A Case of Acute Pemphigus Vulgaris Relapses Associated with Cocaine Use and Review of the Literature

We present the clinical case of the patient, followed for a total of 86  weeks, with ten relapses secondary to probable cocaine use. The patient was admitted to the emergency department after presenting polymorphic dermatosis characterized by blisters, vesicles, and excoriations extending from the oral cavity to the thorax, and to the inguinal and genital regions, aff ecting approximately 35 % of the body surface area with a score of 56 on the Pemphigus Skin Disorder Index. Skin biopsies were compatible with PV diagnosis. The patient had clinical improvement with a combination of methylprednisolone 500 mg intravenously (IV) and cyclophosphamide 500 mg IV every 15 days, along with prednisone 50 mg orally (PO) q24 h and mycophenolic acid 500 mg PO q6 h. Persistent cocaine use is highly likely to be the factor triggering lesion reactivation and responsible for the torpid evolution. We cannot definitively conclude whether the change from azathioprine to mycophenolic acid after the tenth relapse was the adjuvant medication responsible for the end of the consolidation phase and complete remission on therapy. This case study could potentially serve as a guide for management of patients who continuously persist with cocaine use, leading to a clinical picture refractory to multiple therapeutic schemes.
Source: Dermatology and Therapy - Category: Dermatology Source Type: research

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Publication date: Available online 18 January 2020Source: Gynecologic Oncology ReportsAuthor(s): Bijan Morshedi, Kari RingAbstractPemphigus is a group of autoimmune intraepidermal blistering diseases caused by immunoglobulins directed against keratinocyte cell surface components. In this case report, we identify a non-classical paraneoplastic pemphigus (PNP) foliaceous related to an undifferentiated uterine sarcoma.The patient is a 54-year-old female with a past medical history of arthritis who presented with worsening fatigue in November 2017 and an itchy, blistering, erythematous annular plaque that first appeared on her...
Source: Gynecologic Oncology Reports - Category: OBGYN Source Type: research
ConclusionsAdjunctive procedures that can facilitate a decrease in the cumulative dosages of corticosteroids and immunosuppressants have great value in the management of PV. CO2 laser vaporization is a safe procedure with minimal morbidity and no long-term side effects and should be considered as an adjunctive treatment option for management of recalcitrant lesions in patients with oral PV.
Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology - Category: ENT & OMF Source Type: research
AbstractPemphigus vulgaris (PV) is a rare autoimmune blistering disease involving the skin and mucous membranes. The prevalence of esophageal involvement remains uncertain. The aim of our study was to determine the frequency of esophageal involvement in patients with PV. This is a single-center electronic database retrospective review of patients with a diagnosis of PV. Data abstracted included demographics, disease characteristics (biopsy results, symptoms, areas affected, treatments), and esophagogastroduodenoscopy (EGD) reports. Of the 111 patients that met eligibility criteria, only 22 (19.8%) underwent EGD. Demographi...
Source: Dysphagia - Category: Speech-Language Pathology Source Type: research
Publication date: 7–13 September 2019Source: The Lancet, Volume 394, Issue 10201Author(s): Enno Schmidt, Michael Kasperkiewicz, Pascal JolySummaryPemphigus consists of a group of rare and severe autoimmune blistering diseases mediated by pathogenic autoantibodies mainly directed against two desmosomal adhesion proteins, desmoglein (Dsg)1 and Dsg3 (also known as DG1 and DG3), which are present in the skin and surface-close mucosae. The binding of autoantibodies to Dsg proteins induces a separation of neighbouring keratinocytes, in a process known as acantholysis. The two main pemphigus variants are pemphigus vulgaris,...
Source: The Lancet - Category: General Medicine Source Type: research
Autoimmune bullous dermatoses (AIBD) are characterized by circulating autoantibodies that are either directed against epidermal antigens or deposited as immune complexes in the basement membrane zone. The complement system (CS) can be activated by autoantibodies, thereby triggering activation of specific complement pathways. Local complement activation induces a pathogenic inflammatory response that eventually results in the formation of a sub- or intraepidermal blister. Deposition of complement components is routinely used as a diagnostic marker for AIBD. Knowledge from different animal models mimicking AIBD and depositio...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Paraneoplastic autoimmune multi-organ syndrome (PAMS) is a rare clinical condition characterized by variable and heterogeneous clinical phenotypes in the presence of neoplasias which largely depend on the activation of humoral and cellular immune responses. Clinically, these patients present with a spectrum of pemphigus-like lesions to graft-vs-host-disease-like exanthemas with a lichenoid inflammatory infiltrate in the skin. PAMS is occasionally associated with thymoma. We here present a patient with relapsing thymoma who developed PAMS with several cutaneous and extracutaneous autoimmune disorders. Peripheral blood lym...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Authors: Kiritsi D, Schauer F Abstract Autoimmune blistering skin disorders represent a rare group of autoantibody-induced dermatoses against desmosomal and hemidesmosomal molecules. The common age of onset for pemphigus and pemphigoid, as well as dermatitis herpetiformis, encompasses the adult age, but all these disorders can be observed neonatally and/or during childhood. If the disease occurs postpartum or neonatally, physicians should consider transplacental transmission of pathogenic maternal immunoglobulin G (IgG)-autoantibodies, and both mother and child should be included in the diagnostic work up. If ...
Source: Der Hautarzt: Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete - Category: Dermatology Tags: Hautarzt Source Type: research
ConclusionIntractable mucocutaneous lesions with a concurrent tumor in children strongly indicate PNP resulting from CD. Because stomatitis or skin erosion may be the first presentation, mucocutaneous tissue biopsy and early detection of the underlying tumor are important. Earlier diagnosis is mandatory for the effective treatment of PNP and pulmonary involvement.
Source: APLAR Journal of Rheumatology - Category: Rheumatology Authors: Tags: ORIGINAL ARTICLE Source Type: research
ConclusionsWith the presence of multiorgan lesions in patients with lymphoproliferative neoplasia, paraneoplastic pemphigus should always be considered among the possible diagnostic hypotheses, because diagnosis and early treatment may allow a better prognosis for the patient.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
CONCLUSION AND CLINICAL IMPORTANCE: CRP levels are increased in dogs with PF and exceed levels found in most dogs with SP. PMID: 29168248 [PubMed - as supplied by publisher]
Source: Veterinary Dermatology - Category: Veterinary Research Authors: Tags: Vet Dermatol Source Type: research
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