 developed metachronous metastases. Resected lesions in both groups included metastases to lung, bone, liver, brain, and soft tissue. The median survival of all patients from time of resection to death or last follow-up was 5.7 years (range, 2 days to 10.7+ years). Two patients remain alive, both with recurrent disease at 5.3 and 10.7 years. Mean time from nephrectomy to death was 2.69 years for the synchronous group and 5.97 years for the metachronous group (p = 0.0599). The role of surgical resection in metastatic renal cell carcinoma remains unproven. The survival of this population is significantly longer than that typical for the disease. In our experience there is no difference in time to treatment failure or survival between synchronous and metachronous resection of metastatic disease.