scholarly journals PUVA Induced Bullous Pemphigoid in a Patient with Mycosis Fungoides

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Birgül Özkesici ◽  
Saliha Koç ◽  
Ayşe Akman-Karakaş ◽  
Ertan Yılmaz ◽  
İbrahim Cumhur Başsorgun ◽  
...  

Background. Bullous pemphigoid is an autoimmune subepidermal blistering skin disease in which autoantibodies are directed against components of the basement membrane. The disease primarily affects the elderly people and in most of the patients inducing factors cannot be identified. Herein, we report a case of BP that occurred in a patient who was receiving PUVA therapy for the treatment of mycosis fungoides. Main Observation. A 26-year-old woman with mycosis fungoides developed blisters while receiving PUVA therapy. On physical examination tense bullae on the normal skin, remnants of blisters, and erosions were observed on her breasts, the chest wall, and the upper abdomen. Histopathological investigations revealed subepidermal blisters with eosinophilic infiltration and in direct immunofluorescence examination linear deposition of IgG along the basement membrane zone was observed. The diagnosis of bullous pemphigoid was also confirmed by ELISA and BIOCHIP mosaic-based indirect immunofluorescence test. Conclusions. PUVA therapy is an extremely rare physical factor capable of inducing bullous pemphigoid. So the development of blistering lesions during PUVA therapy may be suggestive sign of a bullous disease such as bullous pemphigoid and it should be excluded with proper clinical and laboratory approaches immediately after withdrawal of PUVA therapy.

2020 ◽  
Author(s):  
Antoine Salloum ◽  
Nagham Bazzi ◽  
Maya Habre

UNSTRUCTURED Bullous pemphigoid is among the commonest pruritic skin lesion and affecting mainly the elderly. It is caused by an immunologic reaction between auto-antibodies and hemidesmosome proteins. Several forms of typical bullous pemphigoid post linaglipltin have been reported. However, this is the first reported case of atypical non-bullous pemphigoid after linagliptin intake. The patient presented with multiple erythematous papule and nodules on the upper extremity and on trunk. Diagnosis was made with biopsy followed by direct immunofluorescence. Direct immunofluorescence showed linear IgG and C3 antibodies to hemidesmosomes at lamina Lucida of the basement membrane.


2014 ◽  
Vol 18 (6) ◽  
pp. 392-396 ◽  
Author(s):  
Chris Sladden ◽  
Mark G. Kirchhof ◽  
Richard I. Crawford

Background: Bullous pemphigoid (BP) is an autoimmune polymorphic skin disease characterized by erythematous papules and plaques and tense bullae. A skin biopsy for direct immunofluorescence (DIF) is used to detect autoantibodies and complement proteins. Objective: We sought to determine which location would provide the highest probability of obtaining a positive DIF result. Method: We undertook a retrospective chart review of 1,423 DIF biopsies. Biopsies with a clinical suspicion of BP were designated as either lesional, perilesional, or indeterminate. Results: Fifty percent of lesional DIF biopsies were positive, whereas 22% of perilesional and 12% of indeterminate biopsies had a positive DIF result. The odds ratio of a positive DIF from a lesional versus perilesional biopsy site was found to be 3.45 (95% CI 1.44–8.29). Conclusion: Clinicians are more likely to obtain a positive DIF result from a lesional nonbullous skin biopsy than from a perilesional or normal skin biopsy.


2017 ◽  
Vol 22 (1) ◽  
pp. 22-24 ◽  
Author(s):  
Kerry M. Gardner ◽  
Richard I. Crawford

Background: It has been postulated that periodic acid–Schiff staining of basement membrane can predict direct immunofluorescence patterns seen in epidermolysis bullosa acquisita and bullous pemphigoid. It has also been suggested that the type of inflammatory infiltrate or presence of fraying of basal keratinocytes may differentiate these two conditions. Objective: In this study, we aimed to confirm these observations. Methods: We reviewed 13 cases of direct immunofluorescence-confirmed epidermolysis bullosa acquisita and 19 cases of direct immunofluorescence-confirmed bullous pemphigoid, all with a subepidermal blister in the routinely processed specimen. The gold standard for diagnosis of epidermolysis bullosa acquisita vs bullous pemphigoid was taken to be identification of immune deposits on the dermal side (‘floor’ for epidermolysis bullosa acquisita) or the epidermal side (‘roof’ for bullous pemphigoid) of the salt-split direct immunofluorescence specimen. Our tests to distinguish epidermolysis bullosa acquisita from bullous pemphigoid on the routinely processed biopsy included periodic acid–Schiff basement membrane on the blister roof, neutrophilic infiltrate, lack of eosinophilic infiltrate, and absence of keratinocyte fraying. Results: Sensitivity and specificity for each test were as follows: periodic acid–Schiff staining of roof (sensitivity 25%, specificity 95%), neutrophilic infiltrate (sensitivity 54%, specificity 74%), lack of eosinophilic infiltrate (sensitivity 92%, specificity 68%), and absence of keratinocyte fraying (sensitivity 62%, specificity 58%). Conclusions: Features in the routinely processed biopsy were unable to reliably distinguish between epidermolysis bullosa acquisita and bullous pemphigoid. Direct immunofluorescence on salt-split skin remains the standard for differentiation.


Author(s):  
K. A. Holbrook

The dermal-epidermal junction (DEJ), or basement membrane rone, is the boundary between the epithelial and mesenchymal compartments of the skin; epidermal and fibroblastic cells in these two regions collaborate to synthesire its components. Ultrastructural studies (TEM and SEM) have defined a series of planes or layers (basal epidermal, lamina lucida, lamina densa, sublamina densa) and the morphology and density of attachment structures (hemidesmosomes, anchoring filaments, anchoring fibrils and anchoring plaques) in this region of normal skin. Change in structure of the DEJ provides information about the history of the tissue; reduplication of the lamina densa, for example, indicates a site of cell detachment or migration, or remodelling that accompanies repair of focal damage. In normal skin the structure of the DEJ is stable; in pathologic conditions it can be compromised by the congenital absence of certain structures or antigens (e.g., in the inherited disorders, epidermolysis bullosa [EB]) or by enzymatic degradation (e.g., in tumor invasion). Dissolution of the DEJ can also occur normally during the formation of epidermal appendages (e.g., hair follicles) and as melanocytes and Langerhans cells migrate into the epidermis during development.Biochemical and immunohisto/cytochemical studies have identified more than 20 molecules at the DEJ. These include well known matrix molecules (e.g., types IV and V collagen, laminin and fibronectin) and skin-specific antigens. The latter have been identified by autoantibodies or specific polyclonal or monoclonal antibodies raised against the skin, cultured cells and other epithelia. Some of the molecules of the DEJ are are present in basement membrane zones of many epithelia and thus are considered ubiquitous components (type IV, V, laminin, fibronectin, nidogen, entactin, HSPG, LDA-1, CSP [3B3]). All of them (that have been investigated in developing skin) appear ontogenetically as early as human embryonic tissue can be obtained and their expression is typically normal in patients with EB. The known properties of many of these molecules (particularly the matrix components) suggest functions they might impart to the DEJ: support of an epithelium (type IV collagen), regulation of permeability (heparan sulfate proteoglycan) or facilitation of cell attachment (fibronectin) and movement (laminin). Another group of matrix components and antigens of the DEJ includes molecules that are skin-specific or characteristic of stratified squamous epithelia (type VII collagen=LH 7:2 antigen, bullous pemphigoid antigen, AA3, GB3, KF-1,19-DEJ-1, epidermolysis bullosa acquisita antigen [EBA], AF-1 and AF-2, cicatricial pemphigoid antigen [CPA]) . These molecules are expressed in the DEJ later in development than the first group of molecules, in conjunction with the morphologic appearance of the structure they represent. Their appearance is also coordinated with specific developmental events (e.g., epidermal stratification) and the expression of molecules of differentiation in the epidermis and dermis. One or more of them is typically absent or reduced in expression in the skin of patients with heritable disorders affecting this region. There is no apparent correlation between the location of molecules in the DEJ and the stability of their expression.


2021 ◽  
Vol 42 (Supplement_1) ◽  
pp. S27-S27
Author(s):  
Bonnie C Carney ◽  
Taryn E Travis ◽  
Romina Deldar ◽  
Lauren T Moffatt ◽  
Laura S Johnson ◽  
...  

Abstract Introduction Dyschromic hypertrophic scar (HTS) with areas of hyper- and hypo-pigmentation is a common sequelae of burn injury. The mechanism behind the development of dyschromia has not been elucidated. In this study, we provide a histological analysis of these scars with a focus on rete ridge presence. Rete ridges occur in epithelial tissues such as oral mucosa and skin and can be described as undulating “pegs” that are interdigitated with dermal papillae. Rete ridges enhance adhesion of the epidermis to the dermis. We hypothesize that rete ridge presence is important for normal skin physiology, and their absence or presence may hold mechanistic significance in post-burn HTS dyschromia. Methods Subjects with post-burn dyschromic HTS were consented and enrolled (n=27). Punch biopsies of hyper-, hypo-, and normally pigmented scar and skin were collected and stored in formalin. Biopsies were paraffin embedded, sectioned, stained with H&E, and imaged. The number of rete ridges were investigated by calculating a rete ridge ratio from the length of the basement membrane and the length of the epidermis. Results The patient population was predominantly female (55.5%), black (70.4%), and had Fitzpatrick skin Type V (51.9%). The injuries were primarily as a result of flame (37%) and scald (33.3%) and resulted in a median TBSA burn of 7%. The median age of the scar at the time of sample acquisition was 12.2 months. The rete ridge ratio of normally pigmented, un-injured skin was above 1 (1.31 ± 0.04), indicating that normal skin’s basement membrane is longer than its epidermal length due to the presence of rete ridges. HTSs resulting from burn wounds that healed without split thickness autografts were first investigated. The number of rete ridges was higher in normal skin compared to HTS that was either hypo- or hyperpigmented (1.31 ± 0.04 vs. 1.13 ± 0.05 and 1.14 ± 0.04 vs, p< 0.05). This difference was similar despite pigmentation phenotype. When hyper-pigmented scars resulting from wounds that were treated with split thickness autografts (Hyper(+)) were investigated, rete ridge number was significantly higher than in Hyper(-) (1.89 ± 0.23, p< 0.01). Patient age showed a weak correlation (R=-0.33) with rete ridge ratio where older patients had lower rete ridge ratios in normal, un-injured skin. Hyper(+) showed a weak correlation between rete ridge ratio and age of scar (R=-0.38). Conclusions Post-burn HTS that is dyschromic has fewer rete ridges than normal skin. This finding may explain the decreased epidermal barrier function that is associated with HTS.


Dermatology ◽  
1996 ◽  
Vol 193 (1) ◽  
pp. 74-75
Author(s):  
N. Delpuget-Berlin ◽  
P. Bernard ◽  
C. Bedane ◽  
P.-M. Dang ◽  
J.-M. Bonnetblanc

2016 ◽  
Vol 8 (3) ◽  
pp. 278-282 ◽  
Author(s):  
Prajwal Boddu ◽  
Mojtaba Nadiri ◽  
Owais Malik

Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician. Diagnosis requires a careful review of clinical history, attention to detail on physical and histomorphological examination, and appropriate immunofluorescence testing. We describe the case of a 73-year-old female who presented to our hospital with a painful blistering skin rash developed over 2 days. Examination of the skin was remarkable for numerous flaccid hemorrhagic bullae on a normal-appearing nonerythematous skin involving both the upper and lower extremities. Histopathology of the biopsy lesion showed interface change at the epidermo-dermal region with subepidermal blister formation, mild dermal fibrosis, and sparse interstitial neutrophilic infiltrate. Immunohistological analysis was significant for positive IgG basement membrane zone antibodies with a dermal pattern of localization on direct immunofluorescence and positive IgG antinuclear antibodies on indirect immunofluorescence. Evidence of antibodies to type VII collagen suggested the diagnosis of epidermolysis bullosa acquisita versus bullous systemic lupus erythematosus (BSLE). A diagnosis of BSLE was made based on positive American College of Rheumatology criteria, acquired vesiculo-bullous eruptions with compatible histopathological and immunofluorescence findings. This case illustrates one of many difficulties a physician encounters while arriving at a diagnosis from a myriad of immunobullous dermatoses. Also, it is important for internists and dermatologists alike to be aware of and differentiate this uncommon and nonspecific cutaneous SLE manifestation from a myriad of disorders presenting with vesiculobullous skin eruptions in the elderly.


2021 ◽  
Author(s):  
Meriem Belheouane ◽  
Britt M Hermes ◽  
Nina Van Beek ◽  
Sandrine Benoit ◽  
Philippe Bernard ◽  
...  

Bullous pemphigoid (BP) is an autoimmune skin blistering disease afflicting mostly the elderly and is associated with significantly increased mortality. Here, we conducted the most extensive sampling effort of skin microbiota in BP to date to analyze whether intra-individual, body-site-specific, and/or geographical variation contributes to the emergence of BP. We find marked differences in the skin microbiota of BP patients compared to that of control subjects, and moreover that disease status rather than skin biogeography governs the skin microbiota composition in BP. Our data reveal a discernible transitional stage between normal and diseased skin in BP characterized by a loss of protective microbiota and an increase in sequences matching Staphylococcus aureus, a known inflammation-promoting species. Notably, S. aureus is ubiquitously associated with disease status, suggesting that this taxon is an important indicator of BP. Importantly, differences in a few key indicator taxa are able to reliably discriminate between BP patients and controls, characterized by their opposing abundance patterns. This may serve as valuable information for assessing disease risk and treatment outcomes. Future research will focus on the functional analysis of host-microbe and microbe-microbe interactions and the relevance of the host genome for microbiota abundances to identify novel BP treatment approaches.


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