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Dermatologic Therapy May 2022
Topics: Acrodermatitis; Exanthema; Humans; Psoriasis; Skin Diseases, Vesiculobullous; Thalidomide
PubMed: 35261135
DOI: 10.1111/dth.15423 -
Dermatologic Therapy Sep 2021
Topics: Acrodermatitis; COVID-19; Exanthema; Humans; SARS-CoV-2
PubMed: 34329506
DOI: 10.1111/dth.15071 -
Skin Appendage Disorders Mar 2022Acrodermatitis continua of Hallopeau (ACH) is a rare, chronic, and recurrent form of pustular psoriasis (PP) localized to one or more digits. Due to the rarity of ACH,...
INTRODUCTION
Acrodermatitis continua of Hallopeau (ACH) is a rare, chronic, and recurrent form of pustular psoriasis (PP) localized to one or more digits. Due to the rarity of ACH, no clear treatment guidelines are currently available, making the therapeutic approach more complex.
CASE PRESENTATION
We report the first case of a young woman with ACH who was successfully treated with certolizumab pegol (CZP).
DISCUSSION/CONCLUSION
PP and ACH are difficult to treat, and biologics may be an option more manageable than cyclosporine. CZP is the safest biologic therapy during pregnancy, so there is low risk in prescribing it in women with childbearing potential. However, continuing biological therapy during pregnancy always involves an assessment of the clinical benefits, which must outweigh the risks.
PubMed: 35419415
DOI: 10.1159/000519144 -
The Tokai Journal of Experimental and... Dec 2022Zinc deficiency has long been known as acrodermatitis enteric dermatitis (congenital zinc deficiency). On the other hand, acquired zinc deficiency has attracted...
Zinc deficiency has long been known as acrodermatitis enteric dermatitis (congenital zinc deficiency). On the other hand, acquired zinc deficiency has attracted attention as a familiar disease in recent years. Epidemiological studies in Japan have shown that acquired zinc deficiency is more common than expected. It is also known that serum zinc levels fall markedly with age. In this report, several cases of acquired zinc deficiency that caused cheilitis are described. In all cases, the only symptom was cheilitis, the serum zinc level was low, and all cases were relieved by zinc supplementation. Zinc deficiency is associated with a range of pathological conditions, including mucocutaneous symptoms, delayed wound healing, dysgeusia, anemia, impaired immunity, and retarded growth development disorders. However, zinc deficiency may be overlooked even in cases of cheilitis alone. Especially in intractable cases, it is important to suspect zinc deficiency as one at the differential diagnoses.
Topics: Humans; Cheilitis; Acrodermatitis; Zinc; Intestine, Small; Japan
PubMed: 36420553
DOI: No ID Found -
The Lancet Regional Health. Europe Jul 2021Concerns about long-lasting symptoms attributed to Lyme borreliosis (LB) are widespread in the Western world, while such symptoms are highly prevalent in the general...
BACKGROUND
Concerns about long-lasting symptoms attributed to Lyme borreliosis (LB) are widespread in the Western world, while such symptoms are highly prevalent in the general population.
METHODS
In the largest prospective study to date, adults with physician-confirmed LB were included at the start of antibiotic treatment. Primary outcomes, prevalence of persistent symptoms and symptom severity, were assessed using three-monthly standardised questionnaires during one year. Persistent symptoms were defined as impaired scores for fatigue (CIS, subscale fatigue), cognitive impairment (CFQ) or pain (SF-36, subscale bodily pain) ≥6 months, with onset <6 months. Outcomes were compared with a longitudinal general population and a tick-bite cohort without LB as a reference.
FINDINGS
Of 1135 LB patients (94•8% erythema migrans, 5•2% disseminated LB), 1084 fulfilled primary analysis criteria, as well as 1942 population and 1887 tick-bite controls. Overall prevalence of persistent symptoms in LB patients was 27•2% (95%CI, 24•7%-29•7%); 6•0% and 3•9% higher than in population (21•2%, 95%CI, 19•3%-23•1%; < 0•0001) and tick-bite (23•3%, 95%CI 21•3%-25•3%; = 0•016) cohorts, respectively. At 12 months, fatigue, cognitive impairment, and pain were significantly more severe in erythema migrans patients than in reference cohorts, while in disseminated LB patients, only pain was more severe.
INTERPRETATION
In treated LB patients, persistent symptoms were significantly more prevalent and symptoms were more severe than in individuals without LB, although the background prevalence was substantial. This suggests an association, either direct or indirect, between persistent symptoms and LB in a relatively small subset of patients.
FUNDING
ZonMw; Dutch Ministry of Health, Welfare and Sport.
PubMed: 34557833
DOI: 10.1016/j.lanepe.2021.100142 -
Children (Basel, Switzerland) Aug 2023An infant with a prenatal diagnosis of citrullinemia, who started standard treatment at birth (L-arginine; sodium benzoate and a personalized diet characterized by a low...
An infant with a prenatal diagnosis of citrullinemia, who started standard treatment at birth (L-arginine; sodium benzoate and a personalized diet characterized by a low protein intake and supplementation of essential nutrients and amino acids), presented at 4 months of age with extended, progressive, and severe skin lesions consistent with acrodermatitis dysmetabolica. Guidelines for the diagnosis and management of urea cycle disorders underline that a low-protein diet places patients at risk of essential fatty acids, trace elements, and vitamin deficiency. At hospital admission, our patient had normal levels of zinc and alkaline phosphatases. The plasmatic amino acid profile revealed a severe and generalized deficiency. In particular, the serum levels of arginine, valine, and isoleucine were very low and the dermatitis did not improve until the blood levels of these amino acids increased. In our patient, skin lesions happened despite an early diagnosis of citrullinemia and timely treatment due to compliance issues as a consequence of linguistic barriers.
PubMed: 37761452
DOI: 10.3390/children10091491 -
Genes Aug 2021In a litter of Turkish Van cats, three out of six kittens developed severe signs of skin disease, diarrhea, and systemic signs of stunted growth at 6 weeks of age....
In a litter of Turkish Van cats, three out of six kittens developed severe signs of skin disease, diarrhea, and systemic signs of stunted growth at 6 weeks of age. Massive secondary infections of the skin lesions evolved. Histopathological examinations showed a mild to moderate hyperplastic epidermis, covered by a thick layer of laminar to compact, mostly parakeratotic keratin. The dermis was infiltrated with moderate amounts of lymphocytes and plasma cells. Due to the severity of the clinical signs, one affected kitten died and the other two had to be euthanized. We sequenced the genome of one affected kitten and compared the data to 54 control genomes. A search for private variants in the two candidate genes for the observed phenotype, and , revealed a single protein-changing variant, :c.1057G>C or p.Gly353Arg. The solute carrier family 39 member 4 gene () encodes an intestinal zinc transporter required for the uptake of dietary zinc. The variant is predicted to change a highly conserved glycine residue within the first transmembrane domain, which most likely leads to a loss of function. The genotypes of the index family showed the expected co-segregation with the phenotype and the mutant allele was absent from 173 unrelated control cats. Together with the knowledge on the effects of variants in other species, these data suggest :c.1057G>C as candidate causative genetic variant for the phenotype in the investigated kittens. In line with the human phenotype, we propose to designate this disease acrodermatitis enteropathica (AE).
Topics: Acrodermatitis; Animals; Biopsy; Cation Transport Proteins; Cats; DNA Mutational Analysis; Female; Male; Mutation, Missense; Pedigree; Skin; Whole Genome Sequencing; Zinc
PubMed: 34573291
DOI: 10.3390/genes12091309 -
Annales de Pathologie Oct 2022We report the case of an infant aged 8 and a half months, who had an apparent life-threatening event and died despite optimal resuscitation management. The medical...
We report the case of an infant aged 8 and a half months, who had an apparent life-threatening event and died despite optimal resuscitation management. The medical history was marked by mild symptoms, mainly feeding difficulties and progressively settling skin lesions. Parents were related (first cousins) and the patient had two healthy older sisters. Autopsy showed growth delay, symmetrical erythematous and ulcerated periorificial lesions associated with punctiform erythematous lesions of the face and alopecia. Microscopic examination revealed deep bronchial inhalation with the onset of infectious pneumopathy, major inflammatory ulceration of the gastrointestinal tract, hepatic steatosis, brain stem and pancreas abnormalities. We conclude that the cause of death was a multi-visceral failure with inhalation pneumopathy, in a context of very early onset inflammatory bowel disease (VEO-IBD). Genetic consultation, into a rare disease reference center, allowed to orient the analysis, to identify a homozygous pathogenic variant in the IL10RA gene, confirming the diagnostic of an autosomal recessive very early onset inflammatory bowel disease (inflammatory bowel disease 28, early-onset, autosomal recessive, #613148).
Topics: Age of Onset; Humans; Infant; Inflammatory Bowel Diseases
PubMed: 34980475
DOI: 10.1016/j.annpat.2021.11.007 -
BMJ Case Reports Feb 2023An immunocompetent male in his 70s was diagnosed with psoriatic arthritis based on dactylitis, onycholysis of the nails and scalp psoriasis. He was treated with...
An immunocompetent male in his 70s was diagnosed with psoriatic arthritis based on dactylitis, onycholysis of the nails and scalp psoriasis. He was treated with corticosteroids, methotrexate and local corticosteroid injections without improvements in his symptoms. When tumor necrosis factor-alpha inhibitor treatment was introduced, the symptoms worsened and dactylitis of all digits and a bluish-red rash were observed on the extensor side of the left hand and arm. At this point, a skin biopsy was performed showing histopathological changes compatible with Lyme borreliosis and serum contained IgG antibodies against It was concluded that he was suffering from acrodermatitis chronica atrophicans (ACA) and Lyme dactylitis. Ten days of phenoxymethylpenicillin treatment was initiated, and after 2 weeks, the dactylitis and ACA had regressed substantially. After 6 months, both had resolved. This case emphasises the need for clinical reassessment, when treatment is not effective.
Topics: Humans; Male; Exanthema; Lyme Disease; Borrelia burgdorferi; Arthritis, Psoriatic; Hand
PubMed: 36805866
DOI: 10.1136/bcr-2022-253182 -
Life (Basel, Switzerland) Dec 2022The occurrence of skin lesions is the earliest symptom of Lyme disease, and the diagnosis of these lesions and appropriate treatment may prevent complications of the...
The occurrence of skin lesions is the earliest symptom of Lyme disease, and the diagnosis of these lesions and appropriate treatment may prevent complications of the disease, which are mainly neurological. The cutaneous presentation in borreliosis is heterogeneous. There are typical lesions that constitute the basis for the diagnosis of Lyme disease, and atypical ones, which cause significant diagnostic difficulties especially when the patient does not remember the tick bite. This study aims to describe the heterogeneous skin symptoms of Lyme borreliosis, as well as offer a practical approach for the recognition of the disease. Based on pediatric cases from clinical practice, rare cutaneous presentations of Lyme disease at various stages of illness and therapy are presented. Diagnostic recommendations for recognizing individual forms are discussed.
PubMed: 36676022
DOI: 10.3390/life13010072