-
JAMA Surgery May 2022Endovenous thermal ablations (ETAs) are recommended as first-line treatment for truncal vein reflux, have a short recovery time, and are cost-effective. However, ETAs... (Randomized Controlled Trial)
Randomized Controlled Trial
Pain Outcomes Following Mechanochemical Ablation vs Cyanoacrylate Adhesive for the Treatment of Primary Truncal Saphenous Vein Incompetence: The MOCCA Randomized Clinical Trial.
IMPORTANCE
Endovenous thermal ablations (ETAs) are recommended as first-line treatment for truncal vein reflux, have a short recovery time, and are cost-effective. However, ETAs are associated with discomfort during tumescent anesthesia infiltration. To minimize discomfort, nonthermal, nontumescent ablation techniques had emerged in the form of mechanochemical ablation (MOCA) and cyanoacrylate adhesive injection (CAE).
OBJECTIVE
To assess pain scores immediately after truncal ablation using a 100-mm visual analog scale and 10-point number scale to compare pain-related outcomes following mechanochemical ablation vs cyanoacrylate adhesive treatment.
DESIGN, SETTING, AND PARTICIPANTS
The Multicenter Randomized II Clinical Trial Comparing Mechanochemical Ablation vs Cyanoacrylate Adhesive for the Treatment of Primary Truncal Saphenous Veins Incompetence study was a prospective multicenter randomized clinical trial conducted at 3 sites between November 2017 and January 2020. Inclusion criteria were primary great or small saphenous varicose veins; exclusion criteria included recurrent varicose veins, current deep venous thrombosis, or serious arterial disease. There were 392 participants screened, 225 were excluded, and 167 participants underwent randomization. Four participants did not receive allocated intervention and were included in the intention-to-treat analysis. Follow-up took place at 2 weeks, and 3, 6, and 12 months.
INTERVENTIONS
Patients with primary truncal vein incompetence were randomized to receive either MOCA or CAE.
MAIN OUTCOMES AND MEASURES
The primary outcome measure was pain score immediately after completing truncal ablation using a 100-mm visual analog scale (VAS) and a 10-point number scale. Secondary outcome measures included degree of ecchymosis, occlusion rates, clinical severity, and generic and disease-specific quality of life (QoL) scores.
RESULTS
Of 167 study participants, 99 (59.3%) were women, and the mean (SD) age was 56 (15.8) years. Overall, 155 truncal veins treated (92.8%) were great saphenous veins. Demographic data and baseline status were comparable between treatment groups. A total of 73 patients (47%) underwent adjunctive treatment of varicosities. Overall median (IQR) maximum pain score after truncal treatment was 23 mm (10-44) on the VAS and 3 (2-5) on the number scale, showing no significant difference in median (IQR) pain measured by VAS (MOCA, 24 [11.5-44.7] mm vs CAE, 20 [9.0-42.0] mm; P = .23) or by number scale (MOCA, 4 [2-5] vs CAE, 3 [2-4]; P = .18). Both groups demonstrated significant and comparable improvement in clinical severity, generic and disease-specific QoL scores, and complete occlusion rates. Four patients treated with CAE developed minor complications (superficial thrombophlebitis and thrombus extensions).
CONCLUSIONS AND RELEVANCE
To our knowledge, this was the first randomized clinical trial directly comparing nontumescent ablation techniques. The study demonstrated that the MOCA and CAE techniques have a similar periprocedural pain score.
TRIAL REGISTRATION
ClinicalTrials.gov Identifier: NCT03392753.
Topics: Adhesives; Cyanoacrylates; Female; Humans; Male; Middle Aged; Pain; Prospective Studies; Quality of Life; Saphenous Vein; Treatment Outcome; Varicose Veins; Venous Insufficiency
PubMed: 35385061
DOI: 10.1001/jamasurg.2022.0298 -
Journal of Vascular Surgery. Venous and... Nov 2020Insurance approval for saphenous vein ablation is generally limited to junctional reflux involving the great saphenous vein (GSV) or small saphenous vein. This study was... (Comparative Study)
Comparative Study
BACKGROUND
Insurance approval for saphenous vein ablation is generally limited to junctional reflux involving the great saphenous vein (GSV) or small saphenous vein. This study was designed to investigate prevalence and disease severity of anterior accessory GSV (AAGSV) compared with GSV disease in patients presenting to dedicated outpatient vein centers.
METHODS
Deidentified data were pulled from the American Vein & Lymphatic Society PRO Venous Registry for first and second patient encounters. Variables included age, sex, and body mass index (BMI); clinical class of Clinical, Etiology, Anatomy, and Pathophysiology (CEAP) classification; revised Venous Clinical Severity Score (rVCSS); and duplex ultrasound values for each limb. Data were further scrutinized according to duplex ultrasound findings. Patients with normal findings on duplex ultrasound examination or evidence of acute or chronic thrombosis were excluded. Patients were further characterized into two groups. The primary group had no prior vein treatment vs the progressive group, in which patients have had a superficial venous intervention at some point in the past.
RESULTS
There were 6836 unique patients with duplex ultrasound patterns of either AAGSV or GSV above the knee or both AAGSV and GSV in either group. This pool contained 2604 patients with recorded CEAP class and rVCSS, representing 2664 patient limbs in the final data set. In comparison to limbs in the progressive group, AAGSV reflux was more common in the primary group (78/563 vs 209/2101; P < .01). Demographic features of the groups demonstrated no significant difference. The primary group demonstrated a significantly higher rVCSS (6.95 vs 6.10; P < .01) than the progressive group. Patients in the primary group also demonstrated a significantly higher incidence of more advanced disease (CEAP class C4 and above; 43.1% vs 24.8%; P < .0001) than those in the progressive group. The primary group demonstrated no significant differences in age, sex proportions, or mean BMI. The mean rVCSS for GSV of these patients (7.22) was significantly higher than that of AAGSV patients (5.63; P < .01). The incidence of superficial vein thrombosis for the AAGSV patients (6.41%) was significantly higher than that of the GSV patients (2.17%; P < .05) in the progressive group. Patients in the progressive group demonstrated no significant difference in age, sex proportions, mean BMI, or average rVCSS. The proportion of AAGSV limbs with superficial thrombosis events (37/287 [12.9%]) was significantly higher than that for GSV (59/2214 [2.7%]; P < .01).
CONCLUSIONS
AAGSV reflux is common and carries similar morbidity to GSV reflux. It is manifested with an alarming presence of superficial vein thrombosis.
Topics: Ambulatory Care; Disease Progression; Female; Humans; Incidence; Male; Middle Aged; Prevalence; Registries; Retrospective Studies; Saphenous Vein; Severity of Illness Index; Thrombophlebitis; Ultrasonography, Doppler, Duplex; Venous Insufficiency; Venous Thrombosis
PubMed: 32205127
DOI: 10.1016/j.jvsv.2020.02.010 -
Journal of Medical Case Reports Apr 2021Mondor's disease of the breast (MDB) is a rare and benign disorder of the breast. It is characterized by thrombophlebitis of the superficial veins of the chest wall....
BACKGROUND
Mondor's disease of the breast (MDB) is a rare and benign disorder of the breast. It is characterized by thrombophlebitis of the superficial veins of the chest wall. Clinically, it manifests as a cord-like induration of the breast area. MDB resolves spontaneously without sequela.
CASE PRESENTATION
We report cases of three Caucasian African patients aged 29, 40 and 34, respectively. One patient was under progestative contraception. All the patients had a cord-like induration on the chest wall. Ultrasonography was performed in all patients and was normal in two cases and showed a thrombotic vein in one case. All the patients had symptomatic treatment with total resolution of symptoms within 1 to 4 weeks. No relapse was observed.
CONCLUSION
MDB is benign in most cases. However, it is not to be taken lightly, because it can be the manifestation of an underlying disease such as breast cancer. The diagnosis is based on clinical findings; ultrasonography can be helpful for the diagnosis. Treatment is based on analgesic and anti-inflammatory drugs.
Topics: Adult; Breast; Breast Diseases; Humans; Neoplasm Recurrence, Local; Thrombophlebitis; Ultrasonography
PubMed: 33810810
DOI: 10.1186/s13256-021-02708-6 -
Stem Cells International 2021Vascular adventitia contains progenitor cells and is shown to participate in vascular remolding. Progenitor cells are recruited into the venous thrombi in mice to...
BACKGROUND
Vascular adventitia contains progenitor cells and is shown to participate in vascular remolding. Progenitor cells are recruited into the venous thrombi in mice to promote neovascularization. We hypothesized that the adventitial progenitor cells of human great saphenous vein (HGSV-AdPC) enhance the resolution of venous thrombosis via neovascularization.
METHODS
Human great saphenous vein (HGSV) was harvested from the patients with great saphenous vein varicose and sectioned for immunohistochemistry, or minced for progenitor cell primary culture, or placed in sodium dodecyl sulfate solution for decellularization. Human venous thrombi were collected from patients with great saphenous vein varicose and superficial thrombophlebitis. Infrarenal abdominal aorta of New Zealand white rabbits was replaced with interposing decellularized vessel, and the patency of the grafts was confirmed by ultrasonic examination. Animal venous thrombi in the left infrarenal vena cava of mice were produced with Prolene suture ligation and ophthalmic force clipping of this portion. After HGSVs were digested by collagenase, the CD34CD117 HGSV-AdPC were isolated on FACS system, labelled with CM-Dil, and transplanted into the adventitia of infrarenal vena cava of nude mice. The percentage of thrombus organization area to the thrombus area was calculated as the organization rate. The thrombus cell, endothelial cells, and macrophages in the thrombi were counted in sections. Cell smears and frozen sections of human saphenous veins and venous thrombi were labeled with Sca1, CD34, CD117, Flk1, CD31, and F4/80 antibodies. The CD34CD117 HGSV-AdPC were cultured in endothelial growth medium with vascular endothelial growth factor (VEGF) to induce endothelial cell differentiation and analyzed with real time-PCR, Western blotting, and tube formation assays.
RESULTS
Immunohistochemical staining showed that the CD34CD117 cells were located within the adventitia of HGSVs, and many CD34 and CD117 cells have emerged in the human venous thrombi. The number of progenitor cells within the marginal area of 7 days mice thrombi was shown to be Sca1 ≈21%, CD34 ≈12%, CD117 ≈9%, and Flk1 ≈5%. Many CD34adventitial progenitor cells have migrated into the decellularized vessels. FACS showed that the number of CD34CD117 HGSV-AdPC in primary cultured cells as 1.2 ± 0.07%. After CD34CD117HGSV-AdPC were transplanted into the adventitia of nude mice vena cava with venous thrombi, the organization rate, nucleate cell count, endothelial cells, and macrophage cells of thrombi were shown to be significantly increased. The transplanted CD34CD117 HGSV-AdPC at the adventitia have crossed the vein wall, entered the venous thrombi, and differentiated into endothelial cells. The CD34CD117 HGSV-AdPC in the culture medium in the presence of VEGF-promoted gene and protein expression of endothelial cell markers and induced tube formation.
CONCLUSIONS
HGSV-AdPC could cross the vein wall and migrate from the adventitia into the venous thrombi. Increased HGSV-AdPC in the adventitia has enhanced the resolution of venous thrombi via differentiating into endothelial cells of neovascularization.
PubMed: 33679991
DOI: 10.1155/2021/8816763 -
Journal of Ultrasonography Sep 2014This article has been prepared on the basis of the Ultrasonography Standards of the Polish Ultrasound Society (2011) and updated based on the latest findings and... (Review)
Review
This article has been prepared on the basis of the Ultrasonography Standards of the Polish Ultrasound Society (2011) and updated based on the latest findings and reports. Ultrasound examination of the lower extremity veins is relatively easy and commonly used to confirm or rule out venous thrombosis. However, a relatively easy compression test frequently requires experience, particularly in situations when imaging is difficult (due to lymphedema, dressing or thick tissues). The technique is time-consuming and requires assessment of each deep vein every 1 cm. Lesions in the deep veins cannot be ruled out when the vessels are assessed in only 2-3 points - a full examination is needed. The value of the method is the highest when the proximal section is assessed and the lowest when crural veins are evaluated. Doppler sonography is the basic method used when patients are prepared for a surgery of varicose veins. The assessment of the superficial veins prior to this procedure is tedious and requires knowledge of anatomy together with numerous variants. A considerable challenge is posed by re-assessment of recurrent varicose veins following a previous surgery. The Standards include anatomic nomenclature proposed by the Polish Society for Vascular Surgery and Polish Society of Phlebology, which should facilitate communication with clinicians. The most beneficial patient positions have been thoroughly discussed in terms of safety and effectiveness of the examination. Sometimes during such an examination, no venous pathology is found, but other changes with symptoms that suggest deep thrombophlebitis are detected. In such a situation, it is necessary to conduct an initial (or complete, if possible) assessment of lesions as well as provide recommendations connected with further, more detailed diagnosis.
PubMed: 26675992
DOI: 10.15557/JoU.2014.0029 -
Acta Dermatovenerologica Croatica : ADC Dec 2023Dear Editor, Ticks carry many diseases, bacteria, and viruses and represent a very important healthcare issue both in Croatia and globally. Although most ticks are not...
Dear Editor, Ticks carry many diseases, bacteria, and viruses and represent a very important healthcare issue both in Croatia and globally. Although most ticks are not infected with pathogens dangerous to humans, some ticks can transmit infectious diseases with significant morbidity and mortality. This is caused by the increasing incidence of many tick-borne diseases over a growing geographical area. Many factors influence which species of ticks are present in a given geographical area, as well as the density of their population and the risk of human exposure to infected ticks. The average morbidity from Lyme borreliosis in the Republic of Croatia is 6.51 infected per 100,000 inhabitants. There can be no Lyme borreliosis without ticks infected by Borrelia burgdorferi (1,2). In Europe, Lyme borreliosis (LB) is caused by the Borrelia burgdorferi sensu lato complex genotype. There are three skin manifestations of LB: erythema migrans (EM), borrelial lymphocytoma (BL), and acrodermatitis chronica atrophicans (ACA) (3,4). Herein we describe a female patient with a diagnosis of Lyme disease based on the non-specific clinical picture and laboratory diagnostics, in whom successful treatment led to complete regression of all skin manifestations. The patient was a 58-year-old woman with no previous history of severe illness. Notably, the patient history showed that, eight months prior to presenting for the dermatological exam, the patient had observed the appearance of edema and demarcated macular exanthema around both ankles and subsequently on the dorsum of the right hand, which spread to the left hand and with gradual spread to both lower legs and the lower extremities, with more pronounced changes on the left leg. The initial dermatological examination found pronounced skin changes on both legs, especially the left leg, with erythematous changes in the form of figurate erythema forming confluences up to the size of a smaller palm; the skin of the left leg was partially mottled with normal turgor and elasticity (Figure 1a and Figure 1b). Inguinal lymph nodes were enlarged and painless on palpation. Changes were minimal and discrete on the right leg and were absent on the torso, upper extremities, and skin. Subjectively, there was no itching, burning, or tingling sensation in the affected areas of the skin. The patient subjectively reported feeling well. Family history showed that the patient's father had died from prostate cancer and that the mother had died from melanoma. Laboratory findings were as follows: hematological, biochemical, and immunological parameters were normal. Venous and arterial ultrasound of both legs was normal, with the presence of reactively enlarged left inguinal lymph nodes. Lyme disease was suspected based on the clinical picture, with a differential diagnosis of possible livedo reticularis. A biopsy of the skin changes was also performed, with the results showing that the histological picture in the examined material could be compatible with the provisional clinical diagnosis of livedo reticularis. IgM and IgG specific for Borrelia burgdorferi was also performed: IgG was borderline, whereas IgM was positive at 218 U/mL. Over the next 3 weeks, Amoxil 500 mg thrice daily was introduced to the treatment. After completion of the treatment, there was a gradual regression of all skin changes without the appearance of new lesions (Figure 2a and Figure 2b) (Figure 3a and Figure 3b). Patient follow-up over the next year did not find any recurrence of similar skin changes. Herein we have described the case of a patient with atypical skin changes in which the presence of antibodies for Borrelia burgdorferi was demonstrated, in which regression of all skin manifestations was achieved after diagnosis and adequate antibiotic treatment. Lyme disease has a wide spectrum of clinical manifestations that can generally be observed in three stages: the early localized stage, the early disseminated stage, and the late stage of the disease. However, it is also possible for the different stages to overlap and even for the late stage to manifest without any signs and symptoms of the earlier stages. Early localized stage. Characterized by skin changes - erythema migrans (EM) - usually manifests within a month of the tick bite (usually 7-14 days after the bite) (Figure 4 and Figure 5). EM manifests in approximately 80% of patients, but only 25% of patients can recall the tick bite. The skin changes are usually localized in the axilla, the groin, the cubital area, or around the waist. The changes are generally not painful, but can itch or be warm to the touch. They gradually spread over days or weeks and can grow to a radius of up to 20 cm. Initially, the coloration can be uniform for several days, after which the redness disappears around a central zone (4-6). Multiple skin changes are a sign of spirochetemia and not the result of multiple tick bites. Due to timely antimicrobial treatment, multiple skin changes are much rarer today. In the initial days or weeks after infection, patients with early, localized, or disseminated Lyme disease often present with non-specific signs and symptoms resembling a viral infection: fatigue, headache, loss of appetite, joint pain, and regional lymphadenopathy. Fever can be present in approximately 20% of patients. Laboratory findings in this phase are non-specific. Erythrocyte sedimentation can be slightly increased, leukocyte counts are mostly normal, and anemia and thrombocytopenia are present only rarely (7,8). Early disseminated stage. This stage is marked by numerous EM lesions (that generally appear days or weeks after the infection) and/or neurological and/or cardiac manifestations (occurring weeks or months after infection). Some of these patients have no data on the presence of early localized Lyme disease. The most common triad of neurological manifestations are meningitis, neuropathy (usually of the facial nerve) and motor or sensory radiculopathy (Bannwarth syndrome). All these manifestations can appear individually. Cranial nerve neuropathies can often be bilateral. Late-stage Lyme disease. Characterized by intermittent or permanent arthritis in one joint or several large joints, most commonly the knees, and/or more rarely by neurological symptoms such as discrete encephalopathy or polyneuropathy. Late-stage Lyme disease can develop several years after primary infection, and arthritis can be the first manifestation of the disease, with the early localized and early disseminated stages not manifesting at all. In Europe, patients with late-stage Lyme disease can present with chronic skin changes (acrodermatitis chronica atrophicans), which is not observed in the USA. It is caused by B. afzelii and is typically localized to the extensor surfaces of the hands and feet. It is most common in women >40 years of age but can also present in younger populations. However, due to early antimicrobial treatment of the earlier stages of the disease, late-stage manifestations are rare (9). The discovery of the etiology of this disease showed that some well-known clinical entities were also a manifestation of Borrelia infection. The etiology of other dermatologic diseases was thus determined, such as lymphocytoma (or lymphadenosis cutis benigna), which was recognized as an entity as early as 1884, as well as acrodermatitis chronica atrophicans, described in 1888, erythema chronicum migrans (Afzelius-Lipschütz), and the neurological disease called Bannwarth syndrome, the symptoms of which were described as early as 1922 (10,11). LB and all its dermatological manifestations occur in almost all European countries, predominantly in the central part of the continent. The annual incidence is between 9.4 cases per 100,000 inhabitants in France to 120 cases per 100,000 inhabitants in northeastern Poland, 130 cases per 100,000 inhabitants in Austria, and 155 cases per 100,000 inhabitants in Slovenia (12). The total prevalence of ACA in all European patients with LB is 1-10%, depending on the region. For example, BL and ACA comprise 0.3% of LB cases in Bulgaria. In Norway, ACA comprises 5% of all clinical LB cases, and in northern Italy that number is 2.5%. Establishing a diagnosis of ACA is much more difficult than diagnosing EM or benign lymphocytoma (BL) because the clinical manifestations of ACA can vary. Acrodermatitis chronica atrophicans is probably the most common late and chronic manifestation of LB that can be observed in European patients. The skin changes in our patient were fairly non-specific, based on descriptions from the literature, but positivity for IgM antibodies was important for establishing the diagnosis, along with the very good response to antibiotics regarding regression of skin changes as well as the histological analysis that, according to the pathohistological diagnosis, indicated livedo reticularis, which is in turn also described in the literature as a possible form of ACA depending on the stage of the disease. Skin changes on the lower extremities are often incorrectly interpreted as vascular insufficiency, e.g. chronic venous insufficiency, superficial thrombophlebitis, hypostatic eczema, obliterative arterial disease, acrocyanosis, livedo reticularis, or lymphoedema, but they can also be the result of ACA, as in our case (13-15). In cases such as the one we have described, clinical manifestations of Lyme disease can very often vary and differ greatly from the typical clinical picture. This is demonstrated by our case, which also shows that LB and its idiosyncratic manifestations can lead physicians astray in a condition where failing to establish a timely diagnosis can be fatal for the patient. This case report also serves as a reminder that Lyme disease should be considered whenever atypical skin changes are encountered. Given that ACA is a disease in the late stage of Lyme disease and that the changes in our patient were noticed at the very beginning, the disease did not develop to the later stage.
Topics: Humans; Lyme Disease; Female; Middle Aged
PubMed: 38651851
DOI: No ID Found -
European Journal of Case Reports in... 2022Penile Mondor's disease is a rare condition characterised by superficial thrombophlebitis of the penis which is usually self-limiting. The cause is often unknown. The...
UNLABELLED
Penile Mondor's disease is a rare condition characterised by superficial thrombophlebitis of the penis which is usually self-limiting. The cause is often unknown. The AstraZeneca ChAdOx1-S vaccine has been found to cause a hypercoagulable state, which is well documented. This case report describes a man who presented with Mondor's disease following ChAdOx1-S vaccination with no other risk factors.
LEARNING POINTS
This is the first documented case of penile thrombophlebitis following ChAdOx1-S vaccination.We highlight a rare presentation of an uncommon condition.Clinicians should be aware of the clotting risks associated with ChAdOx1-S vaccination.
PubMed: 35402336
DOI: 10.12890/2022_003258 -
Breast (Edinburgh, Scotland) Dec 2022Mondor's disease is a rare disorder characterised by thrombosis of superficial veins within the subcutaneous tissue of the breast and other organs. While factors such as...
BACKGROUND
Mondor's disease is a rare disorder characterised by thrombosis of superficial veins within the subcutaneous tissue of the breast and other organs. While factors such as trauma, infection, physical exertion, breast cancer and breast surgery have been implicated, in the majority no cause is identified.
PATIENTS
Twenty patients presented with a clinical diagnosis of Mondor's disease to the Edinburgh Breast Services in 2020. We present the etiopathogenic data as well as clinical and imaging diagnostic findings.
RESULTS
During 2020, the annual incidence of Mondor's disease, in the UK's largest breast unit, increased five-fold compared to data from the previous year. This variation in the frequency of cases corresponded to trends in the frequency of Covid-19 infection during the pandemic. None of the patients had diagnosed COVID and few had any known etiopathogenic causes for their Mondor's.
CONCLUSION
Several recent studies have provided evidence for links between Covid-19 and thromboembolic events. Isolated reports have proposed a link between Covid-19 and Mondor's disease of the penis. Here we present data on a large series of Mondor's disease of the breast supporting a link between breast Mondor's and Covid-19.
Topics: Male; Humans; Breast Neoplasms; COVID-19; Thrombophlebitis; Breast; Breast Diseases
PubMed: 36427369
DOI: 10.1016/j.breast.2022.11.006 -
Vascular and Endovascular Surgery Nov 2018We report the case of a 90-year old woman who presented with septic pulmonary emboli due to suppurative thrombophlebitis at an old peripheral intravenous site. (Review)
Review
BACKGROUND:
We report the case of a 90-year old woman who presented with septic pulmonary emboli due to suppurative thrombophlebitis at an old peripheral intravenous site.
METHODS:
After unsuccessful treatment with antibiotics, the patient was taken to the operating room for excision and drainage of the purulent superficial vein.
RESULTS:
We review the literature and discuss the presentation, risk factors, treatment options, and complications of this often-overlooked disease entity.
CONCLUSIONS:
Suppurative thrombophlebitis is a rare but morbid disease that requires a high level of clinical suspicion to diagnose.
Topics: Aged, 80 and over; Anti-Bacterial Agents; Anticoagulants; Computed Tomography Angiography; Drainage; Female; Humans; Pulmonary Embolism; Sepsis; Staphylococcal Infections; Thrombophlebitis; Treatment Outcome
PubMed: 29909751
DOI: 10.1177/1538574418779469 -
Acta Dermatovenerologica Croatica : ADC Sep 2022Protein loss is often the result of kidney or intestinal disease (protein-losing enteropathy) and can cause a number of serious, potentially life-threatening...
Protein loss is often the result of kidney or intestinal disease (protein-losing enteropathy) and can cause a number of serious, potentially life-threatening complications such as hypotension, thrombocytosis, electrolyte imbalance, and cerebellar ischemia. Recent research suggests an association between extremely severe atopic dermatitis (AD) and allergic enteropathy. An exclusively breastfed 6-month-old infant was admitted to our institution due to failure to thrive, electrolyte imbalance, and severe AD (SCORing Atopic Dermatitis; SCORAD 40). On admission, the infant was in poor general condition, dehydrated, malnourished (bodyweight 4870 g, -3.98 z-score), with exudative erythematous morphs scattered throughout the body. Initial laboratory results showed microcytic hypochromic anemia, hypoalbuminemia, hypogammaglobinemia, thrombocytosis, hyponatremia, high values of total immunoglobulin E (IgE), and eosinophilia. Polysensitization to a number of nutritional and inhalation allergens was demonstrated, and an exclusive amino acid-based formula has been introduced into the diet. During the hospital course, the patient developed superficial thrombophlebitis and methicillin-resistant Staphylococcus aureus (MRSA) bacteremia. Eosinophilia was found in a small intestine biopsy sample. Due to severe hypogammaglobulinemia, skin infections, and bacteremia, the differential diagnosis included primary immune deficiency (STAT3 deficiency, DOCK8 deficiency, PGM3 deficiency, IPEX), but all available immunological tests were unremarkable. Exclusive amino acid-based formula diet was continued in the infant, with topical corticosteroids under wet-dressing therapy and intravenous immunoglobulin replacement therapy. With the gradual improvement of the general condition, the introduction of solid foods was started according to the findings of allergy testing. At 17 months of age, the patient gained weight and his skin status has been improving, although frequent use of topical corticosteroids was necessary. There were no infections, no anemia or thrombocytosis, and albumin and immunoglobulin supplementation were no longer required. The main mechanism of protein loss in infants with extremely severe atopic dermatitis is probably due to damaged skin, and partially due to the eosinophilic inflammation of the small intestine. Immunoglobulin loss, potentiated by physiological or transient hypogammaglobulinemia in infants, poses a very high risk for severe, potentially life-threatening infections.
Topics: Adrenal Cortex Hormones; Agammaglobulinemia; Albumins; Amino Acids; Bacteremia; Breast Feeding; Dermatitis, Atopic; Electrolytes; Female; Guanine Nucleotide Exchange Factors; Humans; Immunoglobulin E; Immunoglobulins, Intravenous; Infant; Methicillin-Resistant Staphylococcus aureus; Thrombocytosis
PubMed: 36254543
DOI: No ID Found