Citrobacter freundii sepsis in an immunosuppressed patient with pemphigus vulgaris

Pemphigus vulgaris is an autoimmune bullous disease that involves the skin and mucous membranes. Current therapies aim to decrease antibody production by means of the use of systemic corticosteroids, immunosuppressive agents and, recently, rituximab, an anti-CD20 monoclonal antibody. However, the chronic immune suppression could entail complications, like infections and secondary malignancies. We describe a case of a patient with pemphigus who developed a sepsis due to Citrobacter freundii infection.

Download Full-text

Severe Multi-Resistant Pemphigus vulgaris: prolonged remission with a single cycle of Rituximab

Anais Brasileiros de Dermatologia ◽

10.1590/abd1806-4841.20131990 ◽

2013 ◽

Vol 88 (4) ◽

pp. 639-642 ◽

Cited By ~ 7

Author(s):

Isabela Soubhia Corral ◽

Thais Helena Proença de Freitas ◽

Renata Telles Rudge de Aquino ◽

Daniella Abbruzzini S. Koller ◽

Maria Elisa Ruffolo Magliari ◽

...

Keyword(s):

Pemphigus Vulgaris ◽

Immunosuppressive Drugs ◽

Direct Immunofluorescence ◽

Clinical Relapse ◽

Single Cycle ◽

Bullous Disease ◽

Autoimmune Bullous Disease ◽

Systemic Corticosteroids ◽

Daily Dose

Pemphigus vulgaris is an autoimmune bullous disease whose therapy is based on systemic corticosteroids, with or without immunosuppressants. Rituximab is a chimeric monoclonal antibody of the IgG class, directed at a specific CD20 B cell surface antigen, used in pemphigus vulgaris empirically since 2002, with success in 90% of the cases and long periods of remission. Male patient, 33 years old, diagnosed with pemphigus vulgaris, confirmed by histopathology and direct immunofluorescence. He was treated for seven months with numerous treatments, including immunosuppressive drugs, with an unsatisfactory response, until he had complete remission with the use of rituximab. During a 34-month follow-up period, the patient presented a slight clinical relapse, which was successfully controlled with prednisone in a daily dose of 120mg, soon reduced to 20mg.

Download Full-text

Pemphigus vulgaris in pregnancy with transient neonatal pemphigus - can psychological stress be culprit?

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20174469 ◽

2017 ◽

Vol 6 (10) ◽

pp. 4703

Author(s):

Nisha Bhagat ◽

Rajnish Raj ◽

Ritu Arora

Keyword(s):

Serotonin Reuptake ◽

Genetic Linkage ◽

Pemphigus Vulgaris ◽

Topical Steroids ◽

Second Trimester ◽

Bullous Disease ◽

Autoimmune Bullous Disease ◽

Life Threatening ◽

Peripartum Depression ◽

In Pregnancy

Pemphigus vulgaris (PV) is a rare life threatening, autoimmune bullous disease affecting mucous membrane and skin. Pregnancy and emotional stress may be the possible triggering factors for PV. It is a very rare phenomenon with less than 27 cases of pregnancy induced PV and only 9 cases of neonatal pemphigus reported in literature. The lesions may appear transient in neonate which resolve spontaneously over 2-3 weeks. We hereby, report a case of 26-year-old female who developed PV in second trimester of pregnancy with peripartum depression, which was managed by intravenous, oral and topical steroids and selective-serotonin reuptake inhibitor (SSRI). Furthermore, the development of transient neonatal pemphigus raises the possibility of genetic linkage from maternal to fetal transmission.

Download Full-text

Paraneoplastic Pemphigus: A Case Report

Nepal Journal of Dermatology Venereology & Leprology ◽

10.3126/njdvl.v16i1.19412 ◽

2018 ◽

Vol 16 (1) ◽

pp. 57-58

Author(s):

Nidhi Shah ◽

Rosina Paudel ◽

Arpana Rijal

Keyword(s):

Pemphigus Vulgaris ◽

Lymphoproliferative Disorders ◽

Provisional Diagnosis ◽

Diagnostic Screening ◽

Course Of Disease ◽

Paraneoplastic Pemphigus ◽

Bullous Disease ◽

Autoimmune Bullous Disease ◽

History Of ◽

Characteristic Features

Paraneoplastic pemphigus (PNP) is a rare autoimmune bullous disease associated with underlying neoplasms. Targetoid lesions, intractable stomatitis and refractory course of disease are some of the characteristic features of paraneoplastic pemphigus. It is usually associated with lymphoproliferative disorders, and rarely with solid tumors. We present a case of a 35 years old female with a six-months history of recurrent, severe, recalcitrant stomatitis and widespread blistering and erosions with involvement of palms and soles. A provisional diagnosis of PNP was made based on clinical features. Investigations for an underlying neoplasm revealed presence of solid lesion of size 6.7 x 6.4 cm in left adnexa, likely an ovarian tumor. PNP should be considered in the differential diagnoses in severe atypical mucocutaneous manifestations of pemphigus vulgaris and diagnostic screening for search for an underlying tumor should be performed.

Download Full-text

Management of Pemphigus Vulgaris in Pregnancy - Case Report

Journal of Gynecology & Reproductive Medicine ◽

10.33140/jgrm.03.04.01 ◽

2019 ◽

Vol 3 (4) ◽

Keyword(s):

Case Report ◽

Autoimmune Disease ◽

Pemphigus Vulgaris ◽

Clinical Findings ◽

Moderate Intensity ◽

Primary Target ◽

Bullous Disease ◽

Autoimmune Bullous Disease ◽

Homophilic Adhesion ◽

In Pregnancy

Pemphigus vulgaris is a rare autoimmune bullous disease caused by producing autoantibodies directed against transmembrane glycoproteins of desmosomes that can lead to steric hindrance to homophilic adhesion of desmogleins. The development of pemphigus during the pregnancy is rare. The disruption of the Th1:Th2 balance in the autoimmune disease during the pregnancy plays a crucial role and explains why some studies have reported the exacerbation of disease, while others observe improvements during the pregnancy. We present aclinical case of a 31-year-oldprimigravida, with dichorionic - diamniotic twin pregnancyassociatedwith pemphigus vulgaris, being discovered 2 years ago, who went under treatment with Medrol 64mg/day and Imuran 50mg/day. She was admitted to the hospital at 31 weeks of gestation with non-systemic contractions of moderate intensity. The clinical findings are multiple erosions and blisters which have been accentuated during the second semester and have been remitted after delivery. Nowadays, the therapeutic management of the pemphigus during the pregnancy is not yet established and all of these cases are individual evaluated with the primary target to control the disease with the safety of the fetus.

Download Full-text

An Atypical Presentation of Pemphigus Vegetans in the Umbilicus

SKIN The Journal of Cutaneous Medicine ◽

10.25251/skin.4.5.12 ◽

2020 ◽

Vol 4 (5) ◽

pp. 452-455

Author(s):

Antonio Jimenez ◽

Paige Hoyer ◽

Michael Wilkerson

Keyword(s):

African American Woman ◽

American Woman ◽

Atypical Presentation ◽

Treatment Resistant ◽

Oral Ulcers ◽

Bullous Disease ◽

Autoimmune Bullous Disease ◽

Mucous Membranes ◽

Pemphigus Vegetans ◽

Desmoglein 3

Pemphigus is a chronic, autoimmune bullous disease that affects the skin and mucous membranes. Pemphigus vegetans is a rare variant of pemphigus and presents as oral ulcerations with associated verrucous lesions in intertriginous or flexural areas. A 38-year-old African American woman presented to the clinic with a chief complaint of oral ulcers. She carried a diagnosis of Behcet’s disease and was referred by rheumatology for evaluation of treatment-resistant mucosal ulcerations. At the time of her dermatology visit, she also reported an enlarging umbilical mass that had been present for several months. Further examination of the umbilical lesion identified an exophytic, vegetative mass. Histologic assessment of the lesion identified acanthosis and acantholysis with dermal eosinophils consistent with pemphigus vegetans. A pemphigus antibody panel was done and resulted positive for IgG desmoglein-3 antibodies. The patient was treated with prednisone and rituximab with improvement of her lesions. We present an atypical presentation of pemphigus vegetans involving the umbilicus. This diagnosis should be considered in patients who present with oral erosions and concomitant vegetative lesions, regardless of location or prior diagnoses.

Download Full-text