Anterior scleritis in a patient of pemphigus vulgaris while on immunosuppressive treatment

Purpose: The aim of the study was to report the first case of a patient with Terrien’s Marginal Degeneration (TMD) who developed necrotizing anterior scleritis without systemic disease association, requiring systemic immunosuppressive treatment. Case Report: A 32-year-old female consulted for bilateral ocular burning and hyperemia. Initially, she was diagnosed with conjunctivitis and treated with topical antibiotics and corticosteroids, with mild transitory improvement but the progression of the disease. Years later, she attended the ocular immunology consultation for a second opinion where TMD with ocular inflammatory component OU was diagnosed. Seven months later, she presented with severe pain, decreased visual acuity, and photophobia in OS. At the slit-lamp examination, necrotizing anterior scleritis with a high risk of perforation in OS was observed. The patient was referred to the rheumatologist and started treatment with systemic corticosteroids and cyclophosphamide, exhibiting a clinical improvement. The patient did not meet the criteria for any systemic illness associated with scleritis, such as autoimmune diseases or vasculitis. Thus, scleritis was related to the adjacent inflammatory process associated with TMD, as an atypical presentation of this disease. Conclusion: Although an inflammatory type of TMD has been proposed, it is essential to follow up closely these patients and consider necrotizing anterior scleritis, a severe ocular disease that requires prompt immunosuppressive management, as a possible atypical associated presentation of this disease.

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Case Report: Management of Malignancy-Exacerbated Pemphigus Vulgaris During COVID-19 Pandemic

Frontiers in Medicine ◽

10.3389/fmed.2021.708284 ◽

2021 ◽

Vol 8 ◽

Author(s):

Alberto Corrà ◽

Francesca Cammelli ◽

Lavinia Quintarelli ◽

Giuseppe Barbato ◽

Ornella Le Rose ◽

...

Keyword(s):

Immunosuppressive Treatment ◽

Surgical Excision ◽

Pemphigus Vulgaris ◽

Intravenous Immunoglobulins ◽

Diagnostic Process ◽

Trigger Factors ◽

Blistering Disease ◽

Oncologic Diseases ◽

Bridge Therapy ◽

Rule Out

Pemphigus vulgaris is an intraepidermal autoimmune mucocutaneous blistering disease whose etiopathogenesis includes various trigger factors, i.e., drugs and malignancies. We present a case of malignancy-exacerbated pemphigus vulgaris which required a careful diagnostic process in order to rule out paraneoplastic pemphigus, along with the challenges posed by the need of treating both cutaneous and oncologic diseases. Possible post-operative complications post-poned the start of first-line immunosuppressive treatment of pemphigus. Moreover, the infective risks had to be minimized during the peak of the COVID-19 pandemic in Italy. Intravenous immunoglobulins were chosen as “bridge” therapy before the tumor surgical excision, followed by rituximab in post-operative phase.

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Visceral Leishmaniasis in a Patient with Immunosuppressive Treatment and Pancytopenia Due to Pemphigus Vulgaris

LLM Dergi ◽

10.5578/llm.67574 ◽

2018 ◽

Vol 2 (4) ◽

pp. 98-100

Author(s):

İlker BAY ◽

Zekeriya AKSÖZ ◽

Numan KAVAZ ◽

Rahşan YILDIRIM

Keyword(s):

Visceral Leishmaniasis ◽

Immunosuppressive Treatment ◽

Pemphigus Vulgaris

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Difficulties in the treatment of recurrent juvenile laryngeal papillomatosis associated with pemphigus vulgaris

Romanian Journal of Rhinology ◽

10.2478/rjr-2020-0009 ◽

2020 ◽

Vol 10 (38) ◽

pp. 38-44

Author(s):

Codrut Sarafoleanu ◽

Gheorghe Lapicus ◽

Ana Isabela Lupu ◽

Vlad Budu

Keyword(s):

Surgical Intervention ◽

Immunosuppressive Treatment ◽

Autoimmune Disorder ◽

Pemphigus Vulgaris ◽

Human Papillomaviruses ◽

Laryngeal Papillomatosis ◽

Viral Multiplication ◽

Immunosuppressive Medication ◽

Cellular Dna ◽

Immunosuppressive Cells

AbstractRecurrent laryngeal papillomatosis is a disease caused by human papillomaviruses (HPVs), which currently does not benefit from a curative treatment. Due to the fact that HPV has the action of modifying cellular DNA, with changes in the expression of interleukins and interferon, with insufficient maturation of T cells and intracellular overpopulation of immunosuppressive cells, the association of RRP (Recurrent Respiratory Papillomatosis) with an autoimmune disease may cause particular difficulties in the therapeutic management of patients diagnosed with RRP.Immunosuppressive medication negatively influences the development of papillomatosis, increasing the number of local relapses and, respectively, the need for surgical intervention due to the increased viral multiplication and the proliferation of papillomatous lesions. In order to exemplify the difficulties encountered in treating RRP associated with an autoimmune disorder, the authors present the case of a 21-year-old patient diagnosed with juvenile recurrent laryngeal papillomatosis genotype 6, with multiple antecedent surgeries, who was diagnosed with pemphigus vulgaris at the age of 19. The peculiarity of the case lies in the difficulty of managing the RRP associated with an immunosuppressive disorder whose therapeutic indication is cortisone and immunosuppressive treatment, which led to exacerbation of viral multiplication and proliferation of papillomatous lesions.

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Anterior Scleritis Associated with Pemphigus Vulgaris

Ocular Immunology and Inflammation ◽

10.1080/09273948.2017.1393547 ◽

2017 ◽

Vol 27 (3) ◽

pp. 497-498 ◽

Cited By ~ 2

Author(s):

Tal Zeeli ◽

Efrat Bar-Ilan ◽

Zohar Habot-Wilner ◽

Eli Sprecher

Keyword(s):

Pemphigus Vulgaris ◽

Anterior Scleritis

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Recurrence of pemphigus vulgaris after bilateral breast irradiation: a case report and review of the literature

10.21203/rs.3.rs-403140/v1 ◽

2021 ◽

Author(s):

Camille Invernizzi ◽

Victor Gaultier ◽

Philippe Guilbert ◽

Christelle Jouannaud ◽

Anne Laure Labrousse ◽

...

Keyword(s):

Breast Cancer ◽

Immunosuppressive Treatment ◽

Pemphigus Vulgaris ◽

Review Of The Literature ◽

Case Presentation ◽

Systemic Corticosteroids ◽

End Of Treatment ◽

Rare Side Effect ◽

Radiation Induced ◽

Radiotherapy Treatment

Abstract Background Pemphigus is a serious and rare chronic bullous autoimmune disease. It is characterized by mucocutaneous erosions secondary to autoantibodies directed against desmogleins 1 and 3, proteins involved in intercellular adhesion mechanisms. The occurrence of pemphigus is based on the triggering of genetic and external environmental factors such as drugs, infection and more rarely radiotherapy. To date, only 16 cases of radiation-induced pemphigus are described in a context of breast cancer treatment. Case presentation: We present the case of a 76-year-old woman who had a recurrence of pemphigus vulgaris limited to the irradiation field after exposure to an adjuvant radiotherapy treatment for a bilateral triple negative breast cancer. The onset was bilateral limited to the irradiation area and was treated effectively with local and systemic corticosteroids. After a rigorous review of the literature, only 16 cases of breast cancer radiation-induced pemphigus appeared. In contrast to several cases, the rash was limited to the irradiated area and improved with systemic corticosteroids. Conclusions Pemphigus is a very rare side effect of radiotherapy, notably in breast cancer. For more than three-quarters of the described cases in the literature, this condition occurs within three months following the end of treatment. After systemic immunosuppressive treatment, this disease disappears in the vast majority of the reported cases.

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Macular dual retinitis with Herpes simplex and Cytomegalovirus following periocular corticosteroid in a patient of Pemphigus Vulgaris

European Journal of Ophthalmology ◽

10.1177/11206721211052847 ◽

2021 ◽

pp. 112067212110528

Author(s):

Ramanuj Samanta ◽

Athul S. Puthalath ◽

Gitanjli Sood ◽

Ramandeep Singh ◽

Mohit Dogra

Keyword(s):

Herpes Simplex ◽

Corticosteroid Injection ◽

Immunosuppressive Treatment ◽

Pemphigus Vulgaris ◽

Immunosuppressive Drugs ◽

Corticosteroid Administration ◽

Cmv Retinitis ◽

Dual Positivity ◽

Simplex Virus ◽

The Right

Background Cytomegalovirus (CMV) retinitis may occur in non-HIV individuals following systemic immunosuppressive treatment or periocular corticosteroid administration. However, simultaneous multiple viral retinitis is rare in HIV-negative individuals. We report a case of dual viral retinitis in a non-HIV female on systemic immunosuppressive for pemphigus vulgaris who was administered a periocular corticosteroid injection. Method A 32-year-old female on double immunosuppressive therapy (prednisolone and cyclophosphamide) for pemphigus vulgaris, presented with gradual painless diminution of vision in the right eye for one month. She was initially diagnosed to have possible autoimmune neuroretinitis by the referring ophthalmologist and received a single injection of posterior subtenon triamcinolone acetonide for the same. Her vision however deteriorated further and she received an intravitreal ganciclovir injection with a revised diagnosis of CMV retinitis. Due to suboptimal response she was referred to us. Aqueous Polymerase chain reaction (PCR) revealed dual positivity for CMV and Herpes simplex virus. She was successfully managed with intravitreal ganciclovir injections, systemic acyclovir and tapering of systemic immunosuppressive drugs. Result The retinitis lesions resolved gradually leaving behind a pale optic disc and foveal atrophy at 12 weeks follow-up. Conclusion Infective etiology must be ruled out in immunosuppressed patients before considering periocular corticosteroids. Dual viral involvement, although rare, may cause fulminant retinitis in predisposed individuals. High index of suspicion and PCR from ocular fluids should be performed at the earliest in patients with atypical or poorly responding retinitis lesions.

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Pemphigus vulgaris-associated anterior scleritis: A case study

Middle East African Journal of Ophthalmology ◽

10.4103/meajo.meajo_442_20 ◽

2021 ◽

Vol 28 (1) ◽

pp. 66

Author(s):

Salim Alkeraye ◽

Hanan Alsalman ◽

Albanderi Alhamzah ◽

SulaimanM Alsulaiman

Keyword(s):

Pemphigus Vulgaris ◽

Anterior Scleritis

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Recurrence of Pemphigus Vulgaris after Bilateral Breast Irradiation: A Case Report and Review of the Literature

Case Reports in Oncology ◽

10.1159/000520380 ◽

2021 ◽

pp. 1733-1740

Author(s):

Camille Invernizzi ◽

Victor Gaultier ◽

Philippe Guilbert ◽

Christelle Jouannaud ◽

Anne-Laure Labrousse ◽

...

Keyword(s):

Breast Cancer ◽

Triple Negative ◽

Immunosuppressive Treatment ◽

Pemphigus Vulgaris ◽

Review Of The Literature ◽

Systemic Corticosteroids ◽

End Of Treatment ◽

Irradiation Field ◽

Radiation Induced ◽

Radiotherapy Treatment

Pemphigus is a serious and rare chronic bullous autoimmune disease. It is characterized by mucocutaneous erosions secondary to autoantibodies directed against desmogleins 1 and 3, proteins involved in intercellular adhesion mechanisms. The occurrence of pemphigus is based on the triggering of genetic and external environmental factors such as drugs, infection, and more rarely radiotherapy. To date, only 16 cases of radiation-induced pemphigus are described in the context of breast cancer treatment. We present the case of a 76-year-old woman who had a recurrence of pemphigus vulgaris limited to the irradiation field after exposure to an adjuvant radiotherapy treatment for a bilateral triple-negative breast cancer. The onset was bilateral limited to the irradiation area and was treated effectively with local and systemic corticosteroids. After a rigorous review of the literature, only 16 cases of breast cancer radiation-induced pemphigus appeared. In contrast to several cases, the rash was limited to the irradiated area and improved with systemic corticosteroids. For more than three-quarters of the described cases in the literature, pemphigus occurs within 3 months following the end of treatment. After systemic immunosuppressive treatment, this disease disappears in the vast majority of the reported cases.

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