Eosinophilic pustular folliculitis: A published work‐based comprehensive analysis of therapeutic responsiveness

Eosinophilic pustular folliculitis (EPF) is a non‐infectious inflammatory dermatosis of unknown etiology that principally affects the hair follicles. There are three variants of EPF: (i) classic EPF; (ii) immunosuppression‐associated EPF, which is subdivided into HIV‐associated (IS/HIV) and non‐HIV‐associated (IS/non‐HIV); and (iii) infancy‐associated EPF. Oral indomethacin is efficacious, especially for classic EPF. No comprehensive information on the efficacies of other medical management regimens is currently available. In this study, we surveyed regimens for EPF that were described in articles published between 1965 and 2013. In total, there were 1171 regimens; 874, 137, 45 and 115 of which were applied to classic, IS/HIV, IS/non‐HIV and infancy‐associated EPF, respectively. Classic EPF was preferentially treated with oral indomethacin with efficacy of 84% whereas topical steroids were preferred for IS/HIV, IS/non‐HIV and infancy‐associated EPF with efficacy of 47%, 73% and 82%, respectively. Other regimens such as oral Sairei‐to (a Chinese–Japanese herbal medicine), diaminodiphenyl sulfone, cyclosporin and topical tacrolimus were effective for indomethacin‐resistant cases. Although the preclusion of direct comparison among cases was one limitation, this study provides a dataset that is applicable to the construction of therapeutic algorithms for EPF.     

Eosinophilic pustule smear in the diagnosis of pediatric post-transplant eosinophilic folliculitis

Hematologic-associated eosinophilic pustular folliculitis is a subtype of eosinophilic pustular folliculitis (EPF) which develops in patients with underlying hematological malignancies after treatment with chemotherapy, bone marrow transplant (BMT), or stem cell transplant (SCT). Few cases of hematological-associated EPF have been reported in pediatric patients. Skin biopsy is considered the gold standard for diagnosis. We describe a case in which Wright staining of a pustule smear for eosinophils provided data to rapidly support a clinical diagnosis of hematologic-associated EPF.     

Clinical and histopathological differential diagnosis of eosinophilic pustular folliculitis

Eosinophilic pustular folliculitis (EPF) is an inflammatory disease characterized by repeated pruritic follicular papules and pustules arranged in arcuate plaques, and folliculotropic infiltration of eosinophils. The diagnosis of EPF is occasionally difficult and problematic because EPF may share the clinical appearance and histological findings with other diseases. Moreover, EPF has several clinical subtypes, including the classical type, infantile type and immunosuppression‐associated type. Because the therapies of EPF are relatively specific as compared to eczematous disorders, accurate diagnosis is essential for the management of EPF. Clinical differential diagnoses include tinea, acne, rosacea, eczematous dermatitis, granuloma faciale, autoimmune annular erythema, infestations and pustular dermatosis. Histologically, cutaneous diseases with eosinophilic infiltrates can be differentially diagnosed. Follicular mucinosis, mycosis fungoides and other cutaneous T‐cell lymphomas are the most important differential diagnoses both clinically and histopathologically. It should be kept in mind particularly that the initial lesions of cutaneous T‐cell lymphoma resemble EPF.     

Eosinophilic pustular folliculitis: The transition in sex differences and interracial characteristics between 1965 and 2013

Eosinophilic pustular folliculitis (EPF) is characterized by a non‐infectious infiltration of eosinophils in the hair follicles. It has three variants: (i) classic EPF; (ii) immunosuppression‐associated EPF, which herein is subdivided into HIV‐associated (IS/HIV) and non‐HIV‐associated (IS/non‐HIV); and (iii) infancy‐associated EPF (I‐EPF). The rarity of EPF has hindered our understanding of this entity. To examine the characteristics of EPF, with respect to age, sex, race, and chronology, published in case reports to date, we queried PubMed using the following terms: (“eosinophilic pustular folliculitis” [All Fields] OR “eosinophilic folliculitis” [All Fields]) AND (“1965/1/1” [PDAT]: “2013/12/31” [PDAT]). Additional Japanese cases were collected from Igaku Chuo Zasshi through Ichushi‐Web, JDream III, and secondhand quotations from domestic periodicals published in Japan. Proceedings were excluded. The PubMed search produced 275 citations containing 358 cases of EPF (224 men, 132 women, and two of unspecified sex); these cases involved classic EPF (101 Japanese and 81 non‐Japanese), IS/HIV (4 Japanese and 85 non‐Japanese), IS/non‐HIV (4 Japanese and 20 non‐Japanese), and I‐EPF (4 Japanese and 59 non‐Japanese). Ichushi generated an additional 148 citations containing 207 cases of Japanese (148 men and 59 women), which included cases of classic EPF (181 cases), IS/HIV (14 cases), IS/non‐HIV (9 cases), and I‐EPF (3 cases). There was no sex difference in the classic EPF cases reported between 2003 and 2013, whereas IS/HIV, IS/non‐HIV, and I‐EPF were predominated by men. There is room for reconsideration of sex differences, particularly with regard to classic EPF. The rarity and specificity of I‐EPF in Japan may reflect a state of uncertainty about this entity.     

Eosinophilic Pustular Folliculitis in Children after Stem Cell Transplantation: An Eruption Distinct from Graft‐Versus‐Host Disease

Eosinophilic pustular folliculitis (EPF) is a rare cutaneous disorder that typically occurs in three clinical contexts: men, individuals who are immunosuppressed or have human immunodeficiency virus, and infants. A fourth subtype occurring 2 to 3 months after hematopoietic stem cell transplantation (HSCT) has recently been described in several adults. We report two cases of EPF arising in children after HSCT. It is important to recognize this form of EPF after HSCT and differentiate it from graft‐versus‐host disease since it responds readily to topical steroids and appears to have an excellent prognosis.     

Eosinophilic pustular folliculitis associated with hematological disorders: A report of two cases and review of Japanese literature

Eosinophilic pustular folliculitis (EPF) occurs in patients with hematological disorders. However, clinical information about hematological disorder‐associated EPF is scarce. We report two cases of EPF associated with mantle cell lymphoma and reviewed the available published work on Japanese cases. We identified a total of 23 Japanese cases, including the two cases reported here, who had hematological disorder‐associated EPF. Fourteen cases were associated with treatment for hematological malignancies (transplantation‐related EPF) and nine cases were associated with hematological malignancies themselves (hematological malignancy‐related EPF). Although the skin eruption was clinically indistinguishable between the two subtypes, transplantation‐related EPF occurred on the face and trunk of young and middle‐aged men and women, whereas hematological malignancy‐related EPF occurred mostly on the face of older men. Peripheral blood eosinophilia was more frequently observed in transplantation‐related EPF. These observations suggest variations among patients with EPF associated with hematological disorders.     

Eosinophilic pustular folliculitis: A review of the Japanese published works

Eosinophilic pustular folliculitis (EPF), also known as Ofuji's disease, is an inflammatory dermatosis that was first described in Japan in 1970. More than 300 cases have been reported so far, and 113 Japanese cases have been reported in Japan since 1980. To comprehend the characteristics of Japanese EPF cases, we classified these cases into three types: classic, immunosuppression‐associated (IS‐EPF), and infancy‐associated (I‐EPF). Trends in age of onset and in distribution and characterization of eruptions differed between the types. We found 91 cases of classic EPF (mean age, 39.7 years), consisting of 66 males (73%) and 25 females (27%), in most of which eruptions primarily affected the face; 18 cases of IS‐EPF (44.2 years), consisting of 15 males (83%) and three females (17%), in which eruptions affected the face less predominantly; and four cases of I‐EPF (7.0 years), consisting of two males (50%) and two females (50%), primarily affecting the scalp. The number of IS‐EPF cases has increased since the late 1990s, reflecting the increasing number of HIV‐positive patients in Japan. Systemic non‐steroidal anti‐inflammatory drugs were effective in more than 70% of cases. Dimethyl diphenyl sulfone, antibiotics including minocycline, psoralen plus ultraviolet A therapy and ultraviolet B treatments worked in some cases. Topical steroids and tacrolimus were also effective in some cases of EPF, while topical indomethacin was less effective.     

In vivo confocal microscopy for diagnosis of melanoma and Basal cell carcinoma using a two-step method: analysis of 710 consecutive clinically equivocal cases

We describe two algorithms to diagnose basal cell carcinomas (BCCs) and melanomas (MMs) using in vivo reflectance confocal microscopy (RCM). A total of 710 consecutive cutaneous lesions excised to exclude malignancy (216 MMs, 266 nevi, 119 BCCs, 67 pigmented facial macules, and 42 other skin tumors) were imaged by RCM. RCM features were correlated with pathology diagnosis to develop diagnostic algorithms. The diagnostic accuracy of the BCC algorithm defined on multivariate analysis of the training set (50%) and tested on the remaining cases was 100% sensitivity, 88.5% specificity. Positive features were polarized elongated features, telangiectasia and convoluted vessels, basaloid nodules, and epidermal shadowing corresponding to horizontal clefting. Negative features were non-visible papillae, disarrangement of the epidermal layer, and cerebriform nests. Multivariate discriminant analysis on the training set (excluding the BCCs) identified seven independently significant features for MM diagnosis. The diagnostic accuracy of the MM algorithm on the test set was 87.6% sensitivity, 70.8% specificity. The four invasive MMs that were misdiagnosed by RCM were all of nevoid subtype. RCM is a highly accurate non-invasive technique for BCC diagnosis. Good diagnostic accuracy was achieved also for MM diagnosis, although rare variants of melanocytic tumors may limit the strict application of the algorithm.     

High discordance between punch biopsy and excision in establishing basal cell carcinoma subtype: analysis of 500 cases

Background Basal cell carcinoma (BCC) is the most frequently occurring cancer in humans. Worldwide incidences rise about 10% each year, increasing the burden on dermatologists, general practitioners and pathologists as well as increasing costs for the health care system. Increasingly non‐surgical treatment options are used in the treatment of BCC, without histological confirmation of BCC subtype, potentially resulting in under‐treatment. Objective We evaluated the diagnostic accuracy of a punch biopsy for the BCC histological subytpe in a primary BCC and the prevalence of biopsy‐based under‐diagnosis of aggressive subtypes. Accuracy of a punch biopsy was defined as concordance of the diagnosis of subtype of BCC at punch biopsy and excision. Methods A retrospective chart‐review was performed of primary BCC, which were proven by punch biopsy and subsequently treated by excision. The first 100 consecutive BCCs per year during the years 2004–2009 were included, yielding a total of 500 evaluated BCCs. Results The overall accuracy of punch biopsy for BCC subtype at excision was 69%, in single‐type BCC 83% (n = 343) and in mixed‐type BCC 37% (n = 157). Accuracy varied substantially according to BCC subtype, being highest in the superficial subtype (84%) and subsequently in infiltrative (69%), nodular (63%) and micronodular subtype (38%). In 11% of all cases, an unsuspected more aggressive subtype was present. Conclusion Punch biopsy has a high accuracy in single‐type BCCs and a considerably lower accuracy in mixed‐type BCCs for establishing BCC subtype compared to excision. The presence of an unsuspected aggressive subtype could explain therapy failure of non‐surgical treatments like imiquimod or photodynamic therapy.     

Predictive Model for Differential Diagnosis of Inflammatory Papular Dermatoses of the Face

BackgroundThe clinical features of inflammatory papular dermatoses of the face are very similar. Their clinical manifestations have been described on the basis of a small number of case reports and are not specific.ObjectiveThis study aimed to use computer-aided image analysis (CAIA) to compare the clinical features and parameters of inflammatory papular dermatoses of the face and to develop a formalized diagnostic algorithm based on the significant findings.MethodsThe study included clinicopathologically confirmed inflammatory papular dermatoses of the face: 8 cases of eosinophilic pustular folliculitis (EPF), 13 of granulomatous periorificial dermatitis-lupus miliaris disseminatus faciei (GPD-LMDF) complex, 41 of granulomatous rosacea-papulopustular rosacea complex (GR-PPR) complex, and 4 of folliculitis. Clinical features were evaluated, and area density of papular lesions was quantitatively measured with CAIA. Based on these variables, we developed a predictive model for differential diagnosis using classification and regression tree analysis.Results The EPF group showed lesion asymmetry and annular clusters of papules in all cases. The GPD-LMDF complex group had significantly higher periocular density. The GR-PPR complex group showed a higher area density of unilateral cheek papules and the highest total area density. According to the predictive model, 3 variables were used for differential diagnosis of the 4 disease groups, and each group was diagnosed with a predicted probability of 67%~100%.ConclusionWe statistically confirmed the distinct clinical features of inflammatory papular dermatoses of the face and proposed a diagnostic algorithm for clinical diagnosis.     

Validation of a diagnostic algorithm for the discrimination of actinic keratosis from normal skin and squamous cell carcinoma by means of high‐definition optical coherence tomography

Actinic keratoses (AKs) commonly arise on sun‐damaged skin. Visible lesions are often associated with subclinical lesions on surrounding skin, giving rise to field cancerization. To avoid multiple biopsies to diagnose subclinical/early invasive lesions, there is an increasing interest in non‐invasive diagnostic tools, such as high‐definition optical coherence tomography (HD‐OCT). We previously developed a HD‐OCT‐based diagnostic algorithm for the discrimination of AK from squamous cell carcinoma (SCC) and normal skin. The aim of this study was to test the applicability of HD‐OCT for non‐invasive discrimination of AK from SCC and normal skin using this algorithm. Three‐dimensional (3D) HD‐OCT images of histopathologically proven AKs and SCCs and images of normal skin were collected. All images were shown in a random sequence to three independent observers with different experience in HD‐OCT, blinded to the clinical and histopathological data and with different experience with HD‐OCT. Observers classified each image as AK, SCC or normal skin based on the diagnostic algorithm. A total of 106 (38 AKs, 16 SCCs and 52 normal skin sites) HD‐OCT images from 71 patients were included. Sensitivity and specificity for the most experienced observer were 81.6% and 92.6% for AK diagnosis and 93.8% and 98.9% for SCC diagnosis. A moderate interobserver agreement was demonstrated. HD‐OCT represents a promising technology for the non‐invasive diagnosis of AKs. Thanks to its high potential in discriminating SCC from AK, HD‐OCT could be used as a relevant tool for second‐level examination, increasing diagnostic confidence and sparing patients unnecessary excisions.     

Eosinophilic pustular folliculitis starting initially only with palmoplantar pustular lesions. Report of a case and review of the literature.

We report a 23-year-old Japanese male with eosinophilic pustular folliculitis (EPF) that had started with palmoplantar rash. Only when follicular pustules appeared on the bilateral cheek 31 months later, we revised our initial diagnosis of pustulosis palmaris et plantaris (PPP) to EPF, and all the skin eruptions cleared mostly with indomethacin. A review of the Japanese literature for the past 20 years disclosed that in 207 cases of EPF so far reported, palmoplantar pustular lesion was noted in 38 (18%). Among them, in 16 cases (8%) the skin lesions started first from the palmoplantar region with the average period of 26 months until the appearance of other eruptions of EPF. None of them was diagnosed as EPF when skin lesions were localized only to the palmoplantar region. When we find pustules on the palmoplantar region, we should suspect the possibility of early lesions of EPF as well as PPP. Histopathologic demonstration of multilocular pustules located in the upper epidermis containing numerous eosinophils in the palmoplantar pustular lesions, together with the dramatic therapeutic response to indomethacin greatly favor the diagnosis of EPF.     

Trichoscopy as a useful method to differentiate tinea capitis from alopecia areata in children at Zagazig University Hospitals

Trichoscopy corresponds to the scalp, and hair dermoscopy has been increasingly used as an aid in the diagnosis, follow‐up, and prognosis of hair disorders. Trichoscopy represents a valuable link between clinical and histological diagnosis. Tinea capitis (TC) and alopecia areata (AA) are considered the most common causes of hairless patches of the scalp in pediatrics. TC may have the same clinical appearance of AA, so dermoscopy has recently become a useful diagnostic tool for AA and TC, particularly in doubtful cases. The aim of this study is to identify the trichoscopic features of TC and AA in children that may facilitate in their differentiation from each other and choosing the appropriate treatment, which is a non‐invasive method of diagnosis.     

Pattern analysis,not simplified algorithms,is the most reliable method for teaching dermoscopy for melanoma diagnosis to residents in dermatology

BACKGROUND: Simplified algorithms for dermoscopy in melanoma diagnosis were developed in order to facilitate the use of this technique by non-experts. However, little is known about their reliability compared with classic pattern analysis when taught to untrained observers. OBJECTIVES: To investigate the diagnostic performance of three different methods, i.e. classic pattern analysis and two of the most used algorithms (the ABCD rule of dermoscopy and the seven-point check-list) when used by newly trained residents in dermatology to diagnose melanocytic lesions. Methods Five residents in dermatology (University of Florence Medical School) were submitted to a teaching programme in dermoscopy based on both formal lessons and training and self-assessment using a newly developed, interactive CD-ROM on dermoscopy. The performance of the three diagnostic methods was analysed in a series of 200 clinically equivocal melanocytic lesions including 44 early melanomas (median thickness 0.30 mm; 25th-75th percentile 0.00-0.58 mm). RESULTS: Pattern analysis yielded the best mean diagnostic accuracy (68.7%), followed by the ABCD rule (56.1%) and the seven-point check-list (53.4%, P = 0.06). The best sensitivity was associated with the use of the seven-point check-list (91.9%), which, however, provided the worst specificity (35.2%) of the methods tested. The interobserver reproducibility, as shown by kappa statistics, was low for all the methods (range 0.27-0.33) and did not show any statistical difference among them. CONCLUSIONS: Pattern analysis, i.e. simultaneous assessment of the diagnostic value of all dermoscopy features shown by the lesion, proved to be the most reliable procedure for melanoma diagnosis to be taught to residents in dermatology.     

Diagnosing chronic hand eczema by an algorithm: a tool for classification in clinical practice

Background. Diagnostic classification of chronic hand eczema (CHE) represents a major clinical and taxonomic challenge because of its wide aetiological and clinical heterogeneity. Aim. To develop an algorithm for the diagnosis and classification of CHE. Methods. Well‐defined aetiological, clinical and morphological criteria of CHE were arranged graphically in a diagnostic hierarchy, and validated in 137 German patients with hand eczema. Results. The algorithm distinguished chronic hand eczema due to contact allergy, irritant damage or a combination of the two, each either with or without atopy, and also atopic hand eczema and idiopathic hand eczema lacking obvious causative factors. Foot involvement helped to distinguish idiopathic from irritant hand eczema. Each subtype could occur either with a hyperkeratotic–rhagadiform, dyshidrotic or mixed morphology, but certain hand eczema subtypes had clear morphological preferences. Conclusions. By providing a more precise clinical definition this diagnostic algorithm could improve the classification and taxonomy of hand eczema subtypes, facilitating more rational treatment decisions and allowing better treatment outcome analysis.     

Systematic review of dermoscopy and digital dermoscopy/ artificial intelligence for the diagnosis of melanoma

Background Dermoscopy improves diagnostic accuracy of the unaided eye for melanoma, and digital dermoscopy with artificial intelligence or computer diagnosis has also been shown useful for the diagnosis of melanoma. At present there is no clear evidence regarding the diagnostic accuracy of dermoscopy compared with artificial intelligence. Objectives To evaluate the diagnostic accuracy of dermoscopy and digital dermoscopy/artificial intelligence for melanoma diagnosis and to compare the diagnostic accuracy of the different dermoscopic algorithms with each other and with digital dermoscopy/artificial intelligence for the detection of melanoma. Methods A literature search on dermoscopy and digital dermoscopy/artificial intelligence for melanoma diagnosis was performed using several databases. Titles and abstracts of the retrieved articles were screened using a literature evaluation form. A quality assessment form was developed to assess the quality of the included studies. Heterogeneity among the studies was assessed. Pooled data were analysed using meta‐analytical methods and comparisons between different algorithms were performed. Results Of 765 articles retrieved, 30 studies were eligible for meta‐analysis. Pooled sensitivity for artificial intelligence was slightly higher than for dermoscopy (91% vs. 88%; P = 0·076). Pooled specificity for dermoscopy was significantly better than artificial intelligence (86% vs. 79%; P < 0·001). Pooled diagnostic odds ratio was 51·5 for dermoscopy and 57·8 for artificial intelligence, which were not significantly different (P = 0·783). There were no significance differences in diagnostic odds ratio among the different dermoscopic diagnostic algorithms. Conclusions Dermoscopy and artificial intelligence performed equally well for diagnosis of melanocytic skin lesions. There was no significant difference in the diagnostic performance of various dermoscopy algorithms. The three‐point checklist, the seven‐point checklist and Menzies score had better diagnostic odds ratios than the others; however, these results need to be confirmed by a large‐scale high‐quality population‐based study.     

Comparison of dermatoscopic diagnostic algorithms based on calculation: The ABCD rule of dermatoscopy,the seven‐point checklist,the three‐point checklist and the CASH algorithm in dermatoscopic evaluation of melanocytic lesions

Dermatoscopic analysis of melanocytic lesions using the CASH algorithm has rarely been described in the literature. The purpose of this study was to compare the sensitivity, specificity, and diagnostic accuracy rates of the ABCD rule of dermatoscopy, the seven‐point checklist, the three‐point checklist, and the CASH algorithm in the diagnosis and dermatoscopic evaluation of melanocytic lesions on the hairy skin. One hundred and fifteen melanocytic lesions of 115 patients were examined retrospectively using dermatoscopic images and compared with the histopathologic diagnosis. Four dermatoscopic algorithms were carried out for all lesions. The ABCD rule of dermatoscopy showed sensitivity of 91.6%, specificity of 60.4%, and diagnostic accuracy of 66.9%. The seven‐point checklist showed sensitivity, specificity, and diagnostic accuracy of 87.5, 65.9, and 70.4%, respectively; the three‐point checklist 79.1, 62.6, 66%; and the CASH algorithm 91.6, 64.8, and 70.4%, respectively. To our knowledge, this is the first study that compares the sensitivity, specificity and diagnostic accuracy of the ABCD rule of dermatoscopy, the three‐point checklist, the seven‐point checklist, and the CASH algorithm for the diagnosis of melanocytic lesions on the hairy skin. In our study, the ABCD rule of dermatoscopy and the CASH algorithm showed the highest sensitivity for the diagnosis of melanoma.     

Management of squamous cell and basal cell carcinomas of the head and neck with perineural invasion

Perineural invasion (PNI) occurring in non‐melanoma skin cancers (NMSC) is associated with an increased risk of locoregional recurrence and reduced disease‐free survival. This necessitates early and accurate diagnosis, appropriate risk‐stratification and a clear management strategy. The diagnosis of PNI is based on careful clinical assessment, imaging and histopathology. Surgery, preferably with margin control, and definitive or adjuvant radiotherapy (ART) are established treatment strategies for PNI. Clinical uncertainty remains over the role of ART in incidental PNI. This review synthesises current literature to ascertain which clinicopathological features impart a higher risk to individuals with PNI in NMSC, in order to provide treatment algorithms, including the identification of patient subsets that are most likely to benefit from ART. This includes those with extratumoural PNI, involvement of larger‐calibre nerves, tumour invasion beyond dermis, recurrent tumour or diffuse intratumoural spread. Patients with clinical PNI may be optimally managed by a multidisciplinary head and neck cancer service that is best placed to offer skull base surgery and intensity‐modulated radiation therapy (IMRT). The management options presented are stratified by histological subtype and a new classification of PNI into low‐risk, medium‐risk and high‐risk groups.     

HIV seronegative eosinophilic pustular folliculitis successfully treated with doxicycline

Eosinophilic pustular folliculitis (EPF) is an unusual disease, first described in adult East Asians in 1970 by Ofuji. It is characterized by follicular papules and pustules tending to coalesce and form plaques involving the trunk, face and extremities. In recent years, it has been often associated with human immunodeficiency virus (HIV) infection or with immunosuppressed and/or oncohaematological patients. EPF has been described in immunocompetent adult caucasian patients only occasionally. The diagnosis requires clinical and microbiological features such as sterile folliculitis and histopathological findings characterized by folliculitis and perifolliculitis with eosinophilic infiltrate. We describe an HIV seronegative caucasian male with EPF, allergic to non-steroidal anti-inflammatory drugs and indomethacin, treated with oral doxicycline. The treatment led to the complete remission of the lesions within 2 months.     

Sensitivity of indirect immunofluorescence and ELISA in detecting intercellular antibodies in endemic pemphigus foliaceus (Fogo Selvagem)

BACKGROUND: Since 1967 dermatology has used the classic technique of indirect immunofluorescence (IFI) for the detection of autoantibodies against antigens of the skin in diseased people with endemic pemphigus foliaceus. Thirty years later enzyme-linked immunosorbent assays--ELISA (rDsg1 and rDsg3) appeared as a viable option. A group of highly recognized researchers have concluded that ELISA is a simple, sensitive and highly specific method, allowing for diagnostic differentiation between pemphigus vulgaris (PV) and endemic pemphigus foliaceus (EPF). Scientific literature certifies that both ELISA and IIF bear high sensitivity in spite of the fact that a direct comparison between the ELISA and IIF tests has never been performed. OBJECTIVES: This study was conducted to compare the sensitivity of these tests in detecting antibodies in the EPF. MATERIAL AND METHODS: Thirty-two serum samples were collected from patients with EPF. The control serum of 15 healthy individuals was tested to detect the presence of antibodies of EPF by indirect immunofluorescence and ELISA (rDsg1 and rDsg3). The IIF was performed, taking human skin as a substrate. RESULTS: Antibodies in patients with EPF were detected more commonly by the ELISA (rDsg1) (91%) compared with IIF (81%). CONCLUSIONS: The ELISA (rDsg1) is slightly more sensitive than IIF in detecting antibodies related to EPV. However, according to our results, we do not currently possess a test with 100% accuracy in differentiating EPF from PV. Although previous studies have associated Dsg3 with PV, the tests performed during this study showed that 12% (4/32) of patients with EPF (cutaneous diseases only) also had Dsg3 antibodies.