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JNMA; Journal of the Nepal Medical... Jul 2022Pemphigus vulgaris is a rare autoimmune mucocutaneous blistering disease clinically presenting as vesicles, bullae, and erosion and histologically characterized by...
UNLABELLED
Pemphigus vulgaris is a rare autoimmune mucocutaneous blistering disease clinically presenting as vesicles, bullae, and erosion and histologically characterized by suprabasal split and acantholysis. It usually affects mucous membranes and skin. Recurrent oral ulcers can only be the clinical manifestation before progressing into skin lesions. This can lead to the delayed diagnosis of this disease. Here we report a case of pemphigus vulgaris which was diagnosed after years of suffering from an oral ulcer that eventually progressed to widespread skin blistering and ulceration. The patient was treated with oral prednisolone which showed improvement within a week. Physicians should consider the differential diagnosis of pemphigus vulgaris in patients presenting with a recurrent oral ulcer.
KEYWORDS
delayed diagnosis; oral ulcer; pemphigus vulgaris.
Topics: Humans; Pemphigus; Oral Ulcer; Delayed Diagnosis; Prednisolone; Skin
PubMed: 36705190
DOI: 10.31729/jnma.7594 -
Indian Journal of Dermatology 2022Pemphigus is a group of auto-immune blistering disorders, characterised clinically by mucocutaneous blisters and erosions and histopathologically by intra-epidermal...
BACKGROUND
Pemphigus is a group of auto-immune blistering disorders, characterised clinically by mucocutaneous blisters and erosions and histopathologically by intra-epidermal acantholysis. It was traditionally associated with high morbidity and mortality. The use of rituximab has brought upon a new dawn in the treatment of pemphigus.
AIM
A retrospective analysis to ascertain the efficacy, tolerance, adverse effect profile, remission, and relapse with the use of rituximab.
MATERIAL AND METHODS
A retrospective analysis of all diagnosed pemphigus patients who received rituximab therapy over a period of 3 years was performed. The patient's baseline characteristics, disease duration, clinical presentations, mucosal involvement, disease-severity assessment, and adverse events with rituximab were noted. The outcomes were evaluated based on the definitions of the disease-outcome parameters as early and late endpoints.
RESULTS
Of the 17 pemphigus patients, there were 14 females (82.4%) and three males (17.6%) with a mean age of 35.9 ± 16.5 years (range: 9-65 years). Pemphigus vulgaris (PV) was the predominant type in 11 (64.7%) patients. After rituximab infusion, the 17 patients attained the end of consolidation phase (ECP) within 15 days to 3 months, and the mean duration was 1.24 months. The complete remission (CR on/off) ranged from 0.5 to 35 months, and the mean duration of remission was 21.7 months. Within a median time of 4.2 months, almost 80% patients achieved CR on therapy. Nine (53%) patients were in CR without any therapy till the end of the study period, and eight (47%) were in remission while on minimal therapy.
CONCLUSION
Rituximab is an efficacious therapeutic agent for pemphigus and is better tolerated and safer to all the previous medications used in the treatment.
PubMed: 36578732
DOI: 10.4103/ijd.ijd_169_22 -
Clinical Case Reports Dec 2022The anal region is an unusual site of Hailey-Hailey disease. It manifests with lichenoid lesions with crusted erosions around the anus. It should be differentiated from...
The anal region is an unusual site of Hailey-Hailey disease. It manifests with lichenoid lesions with crusted erosions around the anus. It should be differentiated from condylomata acuminata, extramammary Paget disease, and bowenoid papulosis.
PubMed: 36514472
DOI: 10.1002/ccr3.6702 -
Frontiers in Medicine 2022Pemphigus is a chronic autoimmune skin blistering disease, characterized by acantholysis and by the production of autoantibodies directed against the structural...
Pemphigus is a chronic autoimmune skin blistering disease, characterized by acantholysis and by the production of autoantibodies directed against the structural desmosomal proteins desmoglein 1 (DSG1) and/or DSG3. Model systems allow the identification and testing of new therapeutic targets. Here, we evaluated ultrastructural desmosomal morphology in the human skin organ culture (HSOC) model injected with either anti-desmoglein (DSG) 1/3 single-chain variable fragment (scFv, termed Px4-3), Staphylococcus aureus exfoliative toxin (ETA) as a reference and positive control, and normal human IgG as a negative control. Each experimental condition was evaluated in abdominal skin biopsies from five different donors. After 24 h of incubation, we processed the samples for histological and ultrastructural electron microscopy analyses. We found that Px4-3 or ETA induced a loss of desmosomes and increased interdesmosomal widening, similar to patient skin biopsies and other pemphigus models. Thus, we propose the HSOC pemphigus model as an attractive tool to unravel novel therapeutic targets.
PubMed: 36452895
DOI: 10.3389/fmed.2022.997387 -
JAMA Dermatology Jan 2023
Topics: Humans; Phototherapy; Light; Acantholysis; Ichthyosis
PubMed: 36449285
DOI: 10.1001/jamadermatol.2022.4491 -
Journal of Clinical Medicine Nov 2022pemphigus vulgaris is a rare autoimmune blistering disease that involves the skin and mucous membranes and rarely occurs in pediatric age.
INTRODUCTION
pemphigus vulgaris is a rare autoimmune blistering disease that involves the skin and mucous membranes and rarely occurs in pediatric age.
METHODS
we present a case of childhood pemphigus in a 9-year-old patient from Burkina Faso, which initially manifested with erosive lesions symmetrically distributed in the oral cavity. After a few months, we also observed hyperchromic lesions of the back. Histopathological examination of skin samples showed intraepidermal acantholysis, while direct immunofluorescence showed deposits of complement (C3) and immunoglobulins G (IgG) in the epidermidis; an ELISA test highlighted the presence of circulating autoantibodies against desmoglein 3.
RESULTS
the follow-up of this patient was made difficult by the advent of the COVID-19 outbreak. However, after about one year of combined therapy with systemic steroids and azathioprine the patient reached clinical remission.
PubMed: 36431335
DOI: 10.3390/jcm11226858 -
Case Reports in Women's Health Oct 2022A healthy 25-year-old primiparous woman had an uncomplicated pregnancy and spontaneous vaginal delivery with mediolateral episiotomy. Twenty-four hours postpartum, she...
A healthy 25-year-old primiparous woman had an uncomplicated pregnancy and spontaneous vaginal delivery with mediolateral episiotomy. Twenty-four hours postpartum, she developed increasing perineal pain and swelling. Initial examination showed a localized erythema and tissue oedema at the episiotomy site. The woman was admitted to hospital for management of the infected hematoma at the site of the episiotomy. Thereafter, she was started on intravenous antibiotics, and exploration under anaesthesia was planned. The woman's medical condition deteriorated rapidly, and necrotizing fasciitis (NF) was strongly suspected. Therefore, aggressive medical and surgical management was undertaken, including broader-spectrum antibiotics and multiple surgical debridement. A biopsy of the debrided tissue showed acantholysis and dyskeratosis, which are features of Hailey-Hailey disease of the skin (familial benign chronic pemphigus), a rare condition. The woman eventually had a V-Y advancement fascial flap and made a complete recovery. In this case report, the details of the development of NF in a woman who was found to have Hailey-Hailey disease are discussed.
PubMed: 36389631
DOI: 10.1016/j.crwh.2022.e00461 -
Case Reports in Hematology 2022A 49-year-old male with no past medical history presented with acute-onset painful mucosal erosions along with flaccid bullae on his trunk, scalp, and intertriginous...
A 49-year-old male with no past medical history presented with acute-onset painful mucosal erosions along with flaccid bullae on his trunk, scalp, and intertriginous areas. The patient initially underwent a skin biopsy which demonstrated suprabasilar acantholysis and lichenoid interface dermatitis. This was followed by a computed tomography scan which identified a large abdominal lymph node. Core needle biopsy of this node demonstrated follicular lymphoma. Lastly, indirect immunofluorescence (IIF) in rat bladder was positive (titer 1 : 10,240). This finding confirmed the diagnosis of paraneoplastic pemphigus (PNP) in the setting of follicular lymphoma. The patient's cutaneous disease was treated with a combination of intravenous immunoglobulin and methylprednisolone, along with intravenous rituximab, with a resolution of his cutaneous symptoms. His lymphoma was treated with six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP), with an interval decrease in his tumor burden. PNP is an autoimmune-mediated mucocutaneous disease associated with underlying neoplasm, most commonly non-Hodgkin lymphoma or chronic lymphocytic leukemia. Affected patients develop variable autoantibodies to antigens on keratinocytes and the basement membrane zone. Severe intractable stomatitis is characteristic, in addition to polymorphous cutaneous eruptions including bullae and erosions. Mortality rates can reach up to 90% due to malignancy, sepsis, or bronchiolitis obliterans, an irreversible and often lethal cause of pulmonary insufficiency. We highlight PNP manifesting in a patient with lymphoma, who responded well to the skin- and malignancy-directed treatments. PNP is an exceedingly rare diagnosis that should be considered in a patient with intractable stomatitis.
PubMed: 36330223
DOI: 10.1155/2022/3641474 -
Diagnostics (Basel, Switzerland) Oct 2022Adenosquamous carcinoma (ASC) is an aggressive subtype of squamous cell carcinoma (SCC). Due to its poor prognosis, a precise pathological diagnosis of ASC is essential...
Adenosquamous carcinoma (ASC) is an aggressive subtype of squamous cell carcinoma (SCC). Due to its poor prognosis, a precise pathological diagnosis of ASC is essential but challenging because its pathological criteria are still unclear. Here, we present a rare case of oral ASC accompanied by acantholytic features. The tumor was raised in the mandibular gingiva and recurred locally approximately 13 months after the initial surgery with cervical lymph node metastasis. Pathological specimens of the primary lesion showed acantholysis in a large area of the SCC. Mucous cells, the characteristic finding indicating glandular differentiation, were imperceptible in the initial surgical specimen but increased in the locally recurrent and metastatic lymph node specimens. In a comprehensive literature review of oral ASC cases, the present case was the only case of ASC with acantholytic features. We reconfirmed that ASC has poor prognoses, such as low 5-year overall survival and disease-free survival, high locoregional recurrence, and high distant metastasis rates. A precise diagnosis of ASC is required for estimating prognosis and undergoing close follow-up, even if the adenocarcinomatous component is limited to a small area in the lesion.
PubMed: 36292088
DOI: 10.3390/diagnostics12102398 -
The Pan African Medical Journal 2022Pemphigus vulgaris (PV) is an autoimmune mucocutaneous disorder of the oral cavity and is the most common subtype of pemphigus. The etiology remains obscure, although...
Pemphigus vulgaris (PV) is an autoimmune mucocutaneous disorder of the oral cavity and is the most common subtype of pemphigus. The etiology remains obscure, although the disease is characterized by autoantibodies directed against the desmoglein component of the keratinocytes. It manifests clinically as vesicle, bullae or erosions of skin and mucous membrane and histopathologically shows the presence of acantholysis. The presence of exclusive oral lesions initially increases the chances of misdiagnosing the disease as another condition, posing diagnostic, therapeutic and prognostic difficulties, consequently prompt diagnosis and treatment can prevent untoward consequences. Demonstration of IgG antibodies against desmoglein in Immunofluroscence confirms the diagnosis. In here we report a case of a 55-year-old female patient suffering from PV emphasizing the significance of clinical examination, pertinent investigations, treatment rendered and its outcome.
Topics: Acantholysis; Autoantibodies; Blister; Desmogleins; Female; Humans; Immunoglobulin G; Keratinocytes; Middle Aged; Pemphigus
PubMed: 36212921
DOI: 10.11604/pamj.2022.42.184.34184