scholarly journals Present Tense: A Peculiar Case of Pemphigus Vulgaris Presenting with Tense Blisters during the COVID-19 Pandemic

2021 ◽  
Vol 55 (5) ◽  
Author(s):  
Dianne Katherine R. Salazar-Paras ◽  
Mae N. Ramirez-Quizon
Keyword(s):  
Pemphigus Vulgaris ◽  
Direct Immunofluorescence ◽  
Bullous Dermatosis ◽  
Present Tense ◽  
Peculiar Case

Pemphigus vulgaris is an autoimmune bullous dermatosis presenting with flaccid blisters and erosions. The morphology of pemphigus reflects the more superficial intraepidermal level of split seen histologically in contrast with pemphigoid, where the level of split is deep below the epidermis. This is a case of a 58-year-old male clinically presenting with arcuate tense bullae, which are more characteristic of the pemphigoid group of disorders, which revealed an intraepidermal split and tombstoning pattern of the basal epidermis on histopathology. Direct immunofluorescence revealed intercellular IgG and C3 distribution. Although this patient presented clinically with tense bullae, the histopathology and direct immunofluorescence results were consistent with pemphigus vulgaris.

scholarly journals Nursing diagnoses in patients with immune-bullous dermatosis

2016 ◽  
Vol 24 (0) ◽  
Author(s):  
Euzeli da Silva Brandão ◽  
Iraci dos Santos ◽  
Regina Serrão Lanzillotti ◽  
Adriano Menis Ferreira ◽  
Mônica Antar Gamba ◽  
...  
Keyword(s):  
Pemphigus Vulgaris ◽  
Mato Grosso ◽  
Assessment Protocol ◽  
Descriptive Research ◽  
The North ◽  
Nursing Diagnosis ◽  
Bullous Dermatosis ◽  
Study Participants ◽  
Nursing Diagnoses ◽  
Mato Grosso Do Sul

ABSTRACT Objective: identify nursing diagnoses in patients with immune-bullous dermatosis. Method: a quantitative and descriptive research, carried out in three institutions located in Rio de Janeiro and Mato Grosso do Sul, Brazil, using the Client Assessment Protocol in Dermatology during a nursing consultation. Simple descriptive statistics was used for data analysis. Results: 14 subjects participated in the study, nine with a diagnosis of pemphigus vulgaris, pemphigus two and three of bullous pemphigoid. The age ranged between 27 and 82 years, predominantly females (11). 14 nursing diagnoses were discussed and identified from a clinical rationale in all study participants, representing the most common human responses in this sample. The application of the Assessment Protocol in Dermatology facilitated the comprehensive assessment, in addition to providing the identification of diagnostics according to the North American Nursing Diagnosis Association International. Conclusion: the nursing diagnoses presented confirm the necessity of interdisciplinary work during the care for this clientele. For better description of the phenomena related to the client in question, it is suggested the inclusion of two risk factors related in three diagnoses of this taxonomy. It is worth noting the contribution of the findings for the care, education and research in nursing in dermatology.

scholarly journals A relapse of pemphigus vulgaris in pemphigus herpetiformis or a phenotypic “switch”

2022 ◽  
Vol 13 (1) ◽  
pp. 107-108
Author(s):  
Siham Belmourida ◽  
Meriame Meziane ◽  
Nadia Ismaili ◽  
Laila Benzekri ◽  
Badreddine Hassam ◽  
...  
Keyword(s):  
Corticosteroid Therapy ◽  
Transition Period ◽  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Exceptional Case ◽  
The Body ◽  
Direct Immunofluorescence ◽  
Phenotypic Switch ◽  
Dermatological Examination ◽  
Phenotypic Transition

Sir, Pemphigus herpetiformis (PH) was originally described by Jablonska et al. in 1975. Clinically, PH presents itself as a herpetiform dermatitis with immunopathological characteristics of pemphigus [1,2]. We report an exceptional case of typical pemphigus vulgaris (PV) relapsing after 36 years in PH. A 65-year-old patient, followed for PV for 36 years and treated with corticosteroid therapy with a remission for more than thirty years, consulted for pruriginous lesions evolving for the previous eight months. A dermatological examination revealed urticariform pruriginous ring lesions surmounted by small peripheral vesicles spread throughout the body (Fig. 1), sparing the mucous membranes, and without Nikolsky’s sign. After two non-specific skin biopsies, the histological examination revealed an intraepidermal bubble with acantholytic cells and eosinophilic spongiosis (Figs. 2a and 2b). Direct immunofluorescence confirmed the diagnosis of pemphigus and indirect immunofluorescence was at the upper limit. The diagnosis of a PV relapse in PH was retained and a dapsone-based treatment was initiated at a dose of 150 mg/day and stopped seven days later when met with hemolytic anemia. Oral corticosteroid therapy involving prednisone at a dose of 1 mg/kg/day was initiated but, given the persistence of the pruritus, the decision was to combine methotrexate at a dose of 12.5 mg/week. A good evolution and a decline within eight months were observed. An improved pruritus and the disappearance of the skin lesions were achieved after one month of treatment. PV and PH are two different anatomical and clinical entities of the autoimmune disease pemphigus, with distinct clinical, histopathological, and immunopathological characteristics [1,2]. Our observation documents a complete phenotypic “switch” of pemphigus with a transition from PV to PH both clinically, histologically, and immunologically. Several rare cases of PV switching to superficial pemphigus (SP) (“phenotypic switch”) have, since 1991, been reported, with a higher frequency this direction than otherwise; the transition period varies from six months to twenty years [3]. To the best of our knowledge, no case has been described of a progression from PV to PH. Having observed one firsthand, we are first to describe the case of a complete phenotypic switch from PV to PH. The mechanism of such a transition remains poorly understood and is often observed during a relapse. Some authors suggest that the effect of immunosuppressants on the desmoglein DSG3 more marked than on DSG1 could explain the relapse of PS in PH [3,4]. Future studies on the immunological factors and predictors of PV relapses after the discontinuation of treatment would be useful to better understand the mechanisms of a relapse in pemphigus, with or without a phenotypic transition.

scholarly journals The Significance of Scalp Involvement in Pemphigus: A Literature Review

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Marta Sar-Pomian ◽  
Lidia Rudnicka ◽  
Malgorzata Olszewska
Keyword(s):  
Web Of Science ◽  
Clinical Manifestations ◽  
Pemphigus Vulgaris ◽  
Hair Follicles ◽  
Response To Treatment ◽  
Direct Immunofluorescence ◽  
Pemphigus Foliaceus ◽  
Medical Databases ◽  
Scalp Lesions ◽  
Reliable Diagnostic Method

Scalp is a unique location for pemphigus because of the abundance of desmogleins localized in hair follicles. Scalp involvement is observed in up to 60% of patients in the course of pemphigus. The lesions may occasionally lead to alopecia. Unforced removal of anagen hairs in a pull test is a sign of high disease activity. Direct immunofluorescence of plucked hair bulbs is considered a reliable diagnostic method in patients with pemphigus. Follicular acantholysis is a characteristic histopathological feature of pemphigus lesions localized on the scalp. Trichoscopy may serve as a supplementary method in the diagnosis of pemphigus. This review summarizes the most recent data concerning scalp involvement in pemphigus vulgaris and pemphigus foliaceus. A systematic literature search was conducted in three medical databases: PubMed, Embase, and Web of Science. The analysis included literature data about desmoglein distribution in hair follicles, as well as information about clinical manifestations, histopathology, immunopathology, and trichoscopy of scalp lesions in pemphigus and their response to treatment.

A Case of Autoimmune Bullous Dermatosis with Features of Pemphigus Vulgaris and Bullous Pemphigoid

1995 ◽  
Vol 17 (5) ◽  
pp. 511-516 ◽  
Author(s):  
Satoshi Kore-eda ◽  
Yuji Horiguchi ◽  
Eriko Ohtoshi ◽  
Toshihiro Tanaka ◽  
Kimio Fujii ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Pemphigus Vulgaris ◽  
Bullous Dermatosis

scholarly journals Pemphigus Vulgaris: A Clinical Study of 31 Cases (2004–2014) in Morocco

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Titou Hicham ◽  
Fatima Zahra Chahnoun ◽  
Tarik Hanafi ◽  
Naoufal Hjira ◽  
Boui Mohammed
Keyword(s):  
Oral Cavity ◽  
Age At Onset ◽  
Pemphigus Vulgaris ◽  
Direct Immunofluorescence ◽  
Long Term Outcomes ◽  
Prognosis Factors ◽  
Mucosal Involvement ◽  
Multiple Relapses

Background. Pemphigus vulgaris is a rare bullous autoimmune dermatosis whose evolution and prognosis are unpredictable. Aim. The objective was to analyze long-term outcomes in patients with pemphigus vulgaris by identifying the factors that are able to influence prognosis, in particular the phenotype of pemphigus vulgaris, age at onset, multiplicity of mucosal involvement, relapse and remission rates, and survival functions. Methods. A retrospective analysis of a cohort of 31 patients followed for pemphigus vulgaris during the period from January 2004 to January 2014. Inclusion criteria were a diagnosis of pemphigus vulgaris confirmed by histopathology and direct immunofluorescence (DIF) and a period of follow-up of at least five years from the diagnosis. The following information was collected by a single investigator. Results. In total, 67.7% of patients presented a mucocutaneous pemphigus vulgaris. Male-female sex ratio was 2.4. The median duration of patient’s follow-up was estimated at 7 (6–9) years. Multiple mucosal involvement in the oral cavity and at other mucosal sites was significantly associated with severe mucocutaneous pemphigus vulgaris (p=0.01). Multiple relapses were significantly associated with the disease severity (p=0.04). Conclusion. Poor prognosis factors were severe mucocutaneous type of pemphigus vulgaris and multiple mucosal involvement in the oral cavity and at other mucosal sites.

scholarly journals Lichen Planus Pemphigoides Induced by Enalapril: A Case Report and a Review of Literature

2017 ◽  
Vol 9 (3) ◽  
pp. 217-224 ◽  
Author(s):  
Woranit Onprasert ◽  
Kumutnart Chanprapaph
Keyword(s):  
Lichen Planus ◽  
Early Recognition ◽  
Case Reports ◽  
Direct Immunofluorescence ◽  
Drug Induced ◽  
First Case ◽  
Bullous Dermatosis ◽  
Culprit Drug ◽  
History Of ◽  
Hands And Feet

Lichen planus pemphigoides (LPP) is a rare autoimmune bullous dermatosis. The clinical presentation of LPP may mimic bullous pemphigoid making the diagnosis difficult. A thorough clinical, histopathological, and immunological evaluation is essential for the diagnosis of LPP. The etiology is largely idiopathic; however, there are several case reports of drug-induced LPP. We report an 81-year-old Thai woman with underlying hypertension and type 2 diabetes mellitus who presented with a 4-week history of multiple tense bullae initially on the hands and feet that subsequently expanded to the trunk and face. Enalapril was commenced to control hypertension. The histopathology and direct immunofluorescence were compatible with LPP. Circulating anti-basement antibodies BP180 was also positive. The patient was treated with topical corticosteroid with a modest effect. Enalapril was discontinued and complete resolution of LPP occurred within 12 weeks. There was no recurrence after a 1-year follow-up period. To the best of our knowledge, we present the first case of enalapril-induced LPP. Early recognition and prompt discontinuation of the culprit drug allow resolution of the disease. Medication given for LPP alone, without cessation of the offending drug, may not change the course of this condition.

Other Vesiculobullous Diseases

2020 ◽  
Author(s):  
Aakaash Varma ◽  
Annette Czernik ◽  
Jacob Levitt
Keyword(s):  
Bullous Pemphigoid ◽  
Epidermolysis Bullosa ◽  
Tissue Transglutaminase ◽  
Direct Immunofluorescence ◽  
Cicatricial Pemphigoid ◽  
Positive Serology ◽  
Fixed Drug Eruption ◽  
Varicella Zoster ◽  
Bullous Dermatosis ◽  
Linear Iga Bullous Dermatosis

Less common immunobullous diseases include cicatricial pemphigoid, epidermolysis bullosa acquisita, and linear IgA bullous dermatosis. Diagnosis of these entities are made through direct immunofluorescence, sometimes requires salt-split skin, as well as, in the case of cicatricial pemphigoid, mucosal scarring. As in pemphigus vulgaris and bullous pemphigoid, common therapies include rituximab, prednisone, and IVIg. Dapsone can be particularly effective in linear IgA bullous dermatosis and bullous lupus. Dermatitis herpetiformis is a rare cutaneous manifestation of gluten sensitivity, characterized by pruritic vesicles on extensor surfaces, that responds to dapsone and gluten avoidance. This diagnosis is confirmed with biopsy and positive serology for anti-tissue transglutaminase IgA. Blistering hypersensitivity reactions include TEN, SJS, erythema multiforme, and fixed drug eruption. All are characterized by varying degrees of keratinocyte necrosis. Common to the management of all include cessation of the offending agent. TEN can be managed by cyclosporine, TNF-inhibition, or—more controversially—IVIg. SJS can be effectively managed with systemic steroids. EM responds variably to a number of agents, including antiviral nucleoside analogues, prednisone, thalidomide, apremilast, and tofacitinib. Infectious causes of blisters include Staphylococcus aureus, HSV, and varicella zoster virus. Epidermolysis bullosa comprises a variety of genetically defective structural proteins of the skin. Recessive variants and those affecting deeper proteins carry more severe phenotypes. Management is best achieved at specialty centers and involves careful wound care as well as prevention of friction. Gene therapy is on the horizon for these disorders. Other blistering entities, mechanical or inflammatory in nature, are also discussed at the end of this chapter. This review contains 13 figures, 1 table, and 86 references. Keywords: Blisters, bullae, bullous, pemphigoid, necrolysis

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