scholarly journals Rowell syndrome – a clinical entity that must not be forgotten

2016 ◽  
Vol 63 (4) ◽  
pp. 331-333
Author(s):  
Maria Daniela Tănăsescu ◽  
◽  
Mihai Ovidiu Comşa ◽  
Alexandru Mincă ◽  
Alexandru Cristian Diaconescu ◽  
...  
Keyword(s):  
Erythema Multiforme ◽  
Lupus Erythematosus ◽  
Rare Condition ◽  
Skin Lesions ◽  
Pulse Therapy ◽  
Therapeutic Regimen ◽  
Discoid Lupus Erythematosus ◽  
Cutaneous Lesions ◽  
High Doses ◽  
Minor Criteria

Objective. Rowell Syndrome represents a rare condition, characterised by the existence of erythema multiforme with lupus erythematosus. The diagnosis is made in patients which present both the major criteria (coexistence of discoid lupus erythematosus with erythema multiforme lesions and positivity for speckled - pattern antinuclear antibodies) and at least one of the minor criteria (frostbites-like lesions, positive anti-La or anti-Ro antibodies and reactive rheumatoid factor). The main objective of this article is to evidence a rare case of Rowell Syndrome and the optimal diagnostic and therapeutic management involved for resolving this case. Material and method. This article presents the case of a 47-year-old patient diagnosed with Rowell Syndrome, which met the diagnostic criteria. In her case, we used a therapeutic regimen comprising Hydroxychloroquine 400 mg/day and pulse-therapy with methylprednisolone 1 gram/day, for 3 days. Results. The decremental titration of prednisone resulted in reoccurrence of cutaneous lesions, prompting the return to the high doses, with normalisation of skin lesions. Conclusions. The presented case highlights the importance of keeping in mind the diagnosis of Rowell Syndrome in all the situations of patients with serologic profile of lupus and erythema multiforme lesions. Discussion. The therapeutic regimen and evolution under treatment may have a variable course, imposing the monitoring of patients a course for preventing the disease relapse.

scholarly journals HOMOLOGOUS DISEASE IN THE ADULT RAT, A MODEL FOR AUTOIMMUNE DISEASE

1963 ◽  
Vol 118 (4) ◽  
pp. 635-648 ◽  
Author(s):  
Peter Stastny ◽  
Vernie A. Stembridge ◽  
Morris Ziff
Keyword(s):  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Skin Lesions ◽  
Cutaneous Lesions ◽  
Adult Rats ◽  
Hydropic Degeneration ◽  
Skin Changes ◽  
Adult Rat ◽  
The One

The cutaneous lesions of adult rats with homologous disease are described, and evidence is presented to indicate that they have an immunologic basis. The skin changes included erythema, purpura, edema, and a variety of inflammatory lesions. In the more active lesions, dermal infiltration, hydropic degeneration, acanthosis, and atrophy of the epidermis with hyperkeratosis and follicular plugging were present. In some cases, ulceration and sloughing were also observed. More chronic lesions were characterized by atrophy of the epidermis and collagenization of the dermis with disappearance of the skin appendages. Rejection of autografts was observed simultaneously with acceptance of homografts. The histologic appearance of autografts undergoing rejection was similar to that of the spontaneous skin lesions, suggesting that the latter, too, had an immunologic basis. In favor of this, also, was the specificity of the dermatitis for the skin of the host, with sparing of neighboring homograft tissue. There was a histologic similarity between the spontaneous skin lesions of homologous disease and those of lupus erythematosus on the one hand, and scleroderma on the other, thus supporting the possibility that the cutaneous lesions of these connective tissue diseases of man may also have an immunologic basis. It was concluded that the adult rat with homologous disease may furnish a model for human autoimmune disease.

scholarly journals Clinical profile of cutaneous manifestations of connective tissue diseases in North-East India

2021 ◽  
Vol 7 (5) ◽  
pp. 702
Author(s):  
Deepa Mala Subba ◽  
Nandakishore Thokchom ◽  
Linda Kongbam ◽  
Erika Salam ◽  
Deepa Yumnam
Keyword(s):  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Cutaneous Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Skin Lesions ◽  
Discoid Lupus Erythematosus ◽  
Serological Tests ◽  
Cross Sectional ◽  
Cutaneous Manifestations ◽  
North East

<p class="abstract"><strong>Background:</strong> Connective tissue diseases (CTDs) are a heterogeneous group of autoimmune disorders having overlapping clinical features. Skin is often involved and it may be the earliest sign of the disease. This study highlighted the various cutaneous manifestations of common CTDs.</p><p class="abstract"><strong>Methods:</strong> A hospital-based cross-sectional study was carried out for a period of two years in 83 patients with CTDs in dermatology OPD, RIMS, Imphal. Detailed history taking, examination and relevant serological tests were performed.<strong></strong></p><p class="abstract"><strong>Results:</strong> The mean age was 39.78±17.29 years with female to male ratio of 4.5:1. Majority of the patients had lupus erythematosus (LE) (N=45) followed by systemic sclerosis (SSc) (N=25), rheumatoid arthritis (RA) (N=6), mixed connective tissue disease (MCTD) (N=4) and morphea (N=3). The most common presentation was raised skin lesions (45.8%) followed by Raynaud’s phenomenon (36.1%), photosensitivity (27.7%), skin tightness (26.5%) and joint pain (19.3%). Among LE patients, chronic cutaneous lupus erythematosus (CCLE) was the commonest variant and localised discoid lupus erythematosus (DLE) (22.9%) was the commonest presentation followed by malar rash and annular subacute lupus erythematosus (SCLE). Skin induration, microstomia and sclerodactyly were seen in most patients of SSc. Antinuclear antibodies were positive in 89.1% of patients. Anti-dsDNA and anti-Sm antibodies were positive in 62.2% and 33.3% of LE patients, anti-Scl 70 antibody was positive in 68% of SSc patients.</p><p class="abstract"><strong>Conclusions:</strong> CTDs are rare but potentially life-threatening. Proper understanding of the spectrum of cutaneous manifestations of CTDs is therefore necessary for early diagnosis and efficient management.</p>

Calciphylaxis in a Patient With Non-Hodgkin Lymphoma: Case Report and Literature Review

2018 ◽  
Vol 22 (5) ◽  
pp. 524-526 ◽  
Author(s):  
Meggie Morand ◽  
Myrna Chababi-Atallah ◽  
Sara-Élizabeth Jean ◽  
Carolina Fernandes
Keyword(s):  
Systematic Review ◽  
Hodgkin Lymphoma ◽  
Rare Condition ◽  
Skin Lesions ◽  
Review Of The Literature ◽  
End Stage Kidney Disease ◽  
Cutaneous Lesions ◽  
Non Hodgkin Lymphoma ◽  
End Stage ◽  
High Index Of Suspicion

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.

scholarly journals Successional occurrence of two papulosquamous disorders in HIV positive patient: a rare case report

2021 ◽  
Vol 7 (6) ◽  
pp. 869
Author(s):  
Nitika Sanjay Deshmukh ◽  
Ravindranath Brahmadeo Chavan ◽  
Anil Prakash Gosavi ◽  
Supriya Ashok Kachare
Keyword(s):  
Case Report ◽  
Lichen Planus ◽  
Lupus Erythematosus ◽  
Rare Condition ◽  
Discoid Lupus Erythematosus ◽  
Rare Presentation ◽  
Rare Case Report ◽  
Autoimmune Conditions ◽  
Atypical Presentations ◽  
High Index Of Suspicion

<p class="abstract">Presentation of two papulosquamous disorders in a same individual is rare condition till date. Independently, psoriasis and Lichen planus (LP) are common inflammatory skin conditions affecting around 2-3% and 1% of HIV (Human immune deficiency) positive population respectively. As reviewed in the literature, psoriasis may be independently associated with other autoimmune conditions like vitiligo, alopecia areata, lichen planus, and discoid lupus erythematosus. In this article, we presented a case report of a HIV seropositive patient who suffered from psoriasis and lichen planus. The coexistence of psoriasis and lichen planus in one individual is rare and underreported in literature. Psoriasis or lichen planus may be the presenting feature of HIV infection and tends to be more severe, to have atypical presentations. Psoriasis and lichen planus can be coexistent or successionally appear one after other in one individual though rare presentation. High index of suspicion is always required while dealing with papulosquamous lesions in PLHIV.</p><p> </p>

Paramyxovirus-like Structures in Lupus and Dermatomyositis

1969 ◽  
Vol 27 ◽  
pp. 222-223 ◽  
Author(s):  
Ken Hashimoto
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Skin Lesions ◽  
Collagen Disease ◽  
Discoid Lupus Erythematosus ◽  
Systemic Lupus ◽  
Collagen Diseases ◽  
Identical Structure

Paramyxovirus-like structures in so-called collagen disease have been reported in polymyositis and systemic lupus erythematosus. The identical structure was found in the present study in the skin lesions of discoid lupus erythematosus and dermatomyositis; these two are also called collagen diseases. This report deals with detailed ultrastructure and some aspects of the mode of formation of this structure as they are seen in the skin lesions of discoid lupus and dermatomyositis.

Concurrent presentation of cutaneous lesions of deep linear morphoea and discoid lupus erythematosus

Lupus ◽  
2015 ◽  
Vol 25 (2) ◽  
pp. 204-208 ◽  
Author(s):  
C Bernárdez ◽  
L Prieto-Torres ◽  
E Macías ◽  
J L Ramírez-Bellver ◽  
R Haro-Ramos ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Discoid Lupus Erythematosus ◽  
Cutaneous Lesions

scholarly journals Utilization of Rituximab for Refractory Rowell Syndrome

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Sukhraj Singh ◽  
Sandra Sheffield ◽  
Nisha Chowdhury ◽  
Swetha Nuthulaganti ◽  
Zareen Vaghaiwalla ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Erythema Multiforme ◽  
Lupus Erythematosus ◽  
Cell Activation ◽  
Standard Of Care ◽  
Mast Cell Activation ◽  
Erythematous Rash ◽  
Systemic Lupus ◽  
Cutaneous Lesions ◽  
Excellent Response

Rowell syndrome describes the occurrence of erythema multiforme-like lesions in patients with cutaneous lesions of lupus erythematosus. The clinical picture of atypical erythema multiforme-like lesions, presence of chilblains, speckled ANA pattern, anti-Ro/SSA, or anti-La/SSB antibodies, and absence of infectious or pharmacologic triggers in a patient with systemic lupus erythematosus are some of the classic clinical and serologic features. Histopathologic and serologic findings can help differentiate this process from erythema multiforme. We present a case of young woman with systemic lupus erythematosus, end-stage renal disease due to lupus nephritis, and a remote history of Steven–Johnson syndrome due to sulfa allergy who presented to the hospital with a recurrent, progressive, targetoid erythematous rash involving more than 60% of her body surface area. Our patient had several hospitalizations in the recent past for this erythematous rash and had failed oral therapy with prednisone 1 mg/kg and hydroxychloroquine. In view of the minimal improvement and increasing severity and patient exhibiting early features of mast cell activation syndrome, the patient was treated with pulse intravenous glucocorticoids followed by rituximab with an excellent response. We highlight a unique case report of progressive Rowell syndrome refractory to standard of care with an excellent response to rituximab.

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