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Background: Ibrutinib, an irreversible Bruton tyrosine kinase inhibitor, has been associated with an increased risk of bleeding. There is a paucity of data on the risk of bleeding in patients on ibrutinib undergoing dermatologic surgery., Objective: To determine the frequency of bleeding complications associated with ibrutinib in patients undergoing dermatologic surgery., Materials and Methods: A retrospective, single-center, case-control study of patients on ibrutinib undergoing skin surgery between January 2013 and March 2020 compared with sex, disease, and age-matched control patients undergoing cutaneous surgeries., Results: A total of 75 surgeries performed on 37 case patients and 116 surgeries performed on 64 control patients were included. Ibrutinib was associated with a statistically significant increased rate of bleeding events (6/75 [8%] vs 1/116 [0.8%], p -value = .02). Compared with ibrutinib patients who did not have a bleeding event, those on ibrutinib who suffered bleeding were all men, older (mean age 82.7 vs 73.0, p -value= .01), and had lower mean platelet counts (104.0 vs 150.5 K/μL, p -value = .03)., Conclusion: Ibrutinib may be associated with increased risk of bleeding in patients with hematologic malignancies, particularly older men with lower platelet levels and on multiple anticoagulants. Transient discontinuation of ibrutinib should be considered for dermatologic surgeries., (Copyright © 2022 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Autoimmune progesterone dermatitis (APD) is a rare hypersensitivity disorder characterized by recurring dermatologic manifestations during the luteal phase of the menstrual cycle in women. Well-defined clinical and diagnostic criteria, outcomes measurements, and standard treatments are lacking., Methods: We performed a single-institution retrospective review of adult patients (older than 20 years at the time of diagnosis) with APD., Results: Fourteen patients were included with mean age of clinical onset of 34.3 ± 7.7 (range 24-54) years. There was a delay of 3.9 ± 5.5 (range 0.4-20) years between the onset of disease symptoms and diagnosis. The onset of APD was after exposure to exogenous progesterone in 9 of 14 patients. Progesterone skin test was performed in 9 patients and 6 were positive. Patients frequently presented with urticaria (9/14, 64.3%) and dermatitis (4/14, 28.6%). Continuous combined oral contraceptives (4/14, 28.6%), gonadotropin-releasing hormone agonist (3/14, 21.4%), and hysterectomy with bilateral salpingo-oophorectomy (2/14, 14.3%) were the most common attempted treatments with reliable outcomes., Conclusions: APD is a rare disorder which lacks universal diagnostic measures and criteria, contributing to a significant delay in diagnosis. Large-scale multicenter studies are needed to develop accurate tests, establish diagnostic criteria, and define treatment outcomes., Competing Interests: None., (Copyright © 2022 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of Women’s Dermatologic Society.)

Background/objective: Autoimmune progesterone dermatitis (APD) is a rare autoimmune hypersensitivity reaction that occurs cyclically at the peak of endogenous progesterone production during the menstrual cycle in women. No study characterizing APD in the adolescent population is found; it appears likely to be underdiagnosed and undertreated., Methods: A retrospective, single-center, review of all adolescent and pediatric patients (<20 years old at onset) with documented diagnosis of APD., Results: Seventeen adolescent APD patients were included (mean age at diagnosis: 14.4 ± 2 years, mean interval of 13.6 ± 11.1 months between symptom onset and diagnosis). Twelve patients presented with urticaria, two with fixed drug eruption. Erythema multiforme, eczema, and recurrent aphthous stomatitis were present in one patient each. Exposure to exogenous progestin was present in two patients prior to disease onset. Progesterone skin test was performed in six patients with positive results in two. Fourteen patients received antihistamines and/or a topical corticosteroid. Combined oral contraceptives (COCs) were given to eleven patients, in seven via continuous daily dosing. Gonadotropin-releasing hormone agonist (GnRHa) was used in five, progesterone desensitization in four, omalizumab in two, and danazol in one patient., Conclusions: Adolescent APD is associated with a significant delay in diagnosis. The most common manifestation is urticaria. Exogenous exposure to progestins is uncommon in adolescent APD. Continuous COC, GnRHa, and progesterone desensitization have been used to control symptoms. Large, multicenter studies are required to better define, diagnose, and treat this under recognized condition among adolescent patients., (© 2020 Wiley Periodicals LLC.)

Background/objectives: The aim of this study was to describe a previously unreported association of oral pemphigus vulgaris with short-lived blood-filled painless blisters resembling angina bullosa haemorrhagica (ABH)., Methods: A cross-sectional study of consecutive patients with Pemphigus vulgaris. All patients were examined for the presence of ABH-like lesions, and demographic, clinical and histopathological data were collected. Histopathological examination was performed when feasible., Results: A total of 318 with pemphigus vulgaris were included (63.5% female, mean age: 46 years). ABH-like lesions were present in 82 (25.8%) patients, commonly observed in the buccal mucosa (47, 57.3%) followed by the palate (15, 18.3%). All patients had normal platelet counts with no evidence of bleeding diathesis. Biopsies of the ABH-like lesions showed suprabasal clefts in four of six samples. ABH-like lesions were significantly associated with partial remission of pemphigus vulgaris (47.5%, P = 0.002) and the use of intraoral steroids (P = 0.001, odds ratio: 5.9 [95% confidence interval: 2.5-13.6])., Conclusion: ABH-like lesions may represent a transient or abortive form of oral pemphigus vulgaris and tend to have a benign and self-limiting nature., (© 2018 The Australasian College of Dermatologists.)

Autosomal recessive congenital ichthyosis (ARCI), a phenotypically heterogeneous group of non-syndromic Mendelian disorders of keratinization, is caused by mutations in as many as 13 distinct genes. We examined a cohort of 125 consanguineous families with ARCI for underlying genetic mutations. The patients' DNA was analyzed with a gene-targeted next generation sequencing panel comprising 38 ichthyosis associated genes. The interpretations of results of genomic data were assisted by genome-wide homozygosity mapping and transcriptome sequencing. Sequence data analysis identified biallelic mutations in 106 families out of a total of 125 (85%), most of them (102, 96.2%) being homozygous, reflecting consanguinity in these families. Among the 85 distinct mutations in 10 different genes, 45 (53%) were previously unreported. Phenotype-genotype correlations allowed assignment of specific genes in the majority of the families to a specific subtype of ARCI, lamellar ichthyosis (LI) versus congenital ichthyosiform erythroderma (CIE). Interestingly, mutations in several genes could give rise to an overlapping phenotype consistent with either LI or CIE. Also, this is the third report for SDR9C7 and SULT2B1, and fourth report for CERS3 mutations. Direct comparison of our results with previously published regional cohorts highlights the global mutation landscape of ARCI, however, population specific differences were noted., (© 2018 Wiley Periodicals, Inc.)

Patients with epidermodysplasia verruciformis (EV) and biallelic null mutations of TMC6 (encoding EVER1) or TMC8 (EVER2) are selectively prone to disseminated skin lesions due to keratinocyte-tropic human β-papillomaviruses (β-HPVs), which lack E5 and E8. We describe EV patients homozygous for null mutations of the CIB1 gene encoding calcium- and integrin-binding protein-1 (CIB1). CIB1 is strongly expressed in the skin and cultured keratinocytes of controls but not in those of patients. CIB1 forms a complex with EVER1 and EVER2, and CIB1 proteins are not expressed in EVER1- or EVER2-deficient cells. The known functions of EVER1 and EVER2 in human keratinocytes are not dependent on CIB1, and CIB1 deficiency does not impair keratinocyte adhesion or migration. In keratinocytes, the CIB1 protein interacts with the HPV E5 and E8 proteins encoded by α-HPV16 and γ-HPV4, respectively, suggesting that this protein acts as a restriction factor against HPVs. Collectively, these findings suggest that the disruption of CIB1-EVER1-EVER2-dependent keratinocyte-intrinsic immunity underlies the selective susceptibility to β-HPVs of EV patients., (© 2018 de Jong et al.)

Background: The aim of this study was to explore the experiences of patients who suffer from acute cutaneous leishmaniasis in Iran, focusing on quality of life., Methods: The study was conducted at two different sites in Iran in 2010-2011. Individual in-depth interviews were conducted with six men and six women parasitologically confirmed acute cutaneous leishmaniasis. Interviews were recorded, transcribed verbatim, and translated into English. Qualitative content analysis was used for data analysis., Results: The participants, aged 23 to 63yr, had mild to severe disease. Based on the analysis four main themes were developed. "Fearing an agonizing disease" reflects patients' experiences of disease development resulting in sadness and depression, "struggling to cope" and "taking on the blame" both illustrate how patients experience living with the disease, which included both felt and enacted stigma as major social concerns. "Longing for being seen and heard" refers to patients' experiences with healthcare as well as their expectations and demands from communities and healthcare to be involved in closing the knowledge and awareness gap., Conclusion: Mental and social dimensions of cutaneous leishmaniasis were complex and adversely affected patients' lives by causing psychological burden and limiting their social interactions. Health authorities have to plan programs to increase the disease awareness to prevent the existing stigma to improve patients' social condition and medical care.

There are at least 38 mutant genes known to be associated with the ichthyosis phenotypes, and autosomal recessive congenital ichthyosis (ARCI) is a specific subgroup caused by mutations in 13 different genes. Mutations in some of these genes, such as CERS3 with only two previous reports, are rare. In this study, we identified mutations in candidate genes in consanguineous families with ARCI with a next generation sequencing (NGS) array that incorporates 38 ichthyosis associated genes. We applied this sequencing array to DNA from 140 ichthyosis families with high prevalence of consanguinity. Among these patients we identified six distinct, previously unreported mutations in CERS3 in six Iranian families. These mutations in each family co-segregated with the ichthyosis phenotype. The patients demonstrated collodion membrane at birth, acrogeria, generalized scaling, and hyperlinearity of the palms and soles. The presence of a significant percentage of CERS3 mutations in our cohort depicts a marked difference between the etiology of ichthyoses in genetically poorly characterized regions and well-characterized western populations. Also, it shows that rare alleles are more prevalent in the gene pool of consanguineous populations and emphasizes the importance of these population studies for better understanding of ichthyosis pathogenesis.

We describe a burn patient who developed skin lesions on her skin-graft harvest and skin-graft recipient (burn) sites. Orf virus infection was confirmed by a combination of diagnostic assays, including molecular tests, immunohistochemical analysis, pathologic analysis, and electron microscopy. DNA sequence analysis grouped this orf virus isolate among isolates from India. Although no definitive source of infection was determined from this case, this is the first reported case of orf virus infection in a skin graft harvest. Skin graft recipients with exposures to animals may be at risk for this viral infection., (Published by Oxford University Press for the Infectious Diseases Society of America 2016. This work is written by (a) US Government employee(s) and is in the public domain in the US.)

Background and Objectives: Pemphigus vulgaris (PV) is typically treated with systemic corticosteroids and immunosuppressive agents. Avascular necrosis (AVN) of the femoral head is a well-recognized major complication of corticosteroid therapy. The characteristics of this serious complication in PV remain unknown., Patients and Methods: Uncontrolled, retrospective study of all PV-related AVN cases diagnosed at an Iranian autoimmune bullous disease clinic between 1985 and 2013., Results: Of the 2,321 medical records of PV patients reviewed, 45 (1.93 %) cases showed femoral AVN, with 30 (66.7 %) individuals being male. The mean age at diagnosis of AVN was 47.4 ± 14.2 years. The mean interval between the diagnosis of PV and the onset of AVN was 25.3 ± 18.3 months. With the exception of eight cases (17.8 %), the majority of patients developed AVN within three years after the diagnosis of PV. The mean cumulative dose of prednisolone in patients with AVN was 13,115.8 ± 7041.1 mg. There was a strong correlation between the total prednisolone dose and the time of onset of AVN (p = 0.001). In patients with a history of alendronate intake, that interval was significantly shorter (p = 0.01)., Conclusions: Occurring in about 2 % of patients, AVN is a serious complication of corticosteroid treatment in patients with PV, predominantly in the first three years of treatment. In individuals receiving higher doses of prednisolone, AVN tends to occur earlier., (© 2016 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.)

Hintergrund Und Ziele: Pemphigus vulgaris (PV) wird in der Regel mit systemischen Corticosteroiden und Immunsuppressiva behandelt. Avaskuläre Nekrose (AVN) des Hüftkopfes ist eine gut bekannte schwerere Komplikation einer Corticosteroid-Therapie. Die Charakteristika dieser schweren Komplikation bei PV sind nach wie vor unbekannt., Patienten Und Methoden: Nicht kontrollierte, retrospektive Untersuchung aller PV-bedingten AVN-Fälle, die in einer iranischen Klinik für bullöse Autoimmunerkrankungen zwischen 1985 und 2013 diagnostiziert wurden., Ergebnisse: Anhand der Krankenakten von 2321 untersuchten PV-Patienten wurden 45 Fälle (1,93 %) von femoraler AVN identifiziert. Dreißig davon waren Männer. Das mittlere Alter bei der Diagnose der AVN betrug 47,4 ± 14,2 Jahre. Der mittlere Zeitraum zwischen der Diagnose des PV und dem Einsetzen der AVN lag bei 25,3 ± 18,3 Monaten. Mit Ausnahme von acht Fällen (17,8 %) setzte die AVN bei der Mehrheit der Patienten innerhalb von drei Jahren nach Diagnose des PV ein. Die mittlere kumulative Dosis von Prednisolon bei Patienten mit AVN betrug 13.115,8 ± 7041,1 mg. Zwischen der Prednisolon-Gesamtdosis und dem Zeitraum bis zum Einsetzen der AVN bestand eine starke Korrelation (p = 0,001). Bei Patienten mit Alendronateinnahme in der Vorgeschichte war dieser Zeitraum signifikant kürzer (p = 0,01)., Schlussfolgerungen: Die AVN ist eine schwere Komplikation einer Corticosteroid-Behandlung bei Patienten mit PV. Sie wird bei 2 % der Patienten beobachtet und tritt vor allem in den ersten drei Behandlungsjahren auf. Bei Patienten, die höhere Dosen von Prednisolon erhalten, setzt die AVN tendenziell früher ein., (© 2016 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.)

Ivermectin (IVM) is a broad-spectrum anti-parasitic drug with significant anti-inflammatory properties. The emergence of treatment resistance to lindane, permethrin, and possibly malathion complicates the global strategy for management of common parasitic skin diseases such as scabies and head lice. In this regard. IVM has been safely and effectively used in the treatment of these common human infestations. In addition, IVM may be useful in inflammatory cutaneous disorders such as papulopustular rosacea where demodex may play a role in pathogenesis. Herein, we review the current applications of topical IVM in dermatology.

Background: Allergic contact dermatitis is a common disorder in adults and children alike and appears to be on the increase. The purpose of this study was to determine the sensitization trends in Iranian children with contact dermatitis., Methods: The result of 109 patch tests performed using the 24 allergens of the European Standard Series in patients below 18 years old from September 2007 to March 2009 were recorded and analyzed. The tests were evaluated at 48 and 72 h after performing., Results: The study population consisted of 72 (66.1 %) females and 37 (33.9 %) males. Hands were the most commonly affected anatomic site. In the final evaluation of the tests on day three, 51 (46.8 %) individuals showed a positive reaction to at least one allergen. Females were significantly more likely to show a positive response to at least one allergen (p-value = 0.031, odds ratio: 2.46). The most common allergens were nickel sulfate, cobalt, methylisothiazolinone, and colophony with 21 (19.3 %), 11 (10.1 %), 7 (6.4 %), and 6 (5.5 %) positive reactions, respectively. Contact allergy to nickel sulfate was more common in females than males (23.6 % vs. 10.8 %). There was no statistically significant relationship between personal or family history of atopy and a positive reaction to patch testing. The clinical and practical relevance were assessed for nickel and cobalt with a clinical current relevance in 11 (52.3 %) and 4 (36.4 %), respectively., Conclusions: Nickel sulfate, cobalt, methylisothiazolinone, and colophony are the most common allergens responsible for induction of allergic contact dermatitis in Iranian children and adolescents. Females tended to show more positive reactions to allergens.

Background and Objectives: Erosive oral lichen planus (EOLP) is a chronic inflammatory disease of the oral mucosa causing significant pain and impairment of the quality of life. No immediate and definitive cure is available, especially in its chronic and most recalcitrant forms. The objective of this study was to evaluate the efficacy of methotrexate treatment in EOLP., Patients and Methods: A prospective open trial of oral methotrexate 15 mg QWK in 18 patients with EOLP (clinically and histopathologically confirmed) unresponsive to at least one previous topical or systemic medication., Duration: Twelve weeks followed by twelve weeks of follow-up., Results: Partial response or better was achieved in 15 (83.3%) patients. A statistically significant reduction in Thongprasom scale score was observed (mean value 5 at baseline, 3.08 ± 1.11 at week 12, 2.94 ± 0.97 at week 24, P value < 0.001). A statistically significant reduction was noted in the pain visual analogue scale (mean value 6.55 ± 2.33 at baseline, 2.46 ± 2.18 at week 12, 2.29 ± 1.93 at week 24, P value < 0.001). Adverse events occurred in four (22.2%) patients, requiring discontinuation of treatment in one patient., Conclusion: Methotrexate is a valuable therapeutic option in longstanding recalcitrant EOLP, particularly with respect to the improvement of subjective symptoms. Randomized controlled trials are required., (© 2016 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.)

Background: Nevus sebaceus is a hamartoma composed of abnormal epidermal and dermal components with clinical and histopathological features that change with aging. Prophylactic excision of the lesion was advised historically considering its potential for giving rise to malignant tumors. However, whether early prophylactic excision should be performed has been questioned recently as most of the tumors have been found to be benign in origin., Methods: The histopathologic features of 168 patients with nevus sebaceus were reviewed to determine the frequency of different histologic features as well as associated neoplastic growths., Results: Acanthosis, papillomatosis, and basal layer pigmentation were the most frequent findings. Hyperplastic changes of the sebaceous glands became obvious during the second decade and were present in 60% of the specimens from individuals older than 20. Inflammatory infiltrate was almost invariably present. Skin adnexal changes were frequently noted, including sebaceous gland hyperplasia (93.5%) and primitive hair follicles (76.8%). In 88.5% of scalp lesions, markedly decreased terminal hair was observed. Ectopic apocrine glands were present in 55.4% of specimens, while 24.4% showed anomalous ductal sweat gland structures resembling eccrine hyperplasia. Neoplastic changes were detected in nine cases, including four trichoblastomas, three tricholemmomas, and two syringocystadenoma papilliferums., Conclusion: Development of malignancies in sebaceus nevi is a rare phenomenon, and decision for excision of the lesion should be made after thorough evaluation of the pros and cons., (© 2015 The International Society of Dermatology.)

Background: Phototherapy may alter the morphologic features of melanocytic nevi. Dermoscopy is a non-invasive method for evaluation of skin lesions, specifically melanocytic nevi., Aims and Objectives: This study was designed to evaluate the effects of narrowband ultraviolet B (NB-UVB) and psoralen-ultraviolet A (PUVA) therapy on the dermoscopic features of nevi., Methods: A total of 74 melanocytic nevi were randomly selected from 20 patients. Out of those, 54 nevi received NB-UVB, while 20 received PUVA. 50% of the nevi in each group were exposed to radiation, while the remaining nevi were covered with an opaque tape. All nevi were demoscopically evaluated before and after 30 or 60 sessions of phototherapy., Results: Overall demoscopic changes were observed in 34/37 (91.8%) of the uncovered nevi compared to 16/37 (43.2%) of the covered nevi (P value 0.0001). The most common changes were new dot/globule formation (62.1%), darkening (32.4%), nevus enlargement (27%), and patchy pigmentation (18.8%). Compared to NB-UVB, dermoscopic changes were more frequent in both covered and uncovered nevi of the PUVA group. (P values 0.041 and 0.0172, respectively). New dot/globule formation was observed more frequently in the covered and uncovered nevi of PUVA group., Conclusion: PUVA and NB-UVB induce dermoscopic changes in the majority of the irradiated nevi. However, PUVA is associated with higher frequency of dermoscopic changes in both covered and uncovered nevi.

Paraneoplastic pemphigus (PNP) is an autoimmune blistering disorder that occurs in association with an underlying neoplasm. Inflammatory myofibroblastic tumour (IMT) is a rare low-grade sarcoma of fibroblasts and myofibroblasts associated with inflammatory cells, most commonly occurring in the lung. In this study, a case of PNP associated with IMT of the mediastinum is reported. The patient had a favourable outcome following surgical resection and treatment with a systemic steroid, azathioprine, and i.v. immunoglobulin. The occurrence of PNP with sarcomas, specifically IMT, is noteworthy although it is not well studied in the existing literature., (© 2014 The Australasian College of Dermatologists.)

We herein report two patients with hydroa vacciniforme and concomitant ophthalmic involvement, primarily in the form of keratoconjunctivitis leading to corneal opacity. Awareness of this occasional manifestation may help to prevent severe complications., (© 2014 Wiley Periodicals, Inc.)

Objective: Melanoma is an aggressive tumor with propensity for early metastasis and poor prognosis with delayed diagnosis. Matrix metalloproteinase-2 (MMP-2) is involved in degradation of extra-cellular matrix proteins and tumor progression. Ki-67 is a predictor of tumor proliferation activity but its prognostic significance in melanoma is controversial. In this study, the prognostic value of MMP-2 and Ki-67 via immuno-staining in head and neck melanoma is investigated., Material and Methods: A retrospective analysis of 24 consecutive primary melanoma samples was performed. All samples were stained with monoclonal MMP-2 and Ki-67 antibodies. Two independent investigators evaluated the stained sections. The reactive cells were considered positive regardless of the staining intensity and reported as percentage., Results: A strong correlation between MMP-2 expression, Breslow tumor thickness, and Clark level of invasion was observed (r = 0.85, P < 0.0001 and r = 0.84, P < 0.0001, respectively). A significant positive correlation was observed between MMP-2 expression and number of lymph node involved (r = 0.55, P value 0.005). MMP-2 over expression (>50%) was an independent predictor of lymph node involvement (OR: 2.1, P value: 0.01). No significant association was observed between Ki-67 expression and Breslow tumor thickness, Clark level, or lymph node involvement., Conclusion: MMP-2 expression correlates with tumor thickness in melanoma and is an independent predictive factor for lymph node involvement.

Pemphigus vulgaris (PV) is an autoimmune disorder affecting the skin and mucous membranes. Ocular involvement in PV has been reported but its prevalence and clinical characteristics are not well defined. This prospective cross-sectional study of 103 PV patients was designed to determine the prevalence, clinical types and epidemiological trends of ocular involvement in a population of Iranian patients with PV. Ocular involvement was present in 17 (16.5%) patients. Conjunctivitis was the most prevalent type of ocular involvement (9/17, 52.9%), followed by erosion of the palpebral conjunctiva (7/17, 41.2%). Erosion of the bulbar conjunctiva was noted in only one patient (5.9%). The most commonly reported symptoms were eye irritation (76.5%) and redness (76.5%). No significant relation was found between ocular involvement and disease activity (partial remission or relapse). Mucoid discharge was significantly more common in patients with conjunctival erosions as compared to patients with conjunctivitis (P = 0.038). We conclude that ocular involvement is not rare in PV; 16.5% of PV patients develop ocular disease independent of the disease activity and extension. Conjunctivitis is the most common type of involvement, however, palpebral conjunctival erosion is more frequent than previously realized., (© 2014 Japanese Dermatological Association.)

Importance: Recently, the clinical pemphigus disease activity indexes of Pemphigus Disease Area Index (PDAI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), and Pemphigus Vulgaris Activity Score (PVAS) were validated to correlate with physician global assessment. The antidesmoglein (anti-Dsg) autoantibodies are known to correlate mostly with pemphigus disease activity. The correlation between these indexes and anti-Dsg1 and anti-Dsg3 enzyme-linked immunosorbent assay values has not been previously evaluated., Objectives: To evaluate the PDAI, ABSIS, and PVAS in a large number of patients with pemphigus vulgaris and to compare the interrater reliability of these indexes and the convergent validity according to anti-Dsg values., Design, Setting, and Participants: A cross-sectional study was performed in 2012 in a referral university center for autoimmune bullous diseases. One hundred patients with confirmed diagnoses of pemphigus vulgaris and clinical pemphigus lesions (mean [SD] age, 43.3 [1.7] years; age range, 14-77 years; female-male ratio, 1:3) were studied. Three dermatologists familiar with immunobullous diseases and the indexes rated the patients., Interventions: All 100 patients were evaluated with the PDAI, ABSIS, and PVAS. Three dermatologists independently rated all 3 indexes for each of the patients on the same day. Serum anti-Dsg1 and anti-Dsg3 enzyme-linked immunosorbent assay values were measured simultaneously., Main Outcomes and Measures: Analyses of interrater reliabilities, convergent validities according to anti-Dsg titers, correlation between the distribution and types of lesions with disease activity, predictors of higher titers of antibody (multiple regression analysis), and cutoff values of measures for 2 titers of anti-Dsg with optimal area under the curve, sensitivity, and specificity were performed., Results: The interrater reliabilities were highest for the PDAI, followed by the ABSIS and the PVAS (intraclass correlation coefficients of 0.98 [95% CI, 0.97-0.98], 0.97 [95% CI, 0.96-0.98], and 0.93 [95% CI, 0.90-0.95], respectively). The convergent validity was highest for the PDAI, followed by the PVAS and the ABSIS (Spearman ρ = 0.67, 0.52, and 0.33, respectively). Head, neck, and trunk involvement were predictors of higher titers of anti-Dsg1., Conclusions and Relevance: Among the 3 studied indexes, the PDAI had the highest validity and is recommended for use in multicenter studies for rare diseases, such as pemphigus vulgaris.

Kaposi's sarcoma is a low-grade vascular tumor that its prevalence is increasing all around the world due to the increasing prevalence of HIV and organ transplantations. In this study, we assessed risk factors of Kaposi's sarcoma among Iranian patients were referred to Dermatopathology Department of Razi Hospital of Tehran University of Medical Sciences between the years of 2006 to 2011. Also, disease stages have been assessed on pathologic specimens. Thirty four patients diagnosed with Kaposi's sarcoma entered the study. Of the 34 patients, 29% were female, and 71% were male, mean age was 66 years and average disease duration was 6 months. The most common patient's pathologic stages were reported: patch 32%, patch to plaque 24%, plaque 12%. The most prevalent location of involvement was lower extremity (88%), and upper extremity was involved in 24%. At presentation time, the mean number of lesions was 3 to 4. 15% of patients had a history of smoking and 15% had a history of immunosuppressive drugs. HIV infection did not observe in the patients. Based on the present study, Kaposi's sarcoma is a disease of elderly and is higher in older men. The most common site of Kaposi's sarcoma involvement is lower extremity, and the most common pathologic stage was the patch to plaque.

Pigmented purpuric dermatosis (PPD) is characterized by petechial and pigmented macules on the lower limbs. The aetiology of PPD remains obscure. Some reports have suggested an association between PPD and hepatitis B or C infection. This prospective case-control study was designed to investigate the association of positive hepatitis B or C serology with PPD. A total of 60 PPD patients and 230 randomly selected controls were enrolled. Sera from all patients and controls were tested for liver function tests (LFT), hepatitis B surface antigen (HBS Ag), and hepatitis C virus antibody (HCV Ab). The prevalence of HBS Ag in patients with PPD and the controls was 3 per cent (5/60) and 4.3 per cent (10/230), respectively. The prevalence of HCV Ab was 1.7 per cent (1/60) and 1.3 per cent (3/230) among patients and controls, respectively. No statistically significant difference was noted in the prevalence of positive hepatitis B or C serology (P-values 0.73 and 0.58, respectively). No statistically significant difference in LFT was observed between the two groups. Therefore, the authors believe it is unlikely that HBV or HCV are directly involved in the pathogenesis of PPD., (© 2012 The Authors Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists.)

Adnexal tumors (ATs) are primary skin tumors with benign or rarely, malignant behavior. They have been classified based on differentiation towards hair follicle, sebaceous, apocrine or eccrine gland. Few large-scale studies have focused on ATs. To determine the prevalence of ATs and to assess clinical and histopathological trend of ATs. A retrospective descriptive study of all ATs diagnosed in Razi hospital between 2006 and 2010 was performed. A total of 30,000 pathology records were reviewed, and 1016 ATs were included. The prevalence of ATs was 3.3%. 518 patients (51%) were female, with a mean age of 34.5 years. 953 tumors (93.8%) were benign. ATs were most commonly located in the head and neck area (822, 83.5%). The most common histopathological origin of ATs was sebaceous gland (536, 52.7%). Sebaceous nevus of Jadassohn was the most prevalent single tumor type (40.6% of all ATs). In 63.6% (646) of tumors, ATs were clinically suspected by the clinician prior to biopsy. The most common malignant AT was sebaceous carcinoma (23, 36.5% of all malignant ATs). ATs are infrequent lesions, most commonly occurring in 3rd and 4th decade of life. Diagnosis of ATs is made by histopathological studies as they often express indistinctive clinical features. Malignant ATs are rare, occur at an older age, and are often hard to recognize clinically.

To determine the prevalence of vaccine-associated paralytic poliomyelitis (VAPP) in immunodeficient infants, we reviewed all documented cases caused by immunodeficiency-associated vaccine-derived polioviruses in Iran from 1995 through 2008. Changing to an inactivated polio vaccine vaccination schedule and introduction of screening of neonates for immunodeficiencies could reduce the risk for VAPP infection.