Volume 41 Number 3

Introduction

Pemphigus vulgaris (PV) is a severe autoimmune bullous dermatosis in which the antibodies destroy the desmosomes, resulting in the formation of intraepidermal blisters that affect the skin and mucous membranes. PV occurs mainly between the 4th and 6th decades of life, affecting males and females with an incidence of 0.1–0.5 / 100,000 inhabitants / year and with a mortality rate of 5–10%. The disease distribution is universal, but most commonly affects people of Jewish ancestry^–3.

In PV, autoantibodies act predominantly on desmoglein 3 (Dsg3) which is expressed predominantly in the deeper layers of the epidermis^2,3. Identifying the layer on which acantholysis occurs is a factor that assists in the diagnosis of bullous dermatoses. For example, it is possible to differentiate PV from pemphigus foliaceus by the site where acantholysis occurs, since pemphigus foliaceus affects the granular layer whereas PV affects the spinous layer^2,3. These manifestations involve the formation of blisters with consequent ulceration and skin damage which can be devastating, affecting social interaction and even loss of employment⁴.

The impact of disfigurement associated with PV on patients’ quality of life, self-image, family and social dynamics has been well documented⁴. In addition to cutaneous involvement, PV may involve mucosal tissue in the mouth, pharynx, larynx, nasal passage and ear canals (Figures 1 & 2). Areas affected by the disease can compromise normal breathing as well as the ability to speak and to eat to maintain adequate nutritional intake^2,3.

Figure 1. Cutaneous lesions on trunk

Figure 2. Oral mucosa lesions

The classification of PV has been the object of studies in recent years. The Commitment Index of Skin and Mucous in Pemphigus Vulgaris uses four different parameters to score the disease clinical status: (a) the number of blisters or eroded areas; (b) the size of blisters or eroded areas; (c) evidence of the Nikolsky sign (where sliding the finger firmly with pressure over the skin separates normal-appearing epidermis, producing an erosion); and (d) mucousal involvement and sepsis. The total score may vary from 0–100, and the patients are classified as follows: Class I score 0–30; Class II 35–65; Class III 70–100, meaning that the higher the score, the more critical the status¹.

Pemphigus classifications tools, the Pemphigus Disease Area Index (PDAI) and the Japanese Pemphigus Disease Severity Score (JPDSS), were compared by Shimizu et al.⁵. PDAI measures skin and mucosal involvement by size and number of blisters in each anatomical region, and the score ranges from 0–263. JPDSS uses parameters for scoring: (I) the ratio of affected area of skin to the body’s surface area; (II) the presence or absence of Nikolsky’s sign phenomenon; (III) the number of newly developed blisters per day; (IV) the presence or absence of oral lesions; and (V) the titer of pemphigus antibodies. Each parameter has a score ranging 0–3. In Shimizu et al.’s study⁵, the results show that PDAI more accurately reflects disease severity. The authors therefore propose the use of indexes to guide a uniform treatment according to grading criteria. Corticotherapy is the treatment of choice, and it can be associated with immunosuppressants if there is no improvement with isolated corticotherapy^2,3.

Although considered a relatively rare disease, there is a need for nurses to recognise skin lesions associated with PV and communicate appropriate findings in order to help patients seek early treatment, evaluate disease progression, and monitor responses to treatment⁶. The purpose of this integrative review was to describe the taxonomy for the description and assessment of skin changes related to PV by nurses.

Method

This integrative review was conducted to identify, analyse and synthesise studies that use qualitative, quantitative and mixed methods in this theme⁷. We have chosen the method described by Mendes⁷ to guide the review which consisted of six stages: (1) formulation of the guiding question; (2) establishment of criteria for inclusion and exclusion of studies and data collection (search in the literature); (3) categorisation of studies; (4) evaluation of studies included in the review; (5) analysis and interpretation of data; and (6) synthesis of the knowledge evidenced in the articles analysed (presentation of the results)⁷.

Formulation of the guiding question

The research question was – How are the lesions that characterise PV described in the literature in its definition and classification?

Establishment of criteria for inclusion and exclusion of studies and data collection

The medical and nursing literature was searched from 2011–2019 in conjunction with a librarian to assist in answering the research question. Searches included Web of Science, LILACS, EMBASE, SCOPUS, PUBMED, BVS, CINAHL and COCHRANE with specific inclusion and exclusion criteria. To identify relevant publications, databases were searched using the following key terms – dermatology, pemphigus, skin ulcer, diagnostic and nursing assessment. The inclusion criteria included articles published in English, Spanish and Portuguese, peer-reviewed literature and consensus documents; the dates of publication were from 1 January 2011 to 31 December 2019. Commentary and editorials were excluded.

Categorisation of studies

Selected studies were then categorised according to the six levels of evidence⁸:

• Level 1: evidence from meta-analysis of multiple controlled and randomised studies.

• Level 2: evidence from individual studies with experimental design.

• Level 3: evidence from quasi-experimental studies, time series or case-controls.

• Level 4: evidence from descriptive studies (non-experimental or qualitative approach).

• Level 5: evidence of case / experience reports.

• Level 6: evidence based on expert committee opinions, including interpretations of non-research based information, regulatory or legal opinions.

Evaluation of studies included in the review

This step included the evaluation of the studies as well as data extraction. A standardised data collection form was used to extract the following information: authors; professional category of authors; title of the article; journal; year of publication; level of evidence; goals; methodological design; sampling detail; synthesis of information; evaluation/assessment of skin ulcers in pemphigus; methodology used to validate the instrument; description of the instrument; terminology used to characterise ulcers; and results and conclusions.

Analysis and interpretation of data

The data evaluation stage included evaluating the quality of the primary sources using a specific methodological approach to determine the quality of the source. The data were evaluated and coded according to two criteria – the methodological rigour and the relevance to the topic of skin assessment. Studies were analysed and the rigour rated on a score from 0–4. The relevance to the topic was also scored and indicated, with 1 having no relevance to the topic and 2 indicating the article was relevant.

Synthesis / presentation of results

The data analysis for the qualitative studies were reviewed and were systematically categorised, analysed and synthesised, and placed into distinct themes, patterns and relationships using a matrix method. The synthesis of the studies was organised into three axis: (1) characteristics of scientific publications on PV; (2) terminology used to describe cutaneous lesions related to PV (Table 1); and (3) comparison of terminologies used in studies and dermatological description of skin lesions.

Table 1. Terms identified grouped by level of evidence of the studies

Ethical and legal aspects

The research project was submitted and approved by the Research Ethics Committee / UNIFESP: 0450/2015 and followed the ethical and legal precepts of research with human beings according to Resolution 196/96 of the National Health Council.

Results

The initial search identified 2934 articles; 356 articles were included from review of the titles. After review of the abstracts, another 258 papers were not suitable for review since they did not meet the inclusion criteria; specifically, the excluded papers did not address PV and lesion assessment. A total of 58 articles were then selected for the study after exclusion of 40 duplicates. Of the 58 articles, 37 (63.79%) were primary studies, 17 (29.31%) were series/clinical reviews and four (6.90%) were validation studies.

A variety of terms were used in the literature to describe PV-related lesions. These are summarised below.

Primary skin lesions

Flat spots or maculae

The first is flat skin lesions, including colour changes and blood-vascular stains. Nomenclature used were new erythema, erythema, minimal erythema, and marked erythema^9–15. Erythema is defined as a red colour resulting from vasodilation that disappears by digital pressure or diascopy. Diascopy is a refinement in which a piece of clear glass or plastic is pressed against the skin while the observer looks directly at the lesion under pressure^15,16.

Solid formations / oedematous elevations

Solid formations may include bullae and papulae (small superficial solid elevations of the skin) while oedematous elevations could be cutanaeous lesions, scales or pustulae (defined elevation of the skin containing purulent fluid). These clinical features might be associated with a positive Nikolsky sign¹⁷.

Pigment spots

Nomenclature used in this section were post-inflammatory hyperpigmentation and hyperpigmented macules^{5,11,16, 18–20}. Hyperchromia is defined as a spot of variable colour, caused by the increase of melanin or deposit of another pigment. The increase in melanin / melanodermic spots has a variable colour from light brown to dark bluish or black^16,18.

Liquid content

Nomenclature used here were vesicles, blisters and bullous lesions^{1,5,9,10,12,13,20–55}. Vesicles are defined as of circumscribed elevation, containing clear liquid, up to 1cm in size. The fluid, which is primitively clear (serous), may become cloudy (purulent) or red (haemorrhagic)^16,18. A blister is defined as elevation containing clear liquid, greater than 1cm in size. The fluid, which is primitively clear, may become yellowish-red or reddish, forming a purulent or haemorrhagic blister^16,18. The term bullous lesions is used to refer to any liquid collections.

Secondary skin lesions

Thickness changes

Nomenclature used here are epithelial lesions, denuded epidermis and scar^{9–11,13,19,22,35,51,54,56–58}. A scar is defined as a flat, salient or depressed lesion, without grooves, pores and hairs, movable, adherent or retractable. It associates atrophy with fibrosis and dyschromia. It results from the repair of destructive process of the skin. It can be: atrophic (thin, pleated, papyraceous); pitted (small holes appear); or hypertrophic (nodular, elevated, vascular, with excessive fibrous proliferation, with a tendency to regress)^16,18.

Tissue losses

Nomenclature used are erosions, eroded areas, erosive lesions, crust, ulceration, ulcer, ulcerative lesions, raw erosions and excoriations^{1,5,9–15,19–21,23–60}. Erosion or exulceration is defined as superficial loss that affects only the epidermis. Crust is defined as a concretion of light yellow to greenish or dark red colour which forms in an area of tissue loss. It results from the desiccation of serosity (meliceric), pus (purulent) or blood (haemorrhagic), mixed with epithelial remains^16,18.

Therefore, in view of the integrative review, it was possible to identify studies describing the dermatological alterations of PV ulcers according to criteria established by dermatological glossaries. In order to illustrate the correlation of the identified terms and their corresponding study, the third analysis correlates the terms identified with the year, author category, study category and level of evidence, shown in Table 2.

Table 2. Terms identified in selected articles

Discussion

As evidenced by the results, studies in this area are scant and nurses have had little contribution. However, this is needed to highlight the consistency demonstrated between the terms used by authors and the terminology of elementary lesions.

To promote excellence in dermatological care it is necessary for nurses and health professionals to develop skills for the evaluation of cutaneous manifestations, especially skin lesions. The skin, as the largest organ of the human body, has protection as one of its numerous functions. Far beyond essentially dermatological affections, it can translate the person’s general condition, signalling the presence of systemic disorders and sometimes presenting manifestations that are recognised as cutaneous markers of certain pathologies. However, the paucity of studies in PV has led to poor description and evaluation of skin lesions in PV. This may result in delayed diagnosis and suboptimal care and treatment^6,20,29,36.

In fact, this subject points to the need for further investigation to compose a framework of knowledge based on scientific evidence in this area, so that dermatology nurses may develop an accurate consideration of the assessment of skin manifestations, especially skin ulcers. These professionals publish little on this subject, which corroborates with the lack of formulation of new evidence for their topical care. This lack of evidence makes nursing assistance to these people less secure, since a theoretical framework is essential for evidence-based care given the specificity and complexity of caring for these patients^16,25.

The main researchers in this area, Brandão and Santos^4,6,25,29, investigate this issue from the point of view of integral care, with a social-poetic approach and a nursing diagnosis that support the comfort and relief of pain among those affected.

Through clinical observation, the authors of this study concluded that traditional topical care generates high rates of hospital stay, pain and discomfort. This clinical observation was the trigger to build evidence of the best evaluation tools and descriptors and base this on the use of topical therapies that optimise the potential of healing by managing cofactors such as pH, thermoregulation, humidity, microbial load and adherence of the dressing^25,36.

It can be observed that when the nursing descriptor was used, the literature search resulted in a much smaller number of articles in comparison to those searches without this descriptor. Although dermatological nursing is a recognised specialty in Brazil, the descriptors ‘nursing in dermatology’ or ‘dermatologist nurse’ or ‘dermatological nursing’ do not exist. Therefore, in search of studies, the descriptors ‘nursing’ and ‘nursing assessment’ were used.

Among the nomenclatures used in the different levels of studies, we can observe that all use the dermatological glossary to describe the lesions, with only a small variation with detailed description of the tissue such as exudative erosions. Despite the scarcity of studies written by nurses, it was possible to observe that the terminology used did not differ between professional categories^25,29,36.

According to Sampaio and Rivitti³, skin lesion classification is like letters of the alphabet; just as letters compose words, through their combination they form morphological signs that allow us to “read” the cutaneous manifestations^16,18. As shown in the results, the skin lesions, associated with complementary information, can help in the definition of diagnostic hypotheses, intervention decision and monitoring of healing evolution. The use of clinical descriptors to characterise skin lesions or ulcers standardises this information, making it more understandable for health professionals. In Table 3 examples of PV lesions related to the correspondent elementary skin lesion term are given.

Table 3. Elementary skin lesion terms and corresponding examples of PV lesion

Skin lesions are classified into six groups: colour changes; oedematous elevations; solid formations; liquid formations; thickness changes; and losses and repairs. This classification may be grouped into:

• Primary: flat spots or maculae (colour changes); solid (solid formations); oedematous elevations (rash, oedema); liquid content (liquid formations).
• Secondary: changes in consistency and thickness (changes in thickness); or loss of substance (tissue losses and repairs)^16,18.

Diagnoses, interventions and nursing outcomes according to the North American Nursing Diagnosis Association (NANDA), Nursing Interventions Classification (NIC) and Nursing Outcomes Classification (NOC) for patients with PV were proposed by Pena et al.²⁷. The analysis of the nursing diagnosis ‘impaired skin integrity’ and ‘impaired oral mucous membrane’ made it possible to perceive that the nurse assessment to arrive at this diagnosis does not require a specialised evaluation since the defining characteristics for such diagnoses involve only the rupture of the skin or mucosa, not requiring evaluation of thickness, tissue type or other characteristics assessed in a wound. In the proposed NOC outcome ‘healing’, in which one of the indicators used was granulation tissue, the knowledge about the tissue types that a wound can present is necessary. The proposed NIC intervention ‘wound care’ involves the decision about the intervention to be adopted, presupposing specific knowledge, skills and preparation in this area²⁷. Considering the increasing performance of dermatology nursing as a specialty, these nomenclatures are becoming increasingly familiar to nurses.

As the nomenclature used to describe skin lesions becomes standard, whether secondary to PV or not, the interlocution between professionals tends to become more uniform, avoiding misunderstanding and resulting in safer patient care. However, in wound assessment there is still a great diversity in the terminology used to describe the skin damage, for example: slough and fibrin; wet necrosis and liquefaction necrosis; dry necrosis, ‘scar’ and pressure injury; granulation tissue, extra cellular matrix or viable tissue. This richness of terminologies causes doubt, within professionals consulting the records, often generating interpretations that differ from those of the person who registered them. In order to standardise the register of PV ulcers, we suggest the use of the elementary lesions terms – vesicles, blisters, erosions, ulcers and generalised crusts.

The chronic character and the cutaneous manifestation of PV steal from the patient their right to confidentiality. Frequently, the lesions are associated with contagion, impacting on social and familiar relations, and perpetuating stigma and isolation⁴. For Brandão, nursing actions become productive as they aim to reach the patient’s holistic balance. Thus there is a constant search for alternatives that attend to the needs of people suffering from changes in skin integrity, and the development of technologies and techniques that promote healing in order to improve their quality of life^4,27.

Conclusions

This study compared the terminology used to describe cutaneous ulcers in PV studies with the terminology used in describing elementary lesions. It also demonstrated that the publications authored by physicians were the most common ones, followed by those authored by nurses, and lacked uniformity in the description of the lesions.

This study demonstrated that the skin changes in PV can be classified as primary skin lesions (colour changes and liquid collections) or secondary skin lesions (changes in thickness and tissue loss). In view of the extensive dermatological glossary, lesions secondary to PV can be characterised as vesicles, blisters, erosions, ulcers and generalised crusts to support the practice of nursing in dermatology.

Lastly, it is concluded that studies with a higher level of evidence in this area are necessary in order to determine the best way to evaluate the lesions, as well as the nomenclature to be used for improved nursing care.

Conflict of Interest

The authors declare no conflicts of interest.

Funding

The authors received no funding for this study.

Author(s)

Mariana Takahashi Ferreira Costa*
Master’s Degree student, School of Nursing, Federal University of São Paulo, Brazil
Nurse member of Group for Prevention and Treatment of Wounds, de Emílio Ribas Infectology Institute, State Secretary of Health, São Paulo, SP, Brazil
Email marianatakahashicosta@hotmail.com

Luiza Keiko Matsuka Oyafuso
Supervisor of Health Technical Team, Emílio Ribas Infectology Institute, State Secretary of Health, São Paulo, SP, Brazil
Assistant Professor, ABC University Foundation

Mônica Antar Gamba
Associate Professor, School of Nursing, Federal University of São Paulo, São Paulo, SP, Brazil
Coordinator of the Dermatology Nursing Specialization Course, Mentor Professor at the Nursing Postgraduation Program

Kevin Y Woo PhD RN FAPWCA
Assistant Professor, School of Nursing, Faculty of Health Sciences, Queen’s University, Kingston, ON, Canada
Adjunct Research Professor, MClSc program, School of Physical Therapy, Faculty of Health Sciences, Western University
Wound Care Consultant, West Park Healthcare Centre
Clinical Web Editor, Advances in Skin and Wound Care

* Corresponding author

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