scholarly journals Necrotising Cutaneous Lesions in Anti Neutrophil Cytoplasmic Antibodies- Associated Vasculitis Mimicking Necrotising Fasciitis

Author(s):  
Ahmad Arieff Atan ◽  
Khong Wee Lee ◽  
Sughilan Sundara Murthi ◽  
Muhammad Azri Mohamed Mansoor ◽  
Mustaqim Afifi

Necrotising vasculitis is a very rare occurrence and commonly caused by an underlying autoimmune pathology. Anti Neutrophil Cytoplasmic Antibodies (ANCA) associated vasculitis is an established cause for cutaneous manifestations of necrotising vasculitis, and is associated with multisystemic involvement, in which presentations of one system may feature predominantly and lead to misdiagnosis. A 33-year-old male presented with acute history of shortness of breath and necrotising blisters over both his ankles and arms. He was initially provisionally diagnosed with necrotising fasciitis, which was eventually ruled out as the serological biomarkers (including low level of C3 and presence of cytoplasmic type of ANCA on indirect immunofluorescence) drew suspicion of an autoimmune pathology. A multidisciplinary approach led to the commencement of steroid, which improved the patient’s condition rapidly. The histological examination of sample from the skin biopsy later confirmed the diagnosis of ANCA-Associated Vasculitis (AAV). The patient eventually recovered with excellent outcomes. Despite its rarity, any abnormal skin lesion may be a subtle presentation of necrotising cutaneous vasculitis. A high index of suspicion through a multidisciplinary approach, coupled with meticulous investigative process including serological biomarkers and skin biopsy, helps to avoid misdiagnosis which may be detrimental to the patient.

Dermatology ◽  
2021 ◽  
pp. 1-6
Author(s):  
Arvind Ahuja ◽  
Minakshi Bhardwaj ◽  
Poojan Agarwal

Background: Diagnosis of extrapulmonary histoplasmosis in HIV seronegative and immunocompetent patients is often challenging, so a high index of suspicion is required. Cutaneous manifestation of infection shows a wide spectrum of lesions including erythematous plaques; maculopapules; crusted, verrucous, or desquamative papules and nodules; abscesses; and mucocutaneous ulcers among others. Due to the variations in its clinical presentation, histopathology plays a very important role in the detection of spores and the confirmation of diagnosis. Objectives: The aim of our study was to analyze clinicopathological characteristics of cutaneous manifestations of biopsy-proven histoplasmosis in HIV seronegative individuals. We also examined the utility of Fite stain for the diagnosis of Histoplasma capsulatum on tissue biopsy sections. Methods: This was a retrospective, observational study on 7 patients who were HIV seronegative and clinically manifested with isolated cutaneous lesions or disseminated disease. Skin biopsy from the lesions was performed on all 7 patients. In addition to H&E staining and special stains for detecting fungus, Fite staining was performed on all of the cases to study its utility in detecting H. capsulatum spores. Results: The skin lesions were widely disseminated in all patients and the most common cutaneous lesions were papules, present in all 7 patients. On review of the H&E-stained slides, the most common pattern was histiocytic lobular panniculitis-like infiltrate observed in 4 cases. Fite stain highlighted the yeast as magenta-colored spores on a blue background in all cases, except for 1 with a granulomatous pattern. Conclusion: A primary cutaneous manifestation of H. capsulatum infection in non-HIV-infected individuals is extremely rare. Fite stain could aid in differentiating the spores of H. capsulatum from those of other fungi, Cryptococcus and Candida in particular.


2021 ◽  
Vol 9 ◽  
pp. 2050313X2199719
Author(s):  
Juan Carlos Palomo-Pérez ◽  
Maria Elisa Vega-Memije ◽  
David Aguilar-Blancas ◽  
Erik González-Martínez ◽  
Lucia Rangel-Gamboa

China officially recognized atypical pneumonia outbreak in December 2019; on 11 March 2020, the World Health Organization declared COVID-19 as a pandemic that is produced by a new coronavirus, named SARS-CoV-2, of rapid transmissibility, which can be asymptomatic, with mild to severe respiratory symptoms, and with cardiovascular, neurological, gastrointestinal, and cutaneous complications. Considering that the pandemic prolonged more than initially expected was prognostic, it is essential for the medical community to identify the signs and symptoms of COVID-19. Thus, this work’s objectives were to present cases of cutaneous lesions observed in COVID-19 Mexican patients. We register cutaneous lesions in COVID-19 patients referred from internal medicine and otorhinolaryngology services to dermatology. We presented four interesting cases with cutaneous lesions, including exanthema morbilliform, urticaria, chilblains, ecchymosis, and facial edema, and review the available literature. The most frequent cutaneous markers are rash, chilblains, and urticaria. Skin lesions may be the first manifestation of COVID-19, accompany initial respiratory symptoms, or appear during the disease course. Symptoms associated with vascular changes (livedo reticularis and vasculitis) are considered of poor prognosis.


2022 ◽  
Vol 11 (2) ◽  
pp. 375
Author(s):  
Magdalena Żychowska ◽  
Adam Reich

Background: (Video)dermoscopy is a non-invasive diagnostic technique that has a well-established role in dermatooncology. In recent years, this method has also been increasingly used in the assessment of inflammatory dermatoses. So far, little is known about the (video)dermoscopic features of dermatomyositis (DM). Methods: Consecutive patients with DM were included in the study and videodermoscopic assessments of the nailfolds, scalp, and active skin lesions were performed. Results: Fifteen patients with DM (10 women and 5 men) were included. Capillaroscopy showed elongated capillaries (90.9%), avascular areas (81.8%), disorganized vessel architecture (81.8%), tortuous capillaries (72.7%), dilated capillaries (72.7%), and hemorrhages (72.7%). The trichoscopic findings included linear branched vessels (80.0%), linear vessels (60.0%), linear curved vessels (53.3%), perifollicular pigmentation (40.0%), perifollicular erythema (33.3%), scaling (20.0%), white (20.0%) or yellow (20%) interfollicular scales, and white (20.0%) or pinkish (13.3%) structureless areas. Polymorphic vessels of an unspecific distribution and white or pink structureless areas were frequently observed under dermoscopy in cutaneous manifestations of DM, including Gottron’s papules and Gottron’s sign. Conclusions: Dermoscopy of the nailfolds (capillaroscopy), scalp (tricoscopy), and active cutaneous lesions may be of value in the preliminary diagnosis of DM.


1970 ◽  
Vol 42 (3) ◽  
pp. 231-233
Author(s):  
M Mukhtar-yola ◽  
LI Audu ◽  
AT Otuneye ◽  
AB Mairami ◽  
EC Otubelu ◽  
...  

Hypomelanosis of Ito (HI) though said to be the third most common neurocutaneus disorder, is rarely reported in paediatric practice in Africa. A high index of suspicion must be maintained in children with cutaneous lesions as a seizure may be the first symptom that may bring the child to attention. A case of HI in a neonate is hereby reported to sensitize clinicians about this relatively uncommon disorder


2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Abbas Azadi ◽  
Payman Jafarpour Fard ◽  
Mohammad Almasian

Brucellosis is a disease that is transmitted from animals to humans mainly via the consumption of unpasteurized dairy products, and it can involve any organ all over the body. Here, we report a significant rare case of brucellosis with cutaneous manifestations in a 52-year-old male patient whose disease was diagnosed via a serology test. The patient received standard antibiotic treatment, and his cutaneous lesions healed quickly. Although the cutaneous manifestations of brucellosis are exceedingly rare, in case of encountering ulcerative lesions and other cutaneous findings, particularly in endemic areas, infection with brucellosis should be kept in mind as an important differential diagnosis.


2018 ◽  
Vol 22 (5) ◽  
pp. 524-526 ◽  
Author(s):  
Meggie Morand ◽  
Myrna Chababi-Atallah ◽  
Sara-Élizabeth Jean ◽  
Carolina Fernandes

Background: Calciphylaxis is a well-known entity in end-stage kidney disease and after renal transplant. Cases of nonuremic calciphylaxis (NUC) have also been reported, but data on this rare condition are mainly empirical. Objective: We discuss a case of NUC secondary to spontaneous tumour lysis syndrome in a patient who had chemotherapy for non-Hodgkin lymphoma and present a review of the literature to better characterize malignancy-associated NUC. Methods: We identified 12 published cases of malignancy-associated NUC. Results: This systematic review of malignancy-associated NUC did not show a relationship between the type of malignancy, the distribution of skin lesions, or mortality. However, distal more than proximal involvement seems to be more frequently associated with calcium phosphate imbalance. Conclusion: Clinicians must maintain a high index of suspicion for calciphylaxis when evaluating patients with cutaneous lesions developed in the setting of malignancy.


2020 ◽  
Vol 58 (1) ◽  
pp. 25-31
Author(s):  
Pierre Pfirmann ◽  
Jerome Aupy ◽  
Eva Jambon ◽  
Laetitia Idier ◽  
Mathilde Prezelin-Reydit ◽  
...  

BackgroundTuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disorder. Due to the various manifestations of TSC and their potential complications, a multidisciplinary care approach is recommended by consensus guidelines.ObjectivesOur study aimed to give a complete description of our TSC adult cohort and to evaluate the multidisciplinary and interdisciplinary management model.MethodsData on each adult patient diagnosed with TSC, including disease manifestations, interventions and outcomes, were collected at baseline and updated annually. A multidisciplinary TSC approach with all the recommended explorations was carried out annually.Results90 patients were enrolled in Centre Hospitalier Universitaire de Bordeaux, between January 2000 and September 2018. Median age of patients at inclusion was 37 years (range, 27–47) and 20 years old at diagnosis of TSC. Regarding the occurrence of TSC manifestations, 97% of the patients had cutaneous lesions, 89% had neurological manifestations, 83% had renal manifestations and 100% had dental lesions with pits. More than half the patients had sclerotic bone lesions (68%), TSC-associated neuropsychiatric disorders (64%) and lymphangioleiomyomatosis (59%). A TSC multidisciplinary approach was developed including a global follow-up and an evaluation of TSC targeting organs, according to the recommendations. A satisfaction survey revealed global and entire satisfaction of patients with TSC.ConclusionWe obtained an accurate description of a cohort of adult patients with TSC. Our multidisciplinary approach model allowed us to provide optimal management of patients with TSC with a high level of patient satisfaction.


2017 ◽  
Vol 21 (4) ◽  
pp. 348-350
Author(s):  
Pierre-Olivier Grenier ◽  
Isabelle Auger

Background and Objective: Acquired immune deficiency syndrome (AIDS)–associated Kaposi sarcoma (KS) among the pediatric population is a rare entity in North America and Europe, and its cutaneous manifestations are not well defined in the literature. The investigators report the case of a boy with an AIDS-associated KS presenting as an infiltrated hyperkeratotic plaque of the plantar arch. Methods and Results: An 11-year-old African boy with congenital human immunodeficiency virus (HIV) had a skin biopsy of the plantar lesion that was consistent with a KS. The patient also presented intestinal and pulmonary symptoms; combined chemotherapy regimen and highly active antiretroviral therapy were given in the presence of systemic involvement. Conclusion: AIDS-associated KS poses a particular challenge to clinical diagnosis, since it can manifest with a variety of lesions. Dermatologists should have a low threshold for performing a skin biopsy in patients with HIV.


2017 ◽  
Vol 29 (4) ◽  
pp. 410-413 ◽  
Author(s):  
Linda Tognetti ◽  
Elisa Cinotti ◽  
Sergio Tripodi ◽  
Guido Garosi ◽  
Pietro Rubegni

The worldwide re-emergence of secondary syphilis which happened in the last decade, has led to an increase in primary and secondary syphilis cases, along with the presentation of atypical forms. Nevertheless, reports of renal syphilis with mucosal and/or cutaneous manifestations are nowadays increasing. Typically, secondary syphilis infection in adults causes nephrotic syndrome due to a membranous glomerulonephritis. Here, we report a case of a 30-year-old immunocompetent man presenting with skin rash, oral and perianal erosions and nephritic syndrome. Laboratory investigations revealed a form of membranoproliferative glomerulonephritis secondary to Treponema pallidum infection. Therapy with benzathine penicillin brought prompt and complete remission of the disease. Although well described for congenital syphilis, this histopathologic pattern of renal involvement is very rarely reported in adult patients. In case of detection of an otherwise unexplained nephritic syndrome in sexually active patients with mucosal and/or anal lesions, an unrecognized syphilis infection should be suspected.


2017 ◽  
Vol 4 (6) ◽  
pp. 2060
Author(s):  
Karrunya K. J. ◽  
Sumathi Bavanandam ◽  
Nirmala Dheivamani ◽  
Vijayabhaskhar C. Reddy ◽  
Manoharan K.

Background: Wilson’s disease in children is the most common inherited disorder of copper metabolism in India with varied clinical manifestations secondary to deposition of copper in various organs including skin. Methods: The aim of the study was to study various cutaneous manifestations of Wilson’s disease in south Indian children. A prospective study for a period of one year was conducted at Dermatology and Pediatric Gastroenterology clinic in Government Rajiv Gandhi Hospital, Chennai. Clinical, demographic details, various cutaneous manifestations and results of skin biopsy were analysed.  Results: Out of the total 30 children with Wilson’s disease, 93.3% had at least one cutaneous, mucosal, hair or nail findings but not statistically significant between naive and on treatment group. Cutaneous findings, mucosal involvement, hair changes and nail findings were observed in 90%, 36.7%, 46.7% and 60% respectively. Cutaneous findings were more common in liver disease (P value<0.05). Xerosis was the most common cutaneous manifestation seen in 63.3% patients, followed by pigmentation in 36.7% and infection in 30%.  KF ring was noted in 53.3%. Icterus and pruritus were seen in one fifth of study children and majority had portal hypertension. Vascular changes like spider nevi, purpura and other features of portal hypertension were noted in 16.6% of study population. Trichomegaly was one of the common hair manifestation found in 9 (30% especially in children already on treatment (p value <0.05). There was no evidence of stainable copper on skin biopsy. Conclusions: The physicians should be aware of various cutaneous manifestations of Wilson’s disease in children.


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