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Journal of Internal Medicine Aug 2021Information on large groups of patients with acrodermatitis chronica atrophicans (ACA) is limited.
BACKGROUND
Information on large groups of patients with acrodermatitis chronica atrophicans (ACA) is limited.
METHODS
We assessed clinical and microbiological characteristics of patients with ACA diagnosed at a single medical centre and compared findings in periods 1991-2004 vs. 2005-2018. The cohort is representative of Slovenian ACA patients.
RESULTS
We assessed 693 patients: 461 females and 232 males, with median age of 64 years. Median duration of ACA before diagnosis was 12 months. In all but 2 patients, the skin lesions were located on extremities, more often on the lower (70.0%) than the upper (45.2%), bilaterally in 42.4%. Reddish-blue discoloration, swelling, thinning and wrinkling of skin were present in 95.2%, 28.1%, 46.4% and 20.5% of patients, respectively. Overall, 64.4% of patients reported constitutional symptoms, 23.1% had local symptoms, and 20.8% had symptoms/signs of peripheral neuropathy. Nodules, arthritis, joint deformity, muscle atrophy and paresis were rare (<3%). Borreliae were isolated from 200/664 (30.1%) skin samples; 92.8% were Borrelia afzelii. B. garinii and B. burgdorferi s.s. were more often isolated from the skin of male patients (OR = 4.17) and from those with arthropathy (OR = 11.74). Patients included in the more recent period were older, complained less often of constitutional symptoms but more often of local symptoms, and more often had local swelling but less often skin atrophy and bilateral involvement, probably as a consequence of earlier diagnosis.
CONCLUSIONS
ACA, typically caused by B. afzelii, usually affects older women. Clinical presentation depends on the duration of illness and probably on the Borrelia species causing the disease.
Topics: Acrodermatitis; Adult; Aged; Atrophy; Borrelia burgdorferi Group; Cohort Studies; Female; Humans; Lyme Disease; Male; Middle Aged; Slovenia
PubMed: 33550695
DOI: 10.1111/joim.13266 -
Indian Journal of Dermatology,... 2023
Topics: Humans; Argininosuccinic Aciduria; Acrodermatitis
PubMed: 37317759
DOI: 10.25259/IJDVL_75_2023 -
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 -
Indian Dermatology Online Journal 2023
PubMed: 37089830
DOI: 10.4103/idoj.idoj_249_21 -
Dermatology (Basel, Switzerland) 2015Maculopapular or urticarial eruptions and erythema multiforme sometimes occur in patients affected with Mycoplasma pneumoniae respiratory infections. Further eruptions... (Review)
Review
BACKGROUND
Maculopapular or urticarial eruptions and erythema multiforme sometimes occur in patients affected with Mycoplasma pneumoniae respiratory infections. Further eruptions have also been reported.
OBJECTIVE
To review the literature addressing M. pneumoniae respiratory infection and rather unusual eruptions.
METHODS
Computer-based search in the U.S. National Library of Medicine database as well as in the search engine Google.
RESULTS
We found a possible relationship between M. pneumoniae infection and Fuchs' syndrome (n = 37), varicella-like eruptions (n = 8), Henoch-Schönlein syndrome and further leukocytoclastic vasculitides (n = 21) and erythema nodosum (n = 11). A temporal relationship was also observed with 2 cases of Gianotti-Crosti syndrome. Finally, there exists reasonable evidence that pityriasis rosea Gibert and pityriasis lichenoides et varioliformis acuta Mucha-Habermann are not associated with Mycoplasma infections.
CONCLUSION
This review implies that M. pneumoniae may cause, in addition to erythematous maculopapular (or urticarial) eruptions and erythema multiforme, Fuchs' syndrome and varicella-like eruptions. Furthermore, there is an intriguing link with leukocytoclastic vasculitides or erythema nodosum that deserves further investigation.
Topics: Acrodermatitis; Erythema Nodosum; Humans; IgA Vasculitis; Mycoplasma pneumoniae; Pneumonia, Mycoplasma; Skin Diseases; Stevens-Johnson Syndrome; Vasculitis, Leukocytoclastic, Cutaneous
PubMed: 26067570
DOI: 10.1159/000430809 -
IUBMB Life Mar 2010Studies on dietary zinc absorption are of fundamental nutritional significance, owing to the ubiquity of zinc in biological processes and the severe outcomes of zinc... (Review)
Review
Studies on dietary zinc absorption are of fundamental nutritional significance, owing to the ubiquity of zinc in biological processes and the severe outcomes of zinc deficiency in humans. Insights into the molecular basis of dietary zinc absorption have advanced in recent years through functional characterization of zinc transporters in cell culture, immunohistochemical studies on rodent intestine and analysis of gene knockout mice. Zinc transporters with manifested expression in enterocytes include ZnT1, ZnT2, ZnT4, ZnT5, ZnT6, ZnT7, Zip4, and Zip5. Among them, ZIP4, the gene responsible for Acrodermatitis enteropathica, an inherited human zinc deficiency, mediates dietary zinc uptake into enterocytes across the apical membrane, while ZnT1 is involved in zinc efflux from enterocytes across the basolateral membrane into circulation. The intracellular trafficking pathways for zinc retention and movement between apical and basolateral sides of the enterocytes have yet to be defined. The utilization of Drosophila model in elucidating molecular mechanisms of dietary zinc absorption is also discussed in this review.
Topics: Animals; Carrier Proteins; Cation Transport Proteins; Diet; Drosophila; Enterocytes; Humans; Intestinal Absorption; Intestinal Mucosa; Mice; Mice, Knockout; Zinc
PubMed: 20120011
DOI: 10.1002/iub.291 -
The Journal of Investigative Dermatology Sep 2016Homozygous or compound heterozygous IL36RN gene mutations underlie the pathogenesis of psoriasis-related pustular eruptions including generalized pustular psoriasis,... (Review)
Review
Homozygous or compound heterozygous IL36RN gene mutations underlie the pathogenesis of psoriasis-related pustular eruptions including generalized pustular psoriasis, palmoplantar pustular psoriasis, acrodermatitis continua of Hallopeau, and acute generalized exanthematous pustular eruption. We identified two unreported IL36RN homozygous mutations (c.41C>A/p.Ser14X and c.420_426del/p.Gly141MetfsX29) in patients with familial generalized pustular psoriasis. We analyzed the impact of a spectrum of IL36RN mutations on IL-36 receptor antagonist protein by using site-directed mutagenesis and expression in HEK293T cells. This enabled us to differentiate null mutations with complete absence of IL-36 receptor antagonist (the two previously unreported mutations, c.80T>C/p.Leu27Pro, c.28C>T/p.Arg10X, c.280G>T/p.Glu94X, c.368C>G/p.Thr123Arg, c.368C>T/p.Thr123Met, and c.227C>T/p.Pro76Leu) from mutations with decreased (c.95A>G/p.His32Arg, c.142C>T/p.Arg48Trp, and c.308C>T/p.Ser113Leu) or unchanged (c.304C>T/p.Arg102Trp and c.104A>G/p.Lys35Arg) protein expression. Functional assays measuring the impact of mutations on the capacity to repress IL-36-dependent activation of the NF-κB pathway showed complete functional impairment for null mutations, whereas partial or no impairment was observed for other mutations considered as hypomorphic. Finally, null mutations were associated with severe clinical phenotypes (generalized pustular psoriasis, acute generalized exanthematous pustular eruption), whereas hypomorphic mutations were identified in both localized (palmoplantar pustular psoriasis, acrodermatitis continua of Hallopeau) and generalized variants. These results provide a preliminary basis for genotype-phenotype correlation in patients with deficiency of the IL-36Ra (DITRA), and suggest the involvement of other factors in the modulation of clinical expression.
Topics: Adult; Child; Child, Preschool; Disease Progression; Female; Gene Expression Regulation; Genetic Association Studies; Genotype; Humans; Interleukins; Male; Mutation; Phenotype; Prognosis; Psoriasis; Risk Assessment
PubMed: 27220475
DOI: 10.1016/j.jid.2016.04.038 -
Revista Paulista de Pediatria : Orgao... 2018To report a case of acrodermatitis enteropathica, a rare disease with autosomal recessive inheritance.
OBJECTIVE
To report a case of acrodermatitis enteropathica, a rare disease with autosomal recessive inheritance.
CASE DESCRIPTION
An 11-month-old boy was presenting symmetrical erythematous and yellowish-brownish crusted lesions on his face, feet, hands and knees, intermittent diarrhea, fever, and recurrent infections since the age of six months. He was thin and had scarce hair on the scalp. The serum zinc level was measured and a reduced level of 27.0 mcg/dL (normal range: 50.0-120.0) was identified. Oral supplementation with 2.0 mg/kg/day of zinc sulfate was immediately initiated. A rapid and progressive improvement of symptoms was observed. The symptoms reappeared with an attempt to stop supplementation.
COMMENTS
Recognizing and properly treating acrodermatitis enteropathica is important to prevent complications.
Topics: Acrodermatitis; Humans; Infant; Male; Zinc
PubMed: 29412435
DOI: 10.1590/1984-0462/;2018;36;2;00010 -
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