-
Nutrients Dec 2023Celiac disease (CD) is an immune-mediated systemic gluten-related disorder characterized by a wide spectrum of intestinal and extra-intestinal manifestations, including... (Review)
Review
Celiac disease (CD) is an immune-mediated systemic gluten-related disorder characterized by a wide spectrum of intestinal and extra-intestinal manifestations, including damage to cutaneous and connective tissue. We report a rare case of chronic severe dermatitis involving connective tissue and cutaneous vascular vessels as the main clinical presentation of undiagnosed seronegative gluten disorder. A gluten-free diet dramatically improved the intestinal and cutaneous clinical damage in the patient. Pitfalls and the steps of differential diagnosis are described. We also review the literature regarding studies of CD and connective tissue diseases to extend the knowledge of these rare associations. We propose a practical diagnostic approach in suspected CD in autoimmune cutaneous disorders.
Topics: Humans; Celiac Disease; Skin Diseases; Dermatitis; Glutens; Autoimmune Diseases
PubMed: 38201912
DOI: 10.3390/nu16010083 -
Pathogens (Basel, Switzerland) Nov 2022Gianotti-Crosti syndrome is a cutaneous eruption that occurs rarely in adults. It mostly concerns pediatric population and immunocompromised patients. Cytomegalovirus...
Gianotti-Crosti syndrome is a cutaneous eruption that occurs rarely in adults. It mostly concerns pediatric population and immunocompromised patients. Cytomegalovirus has already been described as one etiology of Gianotti-Crosti acrodermatitis in children and bone-marrow transplanted patients. Here, we present a Cytomegalovirus-associated Gianotti-Crosti syndrome in a 28-year-old immunocompetent female patient diagnosed in CHU Amiens-Picardie (Amiens, France). This type of case has never been shared in literature before. This rare complication of Cytomegalovirus infection indirectly led to disruption of anticoagulant treatment and thromboembolic incident that could have been fatal.
PubMed: 36422590
DOI: 10.3390/pathogens11111338 -
Psoriasis (Auckland, N.Z.) 2019Acrodermatitis continua of Hallopeau (ACH) is a rare, sterile pustular eruption of one or more digits. The condition presents with tender pustules and underlying...
Acrodermatitis continua of Hallopeau (ACH) is a rare, sterile pustular eruption of one or more digits. The condition presents with tender pustules and underlying erythema on the tip of a digit, more frequently arising on a finger than a toe. As far as classification, ACH is considered a localized form of pustular psoriasis. The eruption typically occurs after local trauma or infection, but such a history is not always present and various other etiologies have been described including infectious, neural, inflammatory, and genetic causes. The natural progression of ACH is chronic and progressive, often resulting in irreversible complications such as onychodystrophy that can result in anonychia, as well as osteitis that can result in osteolysis of the distal phalanges. Because of the rarity of ACH, there have been no randomized controlled studies to evaluate therapies, resulting in an absence of standardized treatment guidelines. In clinical practice, a wide variety of treatments have been attempted, with outcomes ranging from recalcitrance to complete resolution. In recent years, the introduction of biologics has provided a new class of therapy that has revolutionized the treatment of ACH. Specifically, rapid and sustained responses have been reported with the use of anti-tumor necrosis factor agents like infliximab, adalimumab, and etanercept; IL-17 inhibitors like secukinumab; IL-12/23 inhibitors like ustekinumab; and IL-1 inhibitors like anakinra. Nevertheless, there remains a considerable need for more research into treatment for the benefit of individual patients with ACH as well as for the clinical knowledge gained by such efforts. The purpose of this review is to provide a comprehensive overview of the key features of ACH as well as a discussion of clinical management strategies for this unique and debilitating condition.
PubMed: 31497529
DOI: 10.2147/PTT.S180608 -
Cureus Apr 2023Acrodermatitis enteropathica is a rare genetic disorder caused by a defect in intestinal zinc absorption, resulting in zinc deficiency and various clinical...
Acrodermatitis enteropathica is a rare genetic disorder caused by a defect in intestinal zinc absorption, resulting in zinc deficiency and various clinical manifestations, including dermatitis, diarrhea, alopecia, and nail abnormalities. Here we present the case of a 10-year-old male child with diarrhea, and abdominal pain for several months who was diagnosed with acrodermatitis enteropathica confirmed by low serum zinc levels. The child had multiple erythematous, scaly, and crusted lesions on the hands and elbows, which resolved after starting oral zinc sulfate supplementation (10 mg/kg/day) in three divided doses. The patient's serum zinc levels normalized (1.0 µg/mL), and the skin lesions completely resolved after six months of follow-up with a regular zinc-rich diet and gradual reduction of zinc sulfate dosage to a maintenance level (2-4 mg/kg/day). This case report emphasizes the importance of timely diagnosis and treatment of acrodermatitis enteropathica to prevent the harmful consequences of zinc deficiency and highlights the need for healthcare providers to consider this disorder in children presenting with skin lesions and diarrhea, particularly those with a positive family history or consanguinity.
PubMed: 37214014
DOI: 10.7759/cureus.37783 -
Tidsskrift For Den Norske Laegeforening... Nov 2021Acrodermatitis chronica atrophicans (ACA) is a late manifestation of Borrelia infection and is easily overlooked, especially in elderly patients.
BACKGROUND
Acrodermatitis chronica atrophicans (ACA) is a late manifestation of Borrelia infection and is easily overlooked, especially in elderly patients.
CASE PRESENTATION
A woman in her fifties presented with dizziness, general muscle stiffness, chills, nausea and a feeling of transient shock in her head during dosage reduction of escitalopram. The symptoms were therefore initially misinterpreted as related to her psychiatric disorder. Four months after the first symptoms presented, she complained that her right foot had become one shoe size larger than her left. Skin manifestations were found to be consistent with acrodermatitis chronica atrophicans.
INTERPRETATION
Acrodermatitis chronica atrophicans is uncommon and affects women more than men. The skin changes mostly occur on the dorsal side of the extremities, often the feet or hands. The diagnosis is confirmed by positive serum antibodies (high level of IgG, and IgM can also be present), and a positive Borrelia PCR skin test.
Topics: Acrodermatitis; Aged; Escitalopram; Female; Hand; Humans; Lyme Disease; Male; Skin
PubMed: 34758594
DOI: 10.4045/tidsskr.21.0201 -
BMJ Case Reports Aug 2021
Topics: Acrodermatitis; Eye; Face; Humans; Zinc
PubMed: 34353838
DOI: 10.1136/bcr-2021-244545 -
Medecine Et Maladies Infectieuses Aug 2019Lyme borreliosis is transmitted en France by the tick Ixodes ricinus, endemic in metropolitan France. In the absence of vaccine licensed for use in humans, primary... (Review)
Review
Lyme borreliosis is transmitted en France by the tick Ixodes ricinus, endemic in metropolitan France. In the absence of vaccine licensed for use in humans, primary prevention mostly relies on mechanical protection (clothes covering most parts of the body) that may be completed by chemical protection (repulsives). Secondary prevention relies on early detection of ticks after exposure, and mechanical extraction. There is currently no situation in France when prophylactic antibiotics would be recommended. The incidence of Lyme borreliosis in France, estimated through a network of general practitioners (réseau Sentinelles), and nationwide coding system for hospital stays, has not significantly changed between 2009 and 2017, with a mean incidence estimated at 53 cases/100,000 inhabitants/year, leading to 1.3 hospital admission/100,000 inhabitants/year. Other tick-borne diseases are much more seldom in France: tick-borne encephalitis (around 20 cases/year), spotted-fever rickettsiosis (primarily mediterranean spotted fever, around 10 cases/year), tularemia (50-100 cases/year, of which 20% are transmitted by ticks), human granulocytic anaplasmosis (<10 cases/year), and babesiosis (<5 cases/year). The main circumstances of diagnosis for Lyme borreliosis are cutaneous manifestations (primarily erythema migrans, much more rarely borrelial lymphocytoma and atrophic chronic acrodermatitis), neurological (<15% of cases, mostly meningoradiculitis and cranial nerve palsy, especially facial nerve) and rheumatologic (mostly knee monoarthritis, with recurrences). Cardiac and ophtalmologic manifestations are very rarely encountered.
Topics: Animals; Babesiosis; Encephalitis, Tick-Borne; France; Humans; Ixodes; Lyme Disease; Practice Guidelines as Topic; Skin Diseases, Bacterial; Societies, Scientific; Tick-Borne Diseases
PubMed: 31097370
DOI: 10.1016/j.medmal.2019.04.381 -
Journal of Cutaneous Pathology Nov 2020Atypical cutaneous lymphoid infiltrates are challenging lesions in dermatopathology. We present a summary of the literature regarding kappa and lambda... (Review)
Review
BACKGROUND
Atypical cutaneous lymphoid infiltrates are challenging lesions in dermatopathology. We present a summary of the literature regarding kappa and lambda immunohistochemistry (IHC) and in situ hybridization (ISH) in the evaluation of atypical cutaneous or mucosal lymphoid infiltrates.
METHODS
Relevant articles from 1967 to 2018 in the English language were identified and summarized. In the absence of larger studies, case series of n ≥ 3 were included.
RESULTS
Sixty-three articles assessing kappa and lambda IHC and/or ISH were identified. Most focused on marginal zone lymphomas. Other lymphomas included follicle center lymphoma, diffuse large B-cell lymphoma, chronic lymphocytic leukemia/small lymphocytic lymphoma, mantle cell lymphoma, lymphoplasmacytic lymphoma, plasmablastic lymphoma, multiple myeloma, monoclonal gammopathy of undetermined significance, and polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS). Non-neoplastic lesions included reactive lymphoid hyperplasia, cutaneous plasmacytosis, connective tissue disease, IgG4-related disease, acrodermatitis chronic atrophicans, Zoon balanitis, dermatitides, and infiltrates around epithelial dysplasias/neoplasias.
CONCLUSION
Kappa and lambda IHC and ISH are useful tools in the evaluation of cutaneous B-cell lymphomas and plasma cell neoplasms. The literature supports that the detection of light-chain restriction by IHC and ISH is one of the most useful findings in the differential diagnosis of reactive lymphoid hyperplasia vs B-cell lymphoma with plasmacytic differentiation.
Topics: Humans; Immunoglobulin kappa-Chains; Immunoglobulin lambda-Chains; Immunohistochemistry; In Situ Hybridization; Lymphocytes; Skin Diseases
PubMed: 32870521
DOI: 10.1111/cup.13858 -
Microbiology Spectrum Apr 2022In this retrospective study, the performance of nine serological screening assays for Lyme borreliosis (LB) diagnostics was evaluated using a study population of LB...
In this retrospective study, the performance of nine serological screening assays for Lyme borreliosis (LB) diagnostics was evaluated using a study population of LB cases and controls. Sera derived from 74 well-defined LB cases and 122 controls were included. The LB cases were diagnosed with erythema migrans (EM; = 11), Lyme neuroborreliosis (LNB; = 35), Lyme arthritis (LA; = 20), or acrodermatitis chronica atrophicans (ACA; = 8). Controls comprised 74 age- and gender-matched healthy individuals and 48 patients with other diseases with anticipated high rates of cross-reactivity. The assays under evaluation were selected based on a literature review and expected continued availability with CE marking under the new diagnostic regulation (European Union) 2017/746. The overall sensitivity (IgG and IgM results combined) among LB cases ranged between 54.5% (6 of 11) and 90.9% (10 of 11) for EM patients and between 97.1% (34 of 35) and 100% for patients with LNB, LA, and ACA. The positivity rate ranged between 8.1% (6 of 74) and 29.7% (22 of 74) among the healthy controls and between 22.9% (11 of 48) and 64.6% (31 of 48) among the cross-reactivity controls. The IgM results were more heterogeneous than the IgG and IgM/IgG results and did not contribute to the overall sensitivity but substantially increased the positivity rates among the controls. In conclusion, all evaluated serological screening assays performed comparably with respect to early- and late-disseminated LB. The addition of an IgM assay to the screening of -specific IgG antibodies had no added value for the diagnosis of Lyme borreliosis. Serology plays an important role in the diagnosis of Lyme borreliosis. Guidelines prescribe a two-tier testing algorithm in which a highly sensitive screening assay is used for screening and reactive sera are retested with an immunoblot to reduce false positivity rates. Recently, two commonly used screening assays were discontinued, including the very well-performing C6 Lyme enzyme-linked immunosorbent assay (ELISA) (Immunetics). This study provides an evaluation of the performance of nine different serology screening assays, eight with expected future availably and the C6 Lyme ELISA, using a well-defined study panel of Lyme borreliosis patients, healthy population controls, and cross-reactivity controls. Evaluation data on multiple assays aid diagnostic laboratories in their choice for a reliable serology screening assay to improve their diagnostic algorithm for Lyme borreliosis.
Topics: Antibodies, Bacterial; Borrelia; Humans; Immunoglobulin G; Immunoglobulin M; Lyme Disease; Retrospective Studies
PubMed: 35297658
DOI: 10.1128/spectrum.00510-22 -
International Journal of Dermatology Sep 2020
Topics: Acrodermatitis; Betacoronavirus; Biopsy; COVID-19; Coronavirus Infections; Edema; Humans; Pain; Pain Measurement; Pandemics; Pneumonia, Viral; SARS-CoV-2; Severity of Illness Index; Skin
PubMed: 32608503
DOI: 10.1111/ijd.15025