The Rare Case of Pemphigus Vegetans in Association with Malnutrition Children in the Multidisciplinary Management

Pemphigus vegetans is a clinical variant of pemphigus vulgaris, accounting for 1–2% of all very rare pemphigus cases in children. The involvement of the oral mucosa in this disease is usually accompanied by severe pain that aggravates the patient’s malnourished condition. Conversely, malnutrition may also reduce vulnerability towards autoimmune diseases. Although pemphigus vegetans has never been reported to develop in a child with marasmus before, we encountered a case of pemphigus vegetans in a severely malnourished patient. A 12-year-old boy in marasmic condition presented with painful, clear, fluid-filled blisters, accompanied by erosions, crusts, and vegetative lesions on almost all parts of the body. Histopathological examination of the lesions revealed a suprabasal cleft, and direct immunofluorescence staining showed deposits of immunoglobulin G in the epidermal intracellular spaces. The patient was treated with a multidisciplinary approach, and intravenous corticosteroid was administered for 2 weeks with an appropriate diet. There were significant improvements in the skin lesions and his nutritional status. Although pemphigus vegetans may occur in children with malnutrition, the underlying mechanism for the development of autoimmune diseases in malnutrition remains unclear.

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

Our Dermatology Online ◽

10.7241/ourd.20221.29 ◽

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.

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Total body irradiation induced mouse small intestine senescence as a late effect

Journal of Radiation Research ◽

10.1093/jrr/rrz026 ◽

2019 ◽

Vol 60 (4) ◽

pp. 442-450 ◽

Cited By ~ 2

Author(s):

Yu Zhao ◽

Junling Zhang ◽

Xiaodan Han ◽

Saijun Fan

Keyword(s):

Small Intestine ◽

Total Body Irradiation ◽

Profile Analysis ◽

Bacterial Flora ◽

Body Weight Loss ◽

Underlying Mechanism ◽

The Body ◽

Mouse Small Intestine ◽

Total Body ◽

Almost All

Abstract Radiation can induce senescence in many organs and tissues; however, it is still unclear how radiation stimulates senescence in mouse small intestine. In this study, we use the bone marrow transplantation mouse model to explore the late effects of total body irradiation on small intestine. Our results showed that almost all of the body hairs of the irradiated mice were white (which is an indication of aging) 10 months after the exposure to radiation. Furthermore, compared with the age-matched control mice, there were more SA-β-galactosidase (SA-β-gal)–positive cells and an upregulation of p16 and p21 in 8 Gy–irradiated mice intestinal crypts, indicating that radiation induced senescence in the small intestine. Intestinal bacterial flora profile analysis showed that the diversity of the intestinal bacterial flora decreased in irradiated mice; in addition it showed that the principal components of the irradiated and control mice differed: there was increased abundance of Bacteroidia and a decreased abundance of Clostridia in irradiated mice. To explore the underlying mechanism, an RNA-sequence was executed; the results suggested that pancreatic secretion, and the digestion and absorption of proteins, carbohydrates, fats and vitamins were damaged in irradiated mice, which may be responsible for the body weight loss observed in irradiated mice. In summary, our study suggested that total body irradiation may induce senescence in the small intestine and damage the health status of the irradiated mice.

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Schimmelpenning Syndrome with Large Nevus Sebaceous and Multiple Epidermal Nevi

Case Reports in Dermatology ◽

10.1159/000509992 ◽

2020 ◽

Vol 12 (3) ◽

pp. 186-191

Author(s):

Reiva Farah Dwiyana ◽

Maryam Nissa Hazari ◽

Inne Arline Diana ◽

Srie Prihianti Gondokaryono ◽

Raden Mohamad Rendy Ariezal Effendi ◽

...

Keyword(s):

Sebaceous Gland ◽

Histopathological Examination ◽

The Body ◽

Epidermal Nevus ◽

Clinical Variant ◽

Central Nervous Systems ◽

Brownish Black ◽

Almost All ◽

Nevus Sebaceous ◽

Congenital Syndrome

Epidermal nevus syndromes are a group of rare multiorgan disorders. Schimmelpenning syndrome is a clinical variant of epidermal nevus syndromes. This congenital syndrome is characterized by the existence of nevus sebaceous and usually accompanied by abnormalities of the eyes, skeletal, cardiovascular, and central nervous systems. A 2-year-old girl presented with large, hairless, yellowish-brown plaques on the scalp and face along with multiple brownish-black verrucose plaques and brownish-black macules on almost all parts of the body. The skin-colored verrucose tumors were also found on the lips and around the mouth. Histopathological examination of the lesion on the forehead revealed hyperkeratosis, acanthosis, and sebaceous gland hyperplasia supporting the diagnosis of nevus sebaceous, while histopathological examination of the lesions on the lips and abdomen demonstrated hyperkeratosis, acanthosis, and papillomatosis consistent with verrucous epidermal nevus. The pediatrician suspected that the patient had mental retardation; however, there were no neurological, cardiac, skeletal, nor ophthalmologic abnormalities. The lesions on the lips and around the mouth were excised, and it demonstrated a good result. To conclude, epidermal nevus syndrome (e.g., Schimmelpenning syndrome) should be considered in children born with nevus sebaceous.

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Primary systemic amyloidosis

Vojnosanitetski pregled ◽

10.2298/vsp0712859t ◽

2007 ◽

Vol 64 (12) ◽

pp. 859-862

Author(s):

Srdjan Tanasilovic ◽

Dubravka Zivanovic ◽

Milos Nikolic ◽

Maja Tomovic ◽

Ivo Elezovic ◽

...

Keyword(s):

Heart Failure ◽

Histopathological Examination ◽

Skin Lesions ◽

The Body ◽

Systemic Amyloidosis ◽

Light Chains ◽

Immunoglobulin Light Chains ◽

Disease Case ◽

Cardiac Evaluation ◽

Urine And Serum

Background. Systemic amyloidosis is a rare disorder which usually occurs in aged persons and has a poor prognosis. Systemic amyloidosis can be primary, occasionally associated with multiple myeloma, or secondary, associated with another disease. Case report. We presented a 72-year-old male patient with periocular purpura ("racoon sign") and waxy papules, petechiae and ecchymoses on the neck and thoracic area. Purpuric macules were present also on the lips and tongue which was edematous (macroglossia). The skin lesions occurred two years earlier, the patient lost more than 15 kilograms of the body mass for less than a year. Immunoelectrophoresis of urine and serum demonstrated the presence of immunoglobulin light chains of the circulating monoclonal protein. Histopathological examination of skin lesions showed Congo red positive deposits in the derm. Cardiac evaluation revealed the signs of heart failure, and renal evaluation revealed nephrotic syndrome, with excessive protein lost. He was treated with oral melphalan and prednisolone, and died 7 days after starting the therapy due to heart failure. Conclusion. This patient considered as a rare case with systemic amyloidosis highlights the importance of histopathological and physical examination in any cases with periocular purpura, petechiae, ecchymoses and macroglossia.

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Regional variations in antigenic properties of skin. A possible cause for disease-specific distribution of skin lesions.

Journal of Experimental Medicine ◽

10.1084/jem.164.6.2125 ◽

1986 ◽

Vol 164 (6) ◽

pp. 2125-2130 ◽

Cited By ~ 34

Author(s):

L Sison-Fonacier ◽

J C Bystryn

Keyword(s):

Regional Differences ◽

Skin Diseases ◽

Pemphigus Vulgaris ◽

Skin Lesions ◽

The Body ◽

Cutaneous Lesions ◽

Specific Distribution ◽

Antigenic Properties ◽

Possible Cause ◽

Disease Specific

The possibility that the distribution of skin lesions in some cutaneous diseases is due to variations in the antigenic properties of skin was investigated by mapping the expression of the skin-specific pemphigus vulgaris and bullous pemphigoid antigens in different regions of the body. The expression of both antigens was relatively stable within the same region, but varied between regions in a pattern that was distinct for each antigen. For each antigen there was a correlation between regions of high expression and location of skin lesions in autoimmune diseases involving the antigen. The results indicate that there are marked regional differences in the antigenic properties of skin and suggest this may influence the distribution of cutaneous lesions in some skin diseases.

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Oral pemphigus vulgaris: clinicopathological study of 35 cases at a tertiary care centre in North India

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20170973 ◽

2017 ◽

Vol 3 (2) ◽

pp. 192

Author(s):

Ameesha Mahajan ◽

Jasleen Kaur ◽

Mridu Manjari ◽

Saurabh Sharma ◽

Pooja Bains ◽

...

Keyword(s):

Histopathological Examination ◽

Tertiary Care ◽

Pemphigus Vulgaris ◽

Skin Lesions ◽

Punch Biopsy ◽

North India ◽

Tertiary Care Centre ◽

Present Specimen ◽

Female Ratio ◽

Oral Ulcers

Background: The purpose of the study was to evaluate the clinical and histopathological features of oral pemphigus vulgaris.Methods: A prospective study of 35 cases of oral pemphigus vulgaris, over a period of 2.5 years from June 2014 to December 2016 in the Department of Dermatology, Sri Guru Ram Das Institute of Medical Sciences and Research, Amritsar was designed. Complete history and detailed mucocutaneous examination was done. Punch biopsy was performed from the oral mucosa and skin lesions (if present). Specimen was sent for histopathological examination and results analysed.Results: Out of 35 patients, 17 (48.57%) were males and 18 (51.42%) were females. Male to female ratio was 1:1.1. The majority of the patients (12, 34.29%) were in the 40-49 year age group. Multiple sites were involved in all patients with bilateral buccal mucosa being involved in all the cases, followed by tongue (30, 85.71%). Histopathological examination showed presence of suprabasal split and acantholytic cells in all cases. Conclusions: Oral ulcers are a presenting feature of pemphigus vulgaris which commonly precede the skin lesions. Histopathological examination enables early diagnosis of the disease which leads to prompt treatment and decreased morbidity.

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Unilateral Dyshidrosiform Pemphigus Vulgaris

Annals of the National Academy of Medical Sciences (India) ◽

10.1055/s-0040-1713184 ◽

2020 ◽

Vol 56 (04) ◽

pp. 231-233

Author(s):

Manju Daroach ◽

Dipankar De ◽

Sanjeev Handa ◽

Rahul Mahajan ◽

Vikarn Vishwajeet ◽

...

Keyword(s):

Autoimmune Disease ◽

Pemphigus Vulgaris ◽

Skin Lesions ◽

Unusual Presentation ◽

Foot Ulcers ◽

Direct Immunofluorescence ◽

Proper Treatment ◽

Rare Presentation ◽

Delay In Diagnosis ◽

Mucosal Surfaces

AbstarctPemphigus vulgaris is an autoimmune disease characterized by fluid-filled blisters over body and mucosal surfaces. Localized pemphigus is a rare presentation of the disease. There are few reports of localized pemphigus in literature presenting as esophageal involvement, recurrent paronychia, conjunctival mass, foot ulcers, discoloration of toes with hyperkeratotic skin lesions, verrucous lesions, and dyshidrosiform pemphigus vulgaris. We present a case of dyshidrosiform pemphigus involving a foot following trauma. Histopathology and direct immunofluorescence confirmed our diagnosis. In our patient, the disease started as localized dyshidrosiform pemphigus and later became generalized. This unusual presentation led to a delay in diagnosis and proper treatment in this patient. Physicians should be aware of such rare presentations to aid in the timely diagnosis and management of such patients.

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Simultaneous Oral and Umbilical Locations as a First Sign of Pemphigus Vulgaris

Case Reports in Dentistry ◽

10.1155/2021/7792360 ◽

2021 ◽

Vol 2021 ◽

pp. 1-7

Author(s):

Eya Moussaoui ◽

Yassine Oueslati ◽

Lamia Oualha ◽

Mohamed Denguezli ◽

Badreddine Sriha ◽

...

Keyword(s):

Early Diagnosis ◽

Early Treatment ◽

Clinical Manifestations ◽

Pemphigus Vulgaris ◽

Skin Lesions ◽

Oral Lesions ◽

Cutaneous Lesions ◽

Autoimmune Bullous Diseases ◽

Bullous Diseases ◽

Pemphigus Vegetans

The place of pemphigus vulgaris (PV) among autoimmune bullous diseases (AIBD) is well established. It is an acquired chronic, autoimmune, vesiculobullous disease in which IgG antibodies target desmosomal proteins to produce intraepithelial mucocutaneous blistering. The diagnosis is often challenging for the clinicians. It requires a combination of three major features: clinical, histopathological, and immunological. Clinically, oral lesions are the first manifestations of the disease in 50-90% of the patients with widespread blisters affecting the oral mucosa. On the skin, lesions are characterized by flaccid blisters that rapidly progress into erosions and crust formation. Umbilical lesions as a clinical manifestation of PV are peculiar and have rarely been reported, and they are not yet completely elucidated. Umbilical region involvement in patients with pemphigus was assessed in a limited study totalling just 10 patients. This localisation may be a valuable hint easing the diagnosis at the clinical level for patients with oral mucosal blisters. Dentists must be familiar with the clinical manifestations of PV to make an early diagnosis and start an early treatment which determines the prognosis of the disease. To the best of the authors’ knowledge, the coexistence of these lesions with the oral lesions as a first sign of PV in the absence of skin involvement was reported in only one case of pemphigus vegetans (PVe). In this paper, we describe an observation of a female patient that was diagnosed with PV that begun with simultaneous oral and umbilical locations which coexisted for a period of 4 months before the appearance of other cutaneous lesions. We highlight the role of dentists, by being familiar with the clinical manifestations of PV, to make an early diagnosis to start an early treatment which determines the prognosis of the disease and to follow closely the evolution of lesions to change treatment if required. We also discuss the clinical, histological, and immunological features of the disease that enabled the differential diagnosis as well as the appropriate therapeutic management.

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Clinical, histopathological and immunofluorescent study of vesicobullous lesions of skin

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20191341 ◽

2019 ◽

Vol 7 (4) ◽

pp. 1288

Author(s):

Seeram Satish Kumar ◽

Bhagyalakshmi Atla ◽

Guru Prasad Patnala ◽

Kukkala Saraswati Sarat Srinivas ◽

Saraswathi Samantra ◽

...

Keyword(s):

Bullous Pemphigoid ◽

Histopathological Examination ◽

Pemphigus Vulgaris ◽

Direct Immunofluorescence ◽

Pemphigus Foliaceus ◽

Female Ratio ◽

Medical College ◽

Intensive Investigation ◽

Male To Female

Background: Vesiculobullous diseases have been the focus of intensive investigation in recent years. However, these disorders are still associated with substantial morbidity, considerable mortality and impaired quality of life. Accurate diagnosis of vesiculobullous lesions of skin entails evaluation of clinical, histopathologic and immunofluorescence findings.Methods: Hospital based prospective study for a period of 24 months from August 2014 to July 2016 in the Department of Pathology at Andhra Medical College, Visakhapatnam, India. Total of 50 patients aged 3-70 years with vesiculobullous lesions of both sexes attending the Department of Dermatology were selected and analysed clinically, histopathological examination and direct immunofluorescence (DIF).Results: In the present study, majority of patients presented between 51-60 yrs of age (32%) with male to female ratio of 1.08:1 and mean age of 46.02 years. Pemphigus vulgaris constituted the most common vesiculobullous disorder (32%) followed by bullous pemphigoid and pemphigus foliaceous, 18% each. Bullae were located intra epidermally in 68% and sub epidermally in 32% of the patients. DIF was positive in 80% of the cases. Overall clinicopathological correlation was established in 74%. Overall histopathological and direct immunofluorescence correlation was established in 78%. Out of 50 cases, 35 cases (70%) correlated clinically and histo-pathologically with direct immunofluorescence.Conclusions: In the present study, on histopathological examination alone pemphigus foliaceus and pemphigus vulgaris could be differentiated. Direct immunofluorescence was useful in differentiating epidermolysis bullosa acquisita from bullous pemphigoid which have similar histopathological picture. This study proves that direct immunofluorescence is confirmatory as well as diagnostic for vesiculobullous disorders.

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A Hypothesis Concerning a Potential Involvement of Ceramide in Apoptosis and Acantholysis Induced by Pemphigus Autoantibodies

Dermatology Research and Practice ◽

10.1155/2010/702409 ◽

2010 ◽

Vol 2010 ◽

pp. 1-8

Author(s):

Wendy B. Bollag

Keyword(s):

Autoimmune Diseases ◽

Ultraviolet Irradiation ◽

Cell Adhesion Molecule ◽

Sun Exposure ◽

Pemphigus Vulgaris ◽

Skin Lesions ◽

Epidermal Keratinocytes ◽

Pemphigus Foliaceus ◽

Endemic Pemphigus Foliaceus ◽

A Cell

Autoimmune diseases affect more than 50 million Americans, resulting in significant healthcare costs. Most autoimmune diseases occur sporadically; however, endemic pemphigus foliaceus (EPF) is an autoimmune skin disease localized to specific geographic loci. EPF, and the related diseases pemphigus vulgaris (PV) and pemphigus foliaceus (PF), are characterized by skin lesions and autoantibodies to molecules found on epidermal keratinocytes. A variant of EPF in patients from El Bagre, Colombia, South America, has recently been reported to be distinct from previously described loci in Brazil and Tunisia epidemiologically and immunologically. As in PF and EPF, El Bagre EPF patients exhibit autoantibodies towards desmoglein-1, a cell adhesion molecule critical for maintaining epidermal integrity. An association of El Bagre EPF with sun exposure has been detected, and ultraviolet irradiation also exacerbates symptoms in PV, PF and EPF. Our hypothesis is that: (1) the autoantibodies generate pathology through an alteration in ceramide metabolism in targeted keratinocytes, resulting in apoptosis and/or cell death and acantholysis, but only when the cell's ability to metabolize ceramide is exceeded, and (2) apoptosis in response to this altered ceramide metabolism is initiated and/or exacerbated by other agents that increase ceramide levels, such as cytokines, ultraviolet irradiation, and senescence.

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