scholarly journals Wells’ Syndrome Successfully Treated with Colchicine

2017 ◽  
Vol 9 (2) ◽  
pp. 65-69 ◽  
Author(s):  
Álvaro Iglesias Puzas ◽  
Laura Mesa Álvarez ◽  
Ángeles Flórez Menéndez ◽  
Susana Romero Yuste ◽  
Olga Prieto Gómez

Eosinophilic cellulitis is an uncommon, inflammatory and chronic disorder of unknown etiology. Corticosteroids are currently considered as the first-line treatment but they are not without significant disadvantages such as contraindications in steroid-resistant cases and patients with frequent recurrences. We report a patient suffering from Wells’ syndrome with a 24-year history of symptomatic and generalized skin lesions. After consultation in our department, treatment with colchicine 1 mg/day was prescribed resulting in large clinical improvement. No side effects have been recorded. To our knowledge, this is an original disease approach. Although small, our clinical experience supports the inclusion of colchicine in the drug armamentarium when treating patients suffering from Wells’ syndrome. Indeed, its excellent safety profile makes it very attractive for patients with frequent recurrent episodes who need secure options for the medium- and long-term disease control.

2015 ◽  
Vol 7 (2) ◽  
pp. 117-122 ◽  
Author(s):  
Maxence Cormerais ◽  
Florence Poizeau ◽  
Laure Darrieux ◽  
Laurent Tisseau ◽  
Gilles Safa

Wells' syndrome (WS), or eosinophilic cellulitis, is an uncommon inflammatory dermatosis of unknown etiology that typically presents with pruritic cellulitis-like plaques on the extremities. Therefore, WS is often misdiagnosed as bacterial cellulitis due to its similarity in presentation. Here, we report two cases of WS that masqueraded as bacterial facial cellulitis. Under treatment with oral prednisone and/or a combination therapy with levocetirizine and hydroxyzine, both patients showed a dramatic improvement of the skin lesions. These cases highlight the need for clinicians to consider WS in the differential diagnosis when evaluating a patient with facial cellulitis that does not respond to an initial antimicrobial regimen. In addition, our cases suggest that combination therapy with levocetirizine and hydroxyzine may be successfully used as corticosteroid-sparing treatment or to prevent relapse after the discontinuation of corticosteroid treatment.


2020 ◽  
Vol 15 ◽  
Author(s):  
Maria Carla Di Paolo ◽  
Cristiano Pagnini ◽  
Maria Giovanna Graziani

: Inflammatory bowel diseases (IBDs) are chronic conditions characterized by unknown etiology and pathogenesis with deregulation of mucosal immunity. Among possible treatments, corticosteroids, already available from the 50’, are still the mainstay of treatment for moderate-severe disease. Nonetheless, the use of steroids is still largely empirical and solid evidence about therapeutic schemes are lacking. Moreover, due to the important side-effects and for the unsatisfactory impact on long-term natural history of disease, the steroid sparing has become an important therapeutic goal in IBD management. Besides conventional steroids, the so called “low bioavailability” steroids, which are steroids with high affinity for peripheral receptors and elevated hepatic first-pass metabolism, have demonstrated efficacy and more favorable safety profile. In the present review of the literature evidence of efficacy and safety of conventional and low bioavailability steroids in IBD patients are evaluated, and practical suggestions for a correct use in clinical practice are presented according to the current clinical guidelines.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
A Azirar ◽  
A Ghannam ◽  
A Elaouli ◽  
M Rkain ◽  
N Benaajiba ◽  
...  

Abstract Background Polyarteritis nodosa is a necrotizing vasculitis of small and medium caliber arteries. Rarely described in children, its pathophysiology is complex and remains poorly elucidated. Two main forms were described in the literature: cutaneous and visceral. Material and results We report the cases of two boys, aged of 7 and 11 years old. They had a history of recurrent sore throat with a recurrent aphthosis in the 11-year-old child. Both boys presented with polyarthralgias, myalgias, polymorphous skin lesions made of livedo reticularis with subcutaneous nodosa on the lower limbs in the first child and distal necrosis of the toes with oedema of the lower limbs in the second. Patients suffered also of cough evolving in a context of alteration of the general state made of fever and asthenia. A biological inflammatory syndrome was present in both children and the skin histology confirmed periarteritis nodosa. The evolution was marked by the disappearance of arthralgias and myalgias after a corticosteroid-based treatment with progressive dose reduction until minimal effective dose. Conclusion The diagnosis of Polyarteritis nodosa should be made in any child presenting with the following signs: fever, altered general condition, myalgias, arthralgias and skin manifestations. The prognosis is usually benign but long-term surveillance is necessary.


2021 ◽  
Vol 9 (5) ◽  
pp. 1131-1135
Author(s):  
Prasanna N Mogasale ◽  
Radhika. C ◽  
Nagaraj S

Erythroderma also known as generalized Exfoliative dermatitis which refers to a scaling involving 90% or more of the cutaneous surface. Clinicians are challenged to find the cause of exfoliative dermatitis by eliciting history of illness prior to erythema. Patients presenting acutely with exfoliative dermatitis often require admission because their total body functions can require monitoring. In western medicine, the principle management is to maintain skin moisture, avoid scratching, apply topical steroids; prolonged glucocorticoids therapy often is needed. From Ayurvedic perspective it could be correlated to Eka Kusta. Eka Kusta is the Vata Kapha Pradhana Vikara. Where skin lesions are Mahavastu, Masthyashaklopamam, Krisha Aruna in Varna. Here presenting a case of 48 years old female with erythroderma who was on long- term steroidal therapy. Treatment was planned with Shamana line of management based on her Bala. The treatment is carried out for one and half month in OPD and IPD section, all the western medicine was stopped and there was total remission of symptoms with Shamana Aushadhis. Keywords: Erythroderma, Eka Kusta, Shamana Aushadhis


CNS Spectrums ◽  
2004 ◽  
Vol 9 (2) ◽  
pp. 110-119 ◽  
Author(s):  
Patrick Kwan ◽  
Martin J. Brodie

AbstractAlthough modern community-based studies have shown that a majority of people with newly diagnosed epilepsy will enter long-term remission, seizures remain refractory to treatment in a substantial proportion of this population—perhaps as much as 40%. A consensus is being reached that, for operational purposes, pharmacoresistance can be suspected when two appropriately chosen, well-tolerated, first-line antiepileptic drugs (AEDs) or one monotherapy and one combination regimen have failed due to lack of efficacy. Poor prognostic factors include lack of response to the first AED, specific syndromes, symptomatic etiology, family history of epilepsy, psychiatric comorbidity, and high frequency of seizures. These observations suggest that prognosis can often be determined early in the course of the disorder. We propose a management paradigm that aims to maximize the chance of successful AED therapy, including the early use of “rational polytherapy” for patients not responding to monotherapy, and to identify efficiently patients suitable for “curative” resective surgery, in particular those with mesial temporal lobe epilepsy. An orderly approach to each epilepsy syndrome will optimize the chance of perfect seizure control and help more patients achieve a fulfilling life.


1985 ◽  
Vol 62 (3) ◽  
pp. 321-323 ◽  
Author(s):  
John A. Jane ◽  
Neal F. Kassell ◽  
James C. Torner ◽  
H. Richard Winn

✓ The authors summarize the findings of previous studies relating to the natural history of aneurysms and arteriovenous malformations (AVM's). Ruptured aneurysms have their highest rate of rebleeding on Day 1, and at least 50% will rebleed during the 6 months after the first hemorrhage. Thereafter, the rate drops to at least 3% a year. This is the same rate as seen in anterior and posterior communicating artery aneurysms treated by anterior cerebral artery clipping and carotid ligation; these operations provide immediate protection but do not result in long-term diminution of the risk of rebleeding. Patients with unruptured incidental and unruptured multiple aneurysms rebleed at a rate of 1% per year, as do patients with subarachnoid hemorrhage of unknown etiology. The risk of rebleeding for AVM's is 3% a year.


2006 ◽  
Vol 42 (6) ◽  
pp. 414-423 ◽  
Author(s):  
Yu-Hsin Lien ◽  
Hui-Pi Huang ◽  
Pen-Heng Chang

Iatrogenic hyperadrenocorticism is an extremely rare condition in cats. Twelve cats with a medical history of progressive skin lesions and long-term treatment with corticosteroids were retrospectively studied. Noncutaneous signs in the cats were variable and included anorexia, lethargy, polydipsia, polyuria, and atrophy of the thigh muscles. Laboratory abnormalities included leukocytosis, elevated alanine aminotransferase levels, and hyperglycemia. Transient diabetes mellitus was a secondary complication in four cats, and transient hypothyroidism was suspected in four cats. The mean time for regression of signs was 4.9 months after corticosteroid withdrawal.


2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Elizabeth A. Brezinski ◽  
Maxwell A. Fung ◽  
Nasim Fazel

Importance. Cutaneous and systemic plasmacytosis are rare conditions of unknown etiology with characteristic red-brown skin lesions and a mature polyclonal plasma cell infiltrate within the dermis. Perineural plasma cell infiltrates may be a histologic clue to the diagnosis of cutaneous plasmacytosis.Observations. Our patient had a five-year history of persistent reddish-brown plaques on the neck and trunk without systemic symptoms. Histologic examination showed dermal perivascular and perineural plasma cells with excess lambda light chain expression. Due to decreased quality of life caused by his skin lesions, he was placed on a chemotherapeutic regimen with bortezomib.Conclusions and Relevance. The patient was diagnosed with cutaneous plasmacytosis based on classic histopathology results with a recently characterized pattern of perineural involvement. Bortezomib therapy was initiated to manage his skin eruption, which has not been previously described as a treatment for this chronic condition.


2021 ◽  
Vol 12 (4) ◽  
pp. 468-469
Author(s):  
Natsuko Matsumura ◽  
Toshiyuki Yamamoto

Sir, Lichen planus (LP) is categorized as a chronic inflammatory skin disease of unknown etiology that involves immune reactions. It is characterized by flat-topped, polygonal, violaceous papules and plaques. It has various clinical presentations, such as classical LP, hypertrophic LP, LP pigmentosus, and linear LP (LLP). Primary aldosteronism (PA) is known to pose a higher risk of causing multiple autoimmune diseases [1]. Herein, we report a case with LLP and PA present at the same time. A 72-year-old Japanese female presented herself to our hospital with a three-month history of slightly itchy skin lesions on the lower right leg. A physical examination revealed flat-topped plaques on the lower right limb extending from the middle of the leg to the dorsum of the foot (Fig. 1a). There was no oral or nail involvement. The patient had a history of hypertension from the age of 62 years and was diagnosed with PA afterward. The patient had been treated with an antihypertensive drug since then without change in internal medication. The patient had no history of a preceding trauma, dental metal fillings, hepatitis, metastatic cancer, or any other infections. A histopathological examination of a skin biopsy from a lesion on the right leg revealed hyperkeratosis, a saw-tooth appearance of the epidermis, and severe liquefaction degeneration. A band-like lymphocytic infiltration was present in the upper dermis (Figs. 2a and 2b), as well as lichenoid infiltration into the dermis composed of CD4+ and CD8+ T lymphocytes. Predominantly, CD8+ T lymphocytes infiltrated into the epidermis (Figs. 2c and 2d). Clinical and histological findings confirmed the diagnosis of LPP.


Author(s):  
Lesa Dawman ◽  
Anit Kaur ◽  
Ritambhra Nada ◽  
Soumalya Chakraborty ◽  
Sanjeev Handa ◽  
...  

AbstractNephrotic syndrome (NS) associated with autosomal recessive congenital ichthyosis (ARCI) is a rare association. We describe a 4-year-old boy with steroid-resistant NS (SRNS) who had a history of ichthyotic skin lesions since birth. Renal biopsy revealed focal segmental glomerulosclerosis (tip variant). The skin biopsy was consistent with the findings of ichthyosis. Next-generation sequencing revealed a homozygous pathogenic variant (c.1625_1626del) in the exon 12 of the ALOX12B gene, confirming the diagnosis of ARCI2. The ALOX12B gene belongs to the lipoxygenase family and has a pivotal role in the formation of lipid layers in the epidermis. Leukotrienes have a counter-regulatory effect within the inflamed glomeruli, which influences the vascular tone and glomerular basement membrane permeability, that can be implicated in the pathogenesis of the NS. This child is currently in remission, on tacrolimus and low-dose prednisolone, with emollients and is on regular follow-up. SRNS associated with congenital ichthyosis secondary to a mutation in the ALOX12B gene has never been reported so far. The knowledge regarding this novel association will help the treating physicians in diagnosing this condition early, which will enable proper genetic counseling and prognostication of the disease to the family.


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