Bullous pemphigoid in India: Review of cases registered in an autoimmune bullous disease clinic.

Background Information on bullous pemphigoid in an Indian context is scarce. Aim To report clinico-demographic profile, associated comorbidities and prescription pattern of bullous pemphigoid patients in India. Methods This was a retrospective study, where past records of all bullous pemphigoid patients diagnosed and treated between November 2013 and October 2019 were accessed and analysed. Patients having a compatible clinical presentation with either histopathological and/or direct immunofluorescence evidence of bullous pemphigoid were included. Results There were 96 bullous pemphigoid patients, with a male: female ratio of 1.6:1. The mean age at diagnosis was 62.5 ± 2.2 years, with mean duration of illness 27.5 ± 4.5 months before presentation. Comorbidities were present in 80 (83%) patients, with type 2 diabetes mellitus (38.5%), hypertension (36.4%) and neurological illness (16.7%) being the commonest ones. Clinically, blisters were the predominant presentation in 81 (84.4%) patients. The majority (87.5%) of patients showed a predominant eosinophilic infiltrate on histopathology. Direct immunofluorescence revealed immunoglobulin G deposits with complement C3 in 77 (80.2%) cases. The majority of patients (77.1%) were treated with oral prednisolone, either alone (11.5%) or in combination (65.6%) with other topical and systemic agents. Topical steroids were used in 29.1%, azathioprine in 28%, dapsone in 16.7% and omalizumab in 6.2% of patients. Limitations The study is retrospective. Immunofluorescence on salt split skin, direct immunofluorescence serration pattern analysis, and immunoblotting were not performed. Hence, there is a possibility that a few included cases were suffering from other subepidermal autoimmune bullous diseases like epidermolysis bullosa acquisita or anti-p200 pemphigoid. Conclusion Bullous pemphigoid patients in this study had a younger age of onset and showed male preponderance. Comorbidities like type 2 diabetes, hypertension and neurological disorders were frequent. Cutaneous blisters were the most frequent clinical presentation. Systemic corticosteroids comprised the mainstay of therapy.

Nail changes in autoimmune blistering disorders: A case-control study.

BACKGROUND: Pemphigus and pemphigoid disorders produce blistering cutaneous lesions. Earlier case reports state that nail involvement is uncommon in these autoimmune blistering disorders. AIMS AND OBJECTIVES: To study nail changes in autoimmune blistering disorders. METHODS: A case-control study was conducted where 40 cases and 40 controls were evaluated for nail changes. RESULTS: Nail changes were seen in 72.5% of cases and 17.5% of controls. The most common nail findings were paronychia and onychorrhexis. LIMITATIONS: Small sample size; short study duration; nail biopsy could not be done. CONCLUSION: Our findings indicate that the inflammatory nature of the blistering cutaneous disease is often reflected conspicuously in the nails.

Chinese version of the treatment of autoimmune bullous disease quality of life questionnaire: Reliability and validity.

BACKGROUND: Treatments for autoimmune blistering disease carry significant risks of medical complications and can affect the patient's quality of life. Recently, the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire was developed in Australia. OBJECTIVE: The objective of this study was to evaluate the reliability and validity of the Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire in Chinese patients with autoimmune blistering diseases. METHODS: The Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire was produced by forward-backward translation and cross-cultural adaptation of the original English version. Autoimmune blistering disease patients recruited in the study self-administered the Chinese Treatment of Autoimmune Bullous Disease Quality of Life questionnaire, the Dermatology Life Quality Index and the 36-item Short-Form Health Survey. Reliability of the Chinese Treatment of Autoimmune Bullous Disease Quality of Life was evaluated using internal consistency and test-retest (days 0 and 7) methods. Validity was analyzed by face, content, construct, convergent and discriminant validity measures. RESULTS: A total of 86 autoimmune blistering disease patients were recruited for the study. Cronbach's alpha coefficient was 0.883 and the intraclass correlation coefficient was 0.871. Face and content validities were satisfactory. Convergent validity testing revealed correlation coefficients of 0.664 for the Treatment of Autoimmune Bullous Disease Quality of Life and Dermatology Life Quality Index and -0.577 for the Treatment of Autoimmune Bullous Disease Quality of Life and 36-item Short-Form Health Survey. With respect to discriminant validity, no significant differences were observed in the Treatment of Autoimmune Bullous Disease Quality of Life scores of men and women (t = 0.251, P = 0.802), inpatients and outpatients (t = 0.447, P = 0.656), patients on steroids and steroid-sparing medications (t = 0.672, P = 0.503) and patients with different autoimmune blistering disease subtypes (F = 0.030, P = 0.971). LIMITATIONS: Illiterate patients were excluded from the study. The patients were from a single hospital and most of their conditions were in a relatively stable status. CONCLUSION: The Chinese version of the Treatment of Autoimmune Bullous Disease Quality of Life questionnaire is a reliable and valid instrument to measure treatment burden and to serve as an end point in clinical trials in Chinese autoimmune blistering disease patients.

Clinical, demographic and immunopathological spectrum of subepidermal autoimmune bullous diseases at a tertiary center: A 1-year audit.

BACKGROUND: The subepidermal autoimmune bullous diseases are a subset of immunobullous diseases encountered less frequently in the Indian population. There is a paucity of data on the prevalence, demographic and clinicopathological spectrum of various subepidermal autoimmune bullous diseases from India. AIM: To determine the demographic and clinicopathological profile of subepidermal autoimmune bullous diseases in Indian patients, presenting to the Immunobullous Disease Clinic of Postgraduate Institute of Medical Education and Research, Chandigarh. METHODS: Patients seen from November 2013 to November 2014 who fulfilled the preset diagnostic criteria of subepidermal autoimmune bullous diseases were identified from case records. Data regarding demographic characteristics, clinical profile, immunopathological findings and treatment were collected from the predesigned proforma. RESULTS: Of 268 cases of autoimmune bullous diseases registered, 50 (18.7%) were subepidermal autoimmune bullous diseases. Bullous pemphigoid was most frequently seen in 20 (40%) cases, followed by dermatitis herpetiformis in 14 (28%), mucous membrane pemphigoid in 6 (12%), chronic bullous dermatosis of childhood / linear immunoglobulin A bullous dermatosis in 5 (10%), lichen planus pemphigoides in 3 (6%), pemphigoid gestationis and epidermolysis bullosa acquisita in 1 (2%) case each. None of the patients had bullous systemic lupus erythematosus. LIMITATIONS: We could not perform direct and indirect immunofluorescence using salt-split skin as a substrate and immunoblotting due to non-availability of these facilities. Therefore, misclassification of subepidermal autoimmune bullous diseases in some cases cannot be confidently excluded. CONCLUSION: Subepidermal autoimmune bullous diseases are not uncommon in Indian patients. Bullous pemphigoid contributes maximally to the burden of subepidermal autoimmune bullous diseases in India, similar to that in the West, although the proportion is lower and disease onset is earlier. Dermatitis herpetiformis was observed to have a higher prevalence in our population, compared to that in the West and the Far East countries. The prevalence of other subepidermal autoimmune bullous diseases is relatively low. Detailed immunofluorescence and immunoblotting studies on larger patient numbers would help better characterize the pattern of subepidermal autoimmune bullous diseases and their features in Indian patients.

Cutaneous reactions simulating erythema multiforme and Stevens Johnson syndrome due to occupational exposure to a plant-growth regulator.

BACKGROUND: In India, hydrogen cyanamide (Dormex) is a plant growth regulator used mainly for the bud-breaking of grapevines. The use of this chemical may result in severe cutaneous reactions simulating erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). METHODS: Studies were conducted on four seasonal grapevine workers who developed severe cutaneous reactions following the unprotected use of Dormex (hydrogen cyanamide). RESULTS: Two of the patients had EM-like skin lesions and the other two developed SJS-TEN-like skin lesions. A latent period of 5-7 days existed between the contact with the chemical and the development of the skin lesions. The histopathological picture was suggestive of EM. All the patients responded to systemic steroids and antihistamines. CONCLUSIONS: Hydrogen cyanamide may act as a hapten, initiating cytotoxic immunological attack on keratinocytes, resulting in EM- and SJS-TEN-like clinical picture. Awareness regarding such severe cutaneous reactions due to the inappropriate handling of Dormex is required. The use of personal protection equipment while handling agricultural chemicals is essential.

Manifestaciones cutáneas de la sarcoidosis / No disponible

Aunque las manifestaciones cutáneas de la sarcoidosis, las agudas, pueden presentar remisiones, las crónicas pueden ser persistentes, y ser motivo importante de consulta. En nuestra serie de 110 pacientes, 25 presentaron afección cutánea (22,52%), de estos 20 tenían lesiones dérmicas específicas (18,2%) y 5 (4,5%) lesiones inespecíficas. Las lesiones específicas que predominaron fueron las erupciones maculopapulares y las placas y todas presentaron relaciones con estadios radiográficos de esta entidad, fundamentalmente en el estadio I y II. La presencia de afectación clínica fue más representativa en forma de erupciones maculopapulares y en placas, al igual que la evolución crónica. El estudio histopatológico, que según el órgano afectado ayudará al diagnóstico, y la piel por su accesibilidad y su especificidad con la demostración de granulomas sarcoideos, orienta a su confirmación y evita otros exámenes más agresivos y más costosos

Although acute cutaneous manifestations of sarcoidosis may present recurrences, those chronic may persist and be important causes of patient remission to health clinics and dermatologists. In our serie of 110 patients, 25 presented cutaneous affection (22,52 %), 20 out of them had specific skin lesions (18,2%) and 5 (4,5%) non specific lesions. The specific lesions that prevailed were maculopapular eruptions and plates, and all had relationships with radiographic studies of this entity, mainly in I and II stages. The persistence of affections were more representative in maculopapular eruptions and disease, biopsy with the histopathology study which depends of the affected organ, will help to the diagnosis; and the skin due to its accessibility and specificity with the demonstration in sarcoid granulomas guides to its confirmation and prevent more aggressive and expensive exams

Generalized anetoderma in a patient with HIV and dual mycobacterial infection.

A middle-aged HIV infected man receiving treatment for pulmonary tuberculosis, presented with a febrile illness along with evanescent, erythematous nodular lesions all over the body. On examination, he had features suggestive of lepromatous leprosy with lesions of erythema nodosum leprosum. In addition, there were multiple small, circumscribed areas of slack skin, clinically and histopathologically suggestive of anetoderma. Both leprosy and HIV infection are known to give rise to lesions of anetoderma. Pathogenesis of anetoderma in these infectious conditions is discussed.

El citodiagnóstico en dermatología: alcances y limitaciones / Cytodiagnosis in dermatology: its' reaches and limitations

El citodiagnóstico, ampliamente difundido, virtualmente carece de aplicación práctica en dermatología. En parte, por las características inherentes al método y además por la particular histoarquitectura de la piel y membranas mucosas sanas y enfermas. Sin embargo, su técnica sencilla puede constituirse en un auxiliar valioso en ciertas dermatosis, en particular vesicoampollares y tumorales. Se revisa éste método diagnóstico y sus limitaciones y se rescatan aquellos casos en los que puede ofrecer utilidad

El citodiagnóstico en dermatología: alcances y limitaciones / Cytodiagnosis in dermatology: its reaches and limitations

El citodiagnóstico, ampliamente difundido, virtualmente carece de aplicación práctica en dermatología. En parte, por las características inherentes al método y además por la particular histoarquitectura de la piel y membranas mucosas sanas y enfermas. Sin embargo, su técnica sencilla puede constituirse en un auxiliar valioso en ciertas dermatosis, en particular vesicoampollares y tumorales. Se revisa éste método diagnóstico y sus limitaciones y se rescatan aquellos casos en los que puede ofrecer utilidad (AU)