Background
Autosomal‐recessive congenital ichthyosis (ARCI) is a heterogeneous group of ichthyoses presenting at birth. Self‐improving congenital ichthyosis (SICI) is a subtype of ARCI and is diagnosed when skin condition improves remarkably (within years) after birth. So far, there are sparse data on SICI and quality of life (QoL) in this ARCI subtype. This study aims to further delineate the clinical spectrum of SICI as a rather unique subtype of ARCI.
Objectives
This prospective study included 78 patients (median age: 15 years) with ARCI who were subdivided in SICI (n = 18) and non‐SICI patients (nSICI, n = 60) by their ARCI phenotype.
Methods
Quality of life (QoL) was assessed using the (Children’s) Dermatology Life Quality Index. Statistical analysis was performed with chi‐squared and t‐Tests.
Results
The genetically confirmed SICI patients presented causative mutations in the following genes: ALOXE3 (8/16; 50.0%), ALOX12B (6/16; 37.5%), PNPLA1 (1/16; 6.3%) and CYP4F22 (1/16; 6.3%). Hypo‐/anhidrosis and insufficient vitamin D levels (<30 ng/mL) were often seen in SICI patients. Brachydactyly (a shortening of the 4th and 5th fingers) was statistically more frequent in SICI (P = 0.023) than in nSICI patients. A kink of the ear’s helix was seen in half of the SICI patients and tends to occur more frequently in patients with ALOX12B mutations (P = 0.005). QoL was less impaired in patients under the age of 16, regardless of ARCI type.
Conclusions
SICI is an underestimated, milder clinical variant of ARCI including distinct features such as brachydactyly and kinking of the ears. Clinical experts should be aware of these features when seeing neonates with a collodion membrane. SICI patients should be regularly checked for clinical parameters such as hypo‐/anhidrosis or vitamin D levels and monitored for changes in quality of life.
Background and objectives: Erysipelas, caused by beta-hemolytic streptococci, and limited cellulitis, frequently caused by Staphylococcus aureus or other bacteria, are skin and soft tissue infections characterized by typical clinical signs. However, despite the therapeutical relevance they are often not differentiated (e.g in clinical trials). Erysipelas are efficiently treated with penicillin, while limited cellulitis is treated with more wide-spectrum antibiotics. This study investigates whether parameters such as CRP, blood counts or novel parameters like immature granulocytes could serve as biomarkers to distinguish between these entities. Patients and methods: For this retrospective analysis 163 patients were included. We compared laboratory markers in patients with erysipelas (n = 68) to those with limited cellulitis (n = 41) of the leg. Both erysipelas and limited cellulitis were defined clinically, with an additional aspect for erysipelas being a prompt response to penicillin. Results: Erysipelas were characterized by higher levels of inflammation. CRP and leukocyte counts are the best parameters to discriminate between both infections. A CRP value ≥ 3.27 mg/dl indicated the diagnosis of erysipelas with 75 % sensitivity and 73.2 % specificity. Conclusions: Our results support the thesis that erysipelas and limited cellulitis are distinct infections as defined in the German guidelines and that an assessment of CRP and leukocytes is useful for differential diagnosis.
Clinical differentiation between superficial skin and soft tissue infections (SSTI, including cellulitis and erysipelas) and herpes zoster of the face can be difficult. Moreover, bacterial superinfection can develop in patients with herpes zoster. This may lead to delayed treatment or unnecessary use of antibiotics, which could lead to further microbial resistance or higher costs of treatment. This analysis of diagnostic parameters is often determined as standard of care in hospitalized patients and can help clinicians to guide their treatment decisions.Clinical differentiation between herpes zoster and bacterial superficial skin and soft tissue infections of the face can be difficult. In addition, diagnosis can be complicated by bacterial superinfection of lesional herpes zoster. The aim of this study was to determine whether inflammatory parameters, such as C-reactive protein (CRP) and blood counts, might be reliable biomarkers to distinguish between skin and soft tissue infections and herpes zoster when the face is infected. The study data (multivariate analysis and area under the curve) identified CRP (0.880) and leukocytes (0.730) together as the parameters that best discriminate between skin and soft tissue infections and herpes zoster. A CRP threshold ≥ 2.05 mg/dl indicated a diagnosis of skin and soft tissue infection with a sensitivity of 80% and specificity of 83.8%. For leukocytes ≥ 7.3×10 9 /l, diagnosis of skin and soft tissue infection had a sensitivity of 75% and specificity of 67.6%. Thus, when differential diagnosis is difficult, CRP and leukocytes should be determined, while parameters such as neutrophils or immature granulocytes do not add diagnostic value.
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