Nonspecific Skin Lesions Research Articles

Risk factors for chronic perforating skin lesions in the area of the digits in cattle on Swiss alpine pastures

Diseases of the digits often occur in cattle on larger cattle mountain pastures. In the late spring 2020, at the time of the ascent of 1554 cattle to 11 high altitude alpine pastures in the Lower Engadine region, lesions in the area of the digits were clinically assessed and documented. 254 cattle were of non-cantonal and 1300 of local origin (Lower Engadine; postal code CH-75XX). Skin lesions in the area of the digits, identified as digital dermatitis (DD; Mortellaro's disease), were further classified according to the DD scoring system. Nonspecific skin lesions with clinical evidence of granulation tissue formation were termed chronic penetrating skin lesions (CPSL). At the end of the alpine pasturing season, in the early fall (descent of cattle from the alpine pastures), the procedure was repeated, and biopsies were taken from randomly selected cattle with CPSL. Digital dermatitis lesions were found in 34 of 1551 cattle at ascent, but no case of CPSL was found at that time. At descent, 19 of 1529 cattle had DD lesions and 88 cattle had CPSL. The clinical appearance of the CPSL was consistent with chronic skin lesions caused by penetrating skin lacerations. Histologically, the majority of the CPSL were classified as chronic hyperplastic dermatitis with granulation tissue formation. In all CPSL biopsies examined by PCR, Fusobacterium necrophorum and Porphyromonas levii, but neither Dichelobacter nodosus nor the tested Treponema species were detected. Fluorescence in situ hybridization revealed a negative result for Treponema species in all biopsies. In the regression analysis, cattle in the age group of 365 to 730 days had an increased risk for the presence of CPSL compared to the age group of 160 to 365 days (odds ratio (OR) = 4,95; confidence interval (CI) = 1,97-12,43). Holstein cattle had an increased risk of developing CPSL compared to Brown cattle (OR = 2,92; CI = 1,46-5,86) and cattle of non-cantonal origin showed a massively higher risk compared to local cattle (OR = 10,59; CI = 5,79 - 19,37). The statistically significant associations found in the present study can be taken into account in the selection of animals for summer pasturing on high altitudes in the future in order to reduce the prevalence of CPSL and consequently reduce the antimicrobial use. Spread of DD during the alpine pasturing season within the cattle groups examined was not found.

Stirring the hornet’s nest

Hornets are widely distributed in India, and their stings manifest from non-specific skin lesions to fatal complications as a result of a myriad of proteins in the venom. We report a case of a 52-year-old man who was attacked by a swarm of hornets and succumbed four hours later in rural Davangere district. Forty sting marks were noted on autopsy. Histopathology revealed histiocytic infiltration surrounding sting marks and laryngeal subepithelial oedema. The cause of death was opined to be laryngeal oedema following anaphylaxis. We conclude that a focus on preventive strategies, public awareness programmes, and providing protective gear to the vulnerable is vital.

Cutaneous manifestations in patients with systemic lupus erythematosus: Forms at the beginning of the disease and during follow-up. Retrospective analysis

ObjectivesTo describe skin involvement (SI) in patients with systemic lupus erythematosus (SLE) at onset and during follow-up of the disease and to determine factors associated with SI at lupus diagnosis. Materials and methodsRetrospective, observational, and descriptive study, from a single centre in patients diagnosed with SLE (ACR 1982-97 or SLICC 2012 criteria). The modified Gilliam classification for SI was used. Descriptive statistics and bivariate and multivariate analysis were performed to evaluate the factors associated with SI at diagnosis of the disease. Results149 patients were included, 91.3% women with a median age at diagnosis of 33 years. SI at onset of the disease occurred in 125 patients (83.9%), followed by joint involvement in 120 cases (80.5%). Non-specific skin lesions were more frequent than specific lesions, 92.8% versus 66.4%, respectively. In the bivariate analysis, a longer delay to diagnosis, the presence of joint involvement, a lower presence of thrombocytopenia, and a higher SLEDAI-2K score were associated with the presence of SI at onset of the disease. In the multivariate analysis, the variable that remained independently associated was joint involvement (OR 2.8%–95% CI 1.1–7.5, p: .04). During follow-up, 4/24 patients who had not presented SI at diagnosis and 51/125 patients who had, had at least one new skin flare (range: 1–5 outbreaks). ConclusionsOur study demonstrates the high frequency of skin involvement in SLE, both diagnostically and evolutionarily, and confirms previously reported data regarding the existence of a skin-articular phenotype.

Difficulties in diagnosing ALK-negative anaplastic large cell lymphoma manifesting from a skin lesion

Anaplastic large cell lymphoma is a rare form of non-Hodgkin's lymphoma that requires an early diagnosis and urgent therapy due to aggressive tumor process. Clinically, most non-Hodgkin's lymphoma present with lymphoadenopathy and В-symptoms (such as weakness, drowsiness, fatigue, subfebrile fever, profuse night sweats). However, anaplastic large cell lymphoma may present with nonspecific skin lesions with minimal or no B-symptoms. The skin lesions are heterogeneous, which may also delay verification of the diagnosis.
 We present a case of widespread anaplastic large cell lymphoma skin lesions mimicking a case of erysipelas, which progressed rapidly from a single rash on the wrist to an extensive lesion of the right breast within 1 month. Prior to verification of the diagnosis, the patient was treated for erysipelas, pyoderma, and herpes zoster with no response to therapy. Immunohistochemical examination of a skin biopsy confirmed the diagnosis of ALK-negative anaplastic large cell lymphoma. At the time of publication, the patient had completed 1 course of the CHOEP regimen, and 6 cycles of the program were planned.
 Our case demonstrates the necessity of a broad differential diagnosis of rashes torpid to the current therapy.

Risk of Nephritis and Recurrence in Kimura Disease: A Retrospective Study.

Kimura disease (KD) presents a diagnostic challenge to clinicians because of its rarity and atypical symptoms in its early stages, and it is difficult to treat and prone to recurrence or involvement of other organs. This study aims to investigate the possible relevance of renal involvement and recurrence by analysing the clinical presentations, laboratory results, histopathological features, therapeutic data and follow-up results of KD. A total of 27 patients diagnosed as KD in two hospitals from January 1999 to December 2021 were analysed retrospectively in this study based on the diagnosis of histopathology. KD mainly affected male more than female (8:1) with the onset age ranging from 3 to 58 years (median 29.8 years). The common initial symptoms included subcutaneous soft tissue or lymph node enlargement, non-specific skin lesions and proteinuria. One patient presented cough and expectoration as the first symptoms. KD patients often had high levels of serum immunoglobulin E (IgE) and basophils, which exhibited a significantly positive correlation with renal involvement and recurrence (p < 0.05). Early mass resection could prevent the development of nephritis and decrease the risk of relapse (p < 0.05). KD should be noted in patients presenting with intractable and relapsing atopic skin lesions and (or) subcutaneous mass. Patients with high levels of serum IgE and blood basophils may be prone to developing KD-associated nephritis and predict a high risk of recurrence. Early surgical removal of the mass may result in a better prognosis.

Radiation Induced Angiosarcoma of the Breast: A Case Report

The incidence of women who receive radiotherapy after breast conserving surgery due to breast cancer is increasing and these patients have a longer life as a cure. Radiation treatment may cause second primary cancer within radiation field areas. Angiosarcoma is the secondary most common cancer in the field of radiotherapy after breast radiotherapy. Radiation-induced angisarcoma (RIAS) of the breast is a rare, poor prognosis disease and usually occurs as non-specific skin lesions within the radiotherapy field. RIAS, known as radiation-induced cancer, occurs approximately 7 years after radiation therapy and diagnosis of this disease is made by skin biopsy. This review aims to report a case of RIAS developed within irradiated field of breast cancer after radiotherapy 6.5 years later.

Hypereosinophilic Syndrome, Erythroderma as Clinical Debut. Case Report and Literature Review

Eosinophilia is a common finding in clinical practice, but when elevated values ​​of eosinophils are found, it constitutes a diagnostic challenge. Hypereosinophilic syndrome is a group of rare disorders defined by persistent blood hypereosinophilia &gt;1.5 × 109/l and associated to organ damage that result in a wide variety of clinical manifestations: fatigue with nonspecific skin lesions, to endomyocardial fibrosis, neurological compromise and life-threatening evolution. The prognosis of the disease is variable and depends on the variant and the availability of specific treatment. 1- 2 We present the clinical case of a patient with a history of B symptoms and the presence of persistent erythroderma. The patient underwent several complementary studies, including skin biopsy without presenting a definitive diagnosis. Laboratory with persistence of hypereosinophilia that guided us towards the diagnosis of Idiopathic Hypereosinophilic Syndrome.

Cutaneous Manifestations of Systemic Lupus Erythematosus

ABSTRACT Inflammation driven by immune-mediated mechanisms can lead to a wide variety of skin manifestations. Cutaneous lupus erythematosus (CLE), for example, is an autoimmune connective tissue disease that may be limited to the skin or be part of the widespread multiorgan involvement seen in systemic lupus erythematosus. Clinical findings are divided into lupus-specific (acute, subacute, and chronic) and nonspecific skin lesions. The diagnosis of CLE requires a thorough physical examination and, in some cases, skin biopsy and laboratory evaluation for any underlying systemic involvement. CLE treatment is dependent on the cutaneous manifestations and severity of disease. In addition to lifestyle measures, particularly avoidance of sunlight, topical and systemic therapies have proven beneficial. Prognosis is varied by disease severity, although chronic CLE notoriously results in cosmetic damage. Early recognition and management of CLE is important as it may also be the presenting manifestation of systemic lupus erythematosus. Patient care includes education and close collaboration with primary care providers, dermatologists, and rheumatologists.

Un cas de manifestation cutanée d’un syndrome hyperéosinophilique lymphoïde

IntroductionLymphoid hypereosinophilic syndrome (HES) is a reactive HES, related to the presence of an abnormal circulating T cell clone. Cutaneous manifestations are frequent and sometimes inaugural, however few studies describe them specifically. Case reportWe report the case of a 63-year old patient, in good general condition, with no previous history and taking no treatment, who was being followed for non-specific skin lesions. Blood and skin examinations showed hypereosinophilia, the presence of an aberrant CD3-CD4+ phenotype and a positive T-clonality test. There was no differential diagnosis or argument for a systemic lymphoma. ConclusionCutaneous manifestations of lymphoid HES are variable, non-specific, and may differ according to lymphocyte phenotype. The discovery of SHE requires an extension workup and the risk of evolution towards a systemic lymphoma justifies a close surveillance. Treatment is adapted to the severity of the symptoms.

Cutaneous manifestations of lupus erythematosus: a practical clinicopathological review for pathologists.

Accurate diagnosis of connective tissue diseases is often challenging, and relies upon careful correlation between clinical and histopathological features, direct immunofluorescence studies and laboratory work-up. Lupus erythematosus (LE) is a prototype of connective tissue disease with a variety of cutaneous and systemic manifestations. Microscopically, cutaneous LE is classically characterised by an interface dermatitis although other histopathological patterns also exist, depending upon the clinical presentation, location and chronicity of the skin lesions. In this article, we review the clinical, serological, histopathological and direct immunofluorescence findings in LE-specific and LE non-specific skin lesions, with an emphasis upon lesser-known variants, newly described features and helpful ancillary studies. This review will guide general pathologists and dermatopathologists in accurately diagnosing and subclassifying cutaneous LE.

Clinico-Pathological Profile of Cutaneous Lupus Erythematosus Patients: A Report from A Tertiary Care Center of Chattogram

Background : Nearly all epidemiologic studies have involved patients with Systemic Lupus Erythematosus (SLE). Few authors have investigated the characteristics of patients with Cutaneous Lupus Erythematosus (CLE). We aim to describe the epidemiologic, clinical, and immunologic characteristics of a series of patients diagnosed with CLE.&#x0D; Materials and methods : This is a descriptive retrospective cross-sectional study carried out using the register records of total 218 patients attending the ‘Lupus Clinic’ in Chittagong Medical College Hospital during the period 2010 and 2020. The disease activity and damage of CLE were assessed according to the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI).&#x0D; Results : There were 187 females (86%) and 31 males (14%) with the female: male ratio being 6.1:1. The mean age was 28 ± 10.06 (mean ± SD) ranging between 11 and 65 years. The Chronic Cutaneous Lupus Erythematosus (CCLE) patients accounted 154 (71%) followed by Acute Cutaneous Lupus Erythematosus (ACLE) 46 (21%) and Subacute Cutaneous Lupus Erythematosus (SCLE) 18 (8%). On the basis of CLASI score, 91 (42%) patients were in mild form, 85 (39%) in moderate form and 42 (19%) in severe state. In LE specific skin lesions, common manifestation was photosensitivity 198 (91%) followed by discoid rash 154 (71%) and maculo-papular lupus rash 55 (25%). Oral ulcer was seen in 49 (22%) patients and malar rash in 46 (21%) patients. Other observed LE specific skin manifestations were papulo squamous rash 11 (5%), Toxic epidermal necrolysis like lesions 7 (3%) and lichenoid lesions 6 (3%). Among LE nonspecific skin lesions, common manifestation was nonscarring alopecia 123 (56%) followed by Raynaud’s phenomenon 17 (8%) livedo reticularis 17 (8%) Vasculitis 15 (7%) Periungual telangiectasia 7 (3%) erythema multiforme 6 (3%) and leg Ulcer 5 (2%). Antinuclear Antibody (ANA) 132 (61%) was the most common autoantibody followed by anti-ds DNA 91 (42%) anti-Sm antibody 2 (1%) anti-phospholipid antibodies 9 (4%) and anti-RNP 3 (1%). Hematological manifestations were seen in 161 (73.85%) where erythrocyte sedimentation rate was the most common hematological abnormality 161(73.85%) followed by lymphopenia 126 (57.80%) leucopenia 113 (51.80%) thrombocytopenia 107 (49.10%) anemia 92 (42%) monocytopenia 37 (16.97%). Conclusions: CCLE was the most common subtypes of CLE. Patients with different subtypes of CLE have distinct clinical and pathological characteristics. The onset or concurrence of SLE mandates the involvement of other disciplines depending on organ involvement. In the absence of consensus on a definition that makes it possible to differentiate cutaneous forms of LE from SLE, the dermatologist’s role in the correct diagnosis and classification of such patients is fundamental.&#x0D; Chatt Maa Shi Hosp Med Coll J; Vol.20 (2); July 2021; Page 79-84

Primary Cutaneous Cryptococcosis of Nasal Ala with Extensive Tissue Destruction

Abstract Introduction/Objective Primary cutaneous cryptococcosis (PCC) is rare and shows skin lesion(s) confined to a circumscribed body region mostly in immunocompromised host, with no sign of simultaneous dissemination condition. PCC usually presents as non-specific skin lesions such as cellulitis, nodules, and ulcers, and can be misdiagnosed in biopsy. We present a case of PCC with extensive tissue destruction. Methods/Case Report A 43-year-old male, with a history of human immunodeficiency virus infection 17 years ago, presented with complaint of his nose slowly “being eaten away” over the past 5 years after a bike accident. Physical examination showed most of the left nasal ala was completely destroyed, with visualizable septum. The biopsy of the left nasal ala showed the dermis has numerous yeasts with marked variation in size and shape, in foamy stroma with little inflammation. The capsules of the yeasts were highlighted by Mucicarmine stain. Grocott methenamine silver stain showed budding yeasts. The diagnosis of cutaneous cryptococcosis, gelatinous type, was rendered. Primary cutaneous cryptococcosis was considered based on no disseminated disease found, positive serum cryptococcus antigen with low titer (1:20), the culture of the nasal lesion positive for Crptococcus Neoformans, and the history of skin injury. The patient received appropriate treatment for PCC subsequently. Results (if a Case Study enter NA) NA Conclusion We have demonstrated a very rare case of undiagnosed/untreated PCC causing extensive destruction of skin and underlying nasal tissue. Identification of the histological features of cutaneous cryptococcosis, shown in this case, is the key for making the correct diagnosis.

Nonspecific skin lesions in malignant lymphomas

Objective: Clinical characteristics of the peculiarities of non-specific skin lesions with patients having malignant lymphomas (ML).Methods: 130 patients having malignant lymphomas were observed, 50 patients – having Hodgkin’s lymphomas, 38 patients - B-cell lymphomas and 42 patients – T-cell lymphomas.Results: Six main types of nonspecific lesions are defined among patients having malignant lymphomas: 1) skin lesions of auto toxic genesis, 2) skin lesions determined with vascular pathology, 3) skin lesions due to hemolysis and hemostatic disorders, 4) skin lesions of those having cryoglobulinemia/cryofibrinogenemia, 5) skin lesions due to metabolic disorder, 6) infectious diseases.Conclusions: Lesions of auto toxic genesis and infectious diseases prevail among nonspecific skin changes when having malignant lymphomas. Patients having Hodgkin’s lymphomas experience dermal itching more often. Among B-cell lymphomas skin lesions are more pathognomonic for chronic lymphocytic leukemia, among T-cell lymphomas – for fungal mycosis. Nonspecific skin lesions, having arisen before typical clinical picture of malignant lymphomas allow to suspect and diagnose the main illness on time. Disappearance of skin lesions against the background of specific therapy can bear evidence to the efficiency of the malignant lymphomas’ treatment.

Cutaneous Manifestations of COVID-19: A Case Series from Brazil

Introduction: Brazil has presented alarming and growing data on infection and deaths from SARS-CoV-2. There are more than 4 500 000 confirmed cases and more than 137 000 deaths ranking second in the world ranking of infected people. In addition to the involvement of the respiratory, cardiovascular, renal and neurological systems, this virus also causes non-specific skin lesions in same patients. Little is known about the pathophysiology of cutaneous involvement. At this moment, we cannot predict a difference in prognosis based on skin lesions.&#x0D; Methods: We conducted an observational case study of 25 patients affected by SARS-CoV-2 who had dermatological lesions. We described the clinical and epidemiological characteristics of each case, demonstrating the polymorphism of the skin lesions and also making a correlation with the chronology of presented systemic symptoms.&#x0D; Results: Twenty five cases of COVID-19 with cutaneous manifestations (urticaria, erythematous rash, maculopapular eruption, pruritus, erythema multiforme-like lesions, dyshidrotic eczema) were observed during, after and before systemic symptoms. A few cases with skin involvement were also observed as isolated symptoms of the viral infection. These data demonstrate the clinical polymorphism related to skin involvement of patients infected with SARS-CoV-2, emphasizing the importance of clinical suspicion by dermatologists when handling suspected cases in the current epidemiological scenario.&#x0D; Conclusion: Although the number of cases in the world stage seems to be regressing, infection by SARS-CoV-2 will be part of the dermatologist's daily routine. As long as we do not have a widely available vaccine and the pandemic takes on an endemic profile, we need to be aware of these manifestations, not only for the proper diagnosis, indication of patient isolation, as well as all the necessary biosafety procedures in dermatology clinics.&#x0D; &#x0D;

Leukemia Cutis in a Patient with Rare Indolent T-Prolymphocytic Leukemia - A Case Report and Review of the Literature

Abstract Introduction/Objective T-cell prolymphocytic leukemia (T-PLL) is rare, comprising 2% of mature lymphocytic leukemias in adults. Clinical course is usually aggressive, however, 20-30% patients can have an indolent early phase. Most patients present with extensive disease with peripheral blood lymphocytosis, hepatosplenomegaly, and lymphadenopathy. T-PLL has a higher incidence in patients with ataxia-telangiectasia. Methods We present a case of a 67-year old man with history of somatic mutation of ataxia-telangiectasia gene (ATM) and multiple malignancies, including melanoma, skin and laryngeal squamous cell carcinomas, adrenal adenoma and spindle cell lung tumor (diagnosed on biopsy), who developed leukemia cutis during an indolent phase of T-PLL. Results Initial diagnosis of T-PLL was on routine complete blood count when patient had mild atypical lymphocytosis (5.5 x 109/L). Flow-cytometry immunophenotyping detected CD4 + 8 positive mature T-cells with no aberrant loss of T- cell antigens, and molecular studies confirmed clonal T-cell receptor beta and gamma gene rearrangements. T-PLL associated leukemia cutis was diagnosed on a leg skin nodule biopsy 10 months later. Subsequent bone marrow biopsy demonstrated low level involvement by T-PLL. The patient’s T-PLL followed an indolent trajectory for three years and treatment was withheld until symptomatic presentation. Rapid disease progression was heralded with emergence of widespread T-PLL leukemia cutis, rapidly increasing lymphocytosis, extensive lymphadenopathy, splenomegaly and increasing LDH levels. Conclusion Skin involvement is reportedly identified in 20% to 50% of T-PLL cases in some series. Skin manifestations can be heterogenous with a broad clinical differential. T-PLL associated leukemia cutis may not be recognized owing to the overall low incidence and in skin lesions appearing during an undiagnosed indolent early phase of T-PLL.We highlight the importance of accurate evaluation of non-specific skin lesions in early detection of T- PLL. Being aware of this diagnosis and early engagement in an interdisciplinary approach may allow for better outcomes of this aggressive malignancy.

Introducing a Case of Pyoderma Gangrenosum in the Breast

Introduction: Pyoderma gangrenosum (PG) of the breast is a rare that present as a painful ulcer on the skin. It usually affects people in their 20s to 50s and occurs in both men and women. Typically, PG affects the legs in adults. In children, it may affect the legs, buttocks, head, and neck. Pyoderma gangrenosum is characterized by a papule, nodule, or pustule that progresses to an injured lesion with unknown boundaries. In this study, a case of Pyoderma gangrenosum is introduced after breast surgery. A 38-year-old woman with a 3 cm wound in the right breast area that has gradually grown has been examined for exudative bloody discharge for the past 2 weeks. Two weeks after breast surgery, a three-centimeter progressive wound has formed on the surface of the breast, which gradually grew larger. During treatment, several oral and injectable antibiotics were prescribed that have not been effective in healing the wound. A biopsy lesion was reported in which a non-specific skin lesion with hyperplasia and vesicle formation without malignancy was reported. The patient had no gastrointestinal symptoms. Infliximab was started and continued for the patient. Conclusion: In the differential diagnosis of resistant skin wounds, especially in the leg area, and in this case in the breast the diagnosis of pyoderma gangrenosome should always be considered. Even if the patient has no history of inflammatory bowel disease, pyoderma gangrenosum may occur before intestinal manifestations.

The ambiguous pruritogenic role of interleukin-31 in cutaneous T-cell lymphomas in comparison to atopic dermatitis: a review.

Cutaneous T-cell lymphomas (CTCLs) comprise a group of chronic heterogeneous diseases of unknown pathogenesis, characterized by non-specific skin lesions such as patches, plaques and tumours. CTCL is accompanied by persistent pruritus poorly responding to antihistamines and therefore significantly reducing quality of life in patients with lymphomas. According to research data, interleukin-31 (IL-31) contributes to initiation and maintenance of the inflammatory process of the skin and pruritus in inflammatory dermatoses such as atopic dermatitis (AD), which is well established. The studies of a similar role of IL-31 in CTCLs are less homogenous. Due to contradictory reports concerning IL-31 and CTCL we have analysed available literature to summarize its role, focusing on CTCL and AD.

Dermatoscopy for the rapid diagnosis of Talaromyces marneffei infection: a case report

BackgroundTalaromyces marneffei is a thermally dimorphic fungus endemic in south-east Asia. It predominantly occurs in both immunocompromised and immunosuppressed patients and can be fatal if diagnosis and treatment are delayed. The clinical manifestations of T. marneffei infection are nonspecific and rapid diagnosis of T. marneffei infection remains challenging.Case presentationA 24-year-old man came to our outpatient department with the sign of common skin lesions. The lesions were cuticolor follicular papules with or without central umbilication, nodules and acne-like lesions, which are common in syringoma, steatocystoma multiplex and trichoepithelioma. A dermatoscopy examination was performed to differentiate these skin lesions. The dermatoscopic images revealed circular or quasi-circular whitish amorphous structure with a central brownish keratin plug, providing the diagnostic clues of T. marneffei infection. Therefore, a skin scrapings culture, skin biopsy and serological detection for human immunodeficiency virus (HIV) were performed. The final diagnosis of this patient was T. marneffei and HIV co-infection.ConclusionRapid diagnosis of T. marneffei infection is clinically challenging since presenting clinical manifestations are nonspecific with significant overlap with other common conditions. This case highlights that dermatoscopy is a promising tool for the rapid diagnosis of T. marneffei infection in patients with nonspecific skin lesions, assisting clinicians to avoid delayed diagnosis or misdiagnosis.

Sjögren's syndrome with juvenile onset

The study included all consequently patients, who hospitalized to the pediatric department of V.A. Nasonova Scientific and Research Institute of Rheumatology from January 2013 to December 2018, which verified the diagnosis of the SS. The diagnosis of SS was established in 30 patients, among whom there were only 5 (16.7%) boys, the ratio of boys and girls was 1:5. According to the results of the examination, the following diagnoses were verified: 4 - primary SS, 9 - systemic lupus erythematosus with SS, 10 - juvenile rheumatoid arthritis with SS, 3 - mixed connective tissue disease, 3 - overlap syndrome, 1 - systemic sclerosis with SS. The median age of rheumatic disease onset was 10.4 (7.0; 13.75) years. The median of disease duration at the time of SS verification - 3.0 (0.85; 4.4) years. Recurrent parotitis were observed in 8 patients. 24 pts had isolated involvement of salivary glands, 6 - combined with lacrimal glands. Sicca syndrome was occurred in 8 patients. All patients had systemic manifestations: constitutional abnormalities - 50%, polyarthritis - 83.3%, lymphadenopathy - 73.3%, cutaneous involvement - 60%, pulmonary involvement - 23.3%. Of the hematological disorders, leuko/lymphopenia was more often recorded - in 30%, polyclonal hypergammaglobulinemia - in 26.7% of patients. ANA were detected in all cases, anti-Ro antibodies - 60%, a positive rheumatoid factor - 56.7% of patients. The most common combination of immunological disorders was the presence of ANA, RF and anti-Ro antibodies (40% of patients). The treatment for each patient was justified by the main manifestations and activity of rheumatic disease: 66.7% received nonsteroidal anti - inflammatory drugs, 80% - glucocorticoids, 46.7% - methotrexate, 20% - azathioprine, 43.3% - hydroxychloroquine, 10% - mycophenolatis mofetilum, 3.4% - cyclophosphamide. 66.7% of patients received the treatment of Biologics. Early diagnosis of SS in children with rheumatic diseases significantly affects to the choice of treatment and prognosis. In children the SS has no clinical manifestations for a long time. Such symptoms as a hypergammaglobulinemia, positive RF without persistant arthritis, nonspecific skin lesions, recurrent parotid swelling may help to diagnosis of SS.

The evolving clinical picture of seronegative spondyloarthropathy based on the example of ankylosing spondylitis in a patient with primary diagnosis of the SAPHO syndrome – case presentation

Seronegative spondyloarthropathies are a group of diseases characterized by several clinical features such as inflammatory back pain, sacroiliac joint inflammation, and the presence of the HLA B27 antigen, which occurs more frequently than the general population. Non-specific bowel inflammation, skin lesions, particularly psoriasis or uveitis, are also typical in patients or their family members [1]. In many situations, clinical progression of spondyloarthropathies can be assessed by imaging of the sacroiliac joints [2, 3]. SAPHO syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis), is a rare disease, classified as seronegative spondyloarthropathy due to many typical clinical features for this group of diseases. A large variety of symptoms and atypical clinical picture of SAPHO syndrome causes significant diagnostic difficulties [3, 4]. Presented here is a case report of a 41-year-old male patient with a twenty-year history of the disease. Initially, his disease took the form of chronic, recurrent multifocal osteomyelitis (CRMO), which finally turned into a spondyloarthropathy that met the classification criteria for ankylosing spondylitis.