Scoring systems in pemphigus

Pemphigus is a dreaded disease encountered not infrequently in dermatology settings. While scoring systems in various dermatological conditions exist, objective parameters for assessing disease activity and therapeutic responses in pemphigus are not uniform and foolproof. This article presents various scoring systems in pemphigus.     

Assessing the autoantibody levels in relation to disease severity and therapy response in pemphigus patients

Background:

In pemphigus, autoantibodies are directed against adhesion molecules, which make the junctions between keratinocytes, and thus determining their level can reflect the disease activity.

Aim:

The purpose of this study is to determine the clinical significance of the autoantibody levels in pemphigus management.

Materials and Methods:

The clinical features of 47 pemphigus vulgaris patients were assessed and patients′ sera were investigated by indirect immunofluorescence using monkey esophagus as a substrate for autoantibody levels.

Results:

We found a significant correlation between antibody titers and mucosal severity scores. Initial antibody titers of the patients with at least one mucosal lesion at the end of the first month of the therapy were found significantly higher than the patients who had no mucosal lesion. With the therapy, lesions resolved earlier than the antibody titers.

Conclusion:

In patients with pemphigus, especially in cases who were not treated before, sera antibody levels are a valuable tool in evaluating disease severity and choosing initial treatment. In patients who had been taking any systemic treatment, it is difficult to make a relationship between antibody levels and disease severity, because therapy improves disease earlier than the antibody titers. However, estimating antibody levels can be helpful for clinicians in disease management, in reducing or ceasing treatment dosage and anticipating recurrence.     

Uric Acid: a new antioxidant in patients with pemphigus vulgaris

Background:

Increased reactive oxygen species (ROS) and lipid peroxidation are seen in many dermatologic disorders, for example, atopic dermatitis, psoriasis, vitiligo, acne vulgaris, pemphigus vulgaris (PV), lichen planus, and alopecia areata. ROS has an important role in the inflammation process. In PV, increased production of ROS leads to decline of antioxidants in plasma and red blood cells which results in oxidative stress. We aimed to evaluate the level of these antioxidants in PV patients and compare it to the controls.

Materials and Methods:

Among patients attending the dermatology clinics, 30 patients with PV, who had never been on treatment, were enrolled to the study. The control group consisted of 30 age- and sex-matched healthy non-smoker individuals. Venous blood was collected from the subjects for the evaluation of plasma levels of glutathione peroxidase, vitamin C, selenium, bilirubin, and uric acid.

Results:

Age mean and standard deviation of the patients (40.83, 12.74) was comparable to the controls (41.96, 13.08). Mean level of uric acid was significantly lower in PV patients compared to the controls (P = 0.006). Other antioxidants were not different between the two groups. Uric acid of the patients with mucosal involvement was significantly lower than patients with mucocutaneous involvement (P = 0.049).

Limitations:

The blood level of other antioxidants (e.g. malondialdehyde) was not evaluated.

Conclusions:

Uric acid as an antioxidant in our study had similar changes to previous studies in the field of other diseases but selenium, bilirubin, and glutathione peroxidase did not differ between patients and controls.     

Pulse Therapy in Pemphigus: Ready Reckoner

Pulse therapy for the treatment of pemphigus has been in vogue for several years and is administered by many dermatologists across the world. However, even though there is enough evidence about its efficacy and methodology, there continue to be doubts and questions regarding the rationale of use of high dose intravenous steroids and steroid-sparing immunosuppressants. This article has aimed to provide clarity to young dermatology residents on the administration of pulse therapy, and the various controversies and modifications that have been mentioned in literature over the past couple of years.     

Psychiatric morbidity in dermatological outpatients: an issue to be recognized

BACKGROUND: There is a high prevalence of psychiatric disorders in dermatological outpatients. OBJECTIVES: To estimate the magnitude of this problem and to identify a set of variables associated with the presence of psychiatric disorder. METHODS: All adults attending the outpatient clinics of a dermatological hospital on predetermined days were given a questionnaire comprising the Skindex-29 and the 12-item General Health Questionnaire (GHQ-12). RESULTS: In total, 4268 questionnaires were given at admission, and 3125 were returned. Of these, 546 were blank or incomplete, leaving 2579 respondents (response rate 60.4%). Using a stringent cut-off threshold (> or = 5) for psychiatric case identification with the GHQ-12, scored in the conventional way, the overall prevalence of psychiatric morbidity was 25.2% (95% confidence interval 23.6-27.0%). We found a higher prevalence of psychiatric disorders in women and in widows/widowers, controlling for age. Health-related quality of life was a much stronger predictor of psychiatric morbidity than physician-rated clinical severity. High prevalence rates (> 30%) were observed among patients with acne, pruritus, urticaria, alopecia and herpesvirus infections, and in subjects without objective signs of dermatological disease. CONCLUSIONS: Our study has depicted the situation that is actually faced by dermatologists in their everyday practice, where they are in a unique position to recognize psychiatric morbidity and to take appropriate measures. The GHQ-12, being easy for patients to compile and for physicians or nurses to score, may be a practical tool to increase identification of patients with substantial psychological distress or formal psychiatric disorder in order to provide more comprehensive and appropriate intervention.     

Quality of Life and Psychological Morbidity in Vitiligo Patients: A Study in a Teaching Hospital from North-East India

Background:

Vitiligo is an acquired discoloration of skin and mucous membrane of great cosmetic importance affecting 1-4% of the world''s population. It causes disfiguration in all races, more so in dark-skinned people because of strong contrast. Men, women, and children with vitiligo face severe psychological and social disadvantage.

Aim:

To assess the impact of the disease on the quality of life of patients suffering from vitiligo, also to ascertain any psychological morbidity like depression associated with the disease and to compare the results with that of healthy control group.

Materials and Methods:

Dermatology Life Quality Index (DLQI) and Hamilton Depression Rating Scale (HAMD) are administered to 100 vitiligo patients presenting to the Dermatology OPD and 50 age- and sex-matched healthy controls. Results were analyzed and compared with that of control group. Findings are also correlated in relation to demographic and clinical profile of the disease. Statistical analysis is made to see the significance.

Results:

Vitiligo-affected patients had significantly elevated total DLQI scores (P < 0.001) compared to healthy controls. There is increase in parameters like itch, embarrassment, social and leisure activities in the patient cohort than the control group. Patients of vitiligo are also found to be more depressed (P < 0.001) than the controls.

Conclusion:

Quality of life (QOL) in patients affected with vitiligo declined more severely, and also there is increase in incidence of depression than in the control group. These changes are critical for the psychosocial life of the affected people.     

AVASCULAR NECROSIS: A RARE COMPLICATION OF STEROID THERAPY FOR PEMPHIGUS

A patient of pemphigus vulgaris presented with avascular necrosis of the femur after long-term high-dose corticosteroid therapy. Corticosteroids used on a long-term basis can cause avascular necrosis of bone and this has been seen in various diseases. This is attributable to both the disease process itself and the therapy i.e. corticosteroid usage. In dermatological practice avascular necrosis of bone has been seen more commonly with SLE and also with psoriasis using long-term steroids. Avascular necrosis in a case of pemphigus on steroid therapy is a rare finding. We report such a case of pemphigus vulgaris developing avascular necrosis of bone following corticosteroid therapy.     

Pemphigus Foliaceus Masquerading as IgA Pemphigus and Responding to Dapsone

A 14-year-old male presented with seven years history of recurrent episodes of fluid filled, itchy and eroded lesions over the body not responding to oral corticosteroids and azathioprine. Dermatological examination revealed crusted plaques and erosions in a seborrheic distribution. Histopathology of skin lesions and direct immunofluorescence were characteristic of pemphigus foliaceus. He was treated with dexamethasone pulse therapy with inadequate response. However, relapsing skin lesions revealed a circinate arrangement with a predilection to trunk and flexures. In view of clinical features suggestive of IgA pemphigus, he was started on dapsone, to which he responded dramatically in four weeks. However, repeat biopsy continued to reveal features of pemphigus foliaceus and ELISA for anti-desmoglein 1 antibodies was positive.     

Hailey-Hailey Disease Responding to Thalidomide

Familial benign chronic pemphigus or Hailey-Hailey disease (HHD) is a rare autosomal dominant disorder characterized by the development of recurrent blisters and erosions in the intertriginous areas. Various topical and systemic treatment options include corticosteroids, topical 5-fluorouracil, topical vitamin D analogs, topical zinc oxide, dapsone, psoralen plus ultraviolet A, systemic retinoids, cyclosporine, methotrexate, and photodynamic therapy. In recalcitrant cases, further options including, invasive methods such as grenz ray therapy, carbon dioxide laser abrasion, and erbium: YAG laser ablation, dermabrasion, electron beam therapy, botulinum toxin, and full-thickness excision of affected skin with repair by split-thickness grafting have been reported as useful in treatment of HHD. We describe a case of HHD who was treated with several treatment modalities including antibiotics, corticosteroids, and dapsone earlier and when presented to us had a severe recalcitrant disease. Thalidomide, as a modality of treatment has been successfully used in few cases earlier. Our patient responded well to thalidomide.     

Allogenic hematopoietic stem cell transplantation in pemphigus vulgaris: a single-center experience

Background:

Pemphigus vulgaris (PV), an autoimmune disorder characterized by blistering skin/mucus membrane lesions, is mediated by desmoglein-3 autoantibodies. We carried out a prospective clinical trial of hematopoietic stem cell transplantation (HSCT) in thymus, bone marrow (BM) and periphery to reconstitute central and peripheral arms of self-tolerance.

Materials and Methods:

Eleven (M:F=5:6) patients with mean age 33.5 years and mean duration of disease 22.8 months, having painful pruritic blisters and ulcers resistant to corticosteroids, were treated with cytokine-stimulated allogeneic HSCT (mean dose: 21.8 × 10⁸ cells/kg BW) from blood group-matched related donors. BM with mean CD34+ count 1.1% was inoculated into thymus, marrow and periphery, followed by two peripheral blood stem cell (PBSC) infusions.

Results:

Recovery began within 24 hours of HSCT and new lesions stopped after 6 months. No graft versus host disease (GvHD)/adverse effect was observed in any patient/donor. Over a mean follow-up of 8.02 years, all patients were well without recurrence/new lesions.

Conclusion:

Drug-resistant PV can be successfully and safely treated by allogeneic HSCT.     

A Comparative Effectiveness Research of Azathioprine and Cyclophosphamide on the Clinical and Serological Response in Pemphigus Vulgaris

Context:A prospective study was carried out to examine the efficacy of cyclophosphamide and azathioprine in pemphigus vulgaris.Aims:To compare the clinical and serological effect of azathioprine and cyclophosphamide in pemphigus patients.Results:Azathioprine had a slower onset of action with a statistically significant improvement seen by 6 months (P = 0.016). Cyclophosphamide had a faster onset of action (3 months) though there was no statistical difference in the efficacy between the two at the end of 6 months. The (RonT) was 33.3–44.4% for azathioprine and 28.8–42.9% for cyclophosphamide at 6 months. Though ELISA had a high sensitivity and specificity for diagnosis, as a tool for assessing therapeutic response a significant decrease was seen only till 3 months. This was restricted to Dsg1 for the azathioprine group and both Dsg3 and Dsg1 levels for the cyclophosphamide group. There were two deaths, both in the cyclophosphamide group.Conclusions:Azathiorpine and cyclophosphamide are equally effective for mucosal and cutaneous disease in pemphigus after 6 months of therapy. Dsg ELISA is useful for diagnosis of pemphigus but is not a useful tool for monitoring response to therapy.     

BLISTER FLUID IMMUNOFLUORESCENCE IN A CASE OF PEMPHIGUS VULGARIS

Indirect immunofluorescence with serum is used in the diagnosis of pemphigus. We report a case in whom blister fluid was used as the specimen for indirect immunofluorecscence.     

Seborrheic keratoses in five elderly patients: an appearance of raindrops and streams

Five Chinese patients were found to have a linear, splayed, vertical pattern of lesions on their back, chest and abdomen. These lesions were clinically and pathologically diagnosed as seborrheic keratosis. The mean age of our patients was 77.6 years. During the follow-up period of 12-20 years, the lesions appeared to increase with age, and no malignant changes were observed on these sites. These patients had no serious underlying medical problems or malignant neoplasm, except for one patient with duodenal ulcer. While we are uncertain as to the cause of this patterning of seborrheic keratoses, we believe that it is distinct from previously reported patterns; this will contribute to research on the pathogenesis of seborrheic keratosis.     

Atypical Cutaneous Sporotrichosis in an Immunocompetent Adult: Response to Potassium Iodide

Cutaneous sporotrichosis, also known as “Rose Gardener''s disease,” caused by dimorphic fungus Sporothrix schenkii, is usually characterized by indolent nodular or nodulo-ulcerative lesions arranged in a linear pattern. We report bizarre nonlinear presentation of Sporotrichosis, in an immunocompetent adult occurring after a visit to Amazon rain forest, speculating infection with more virulent species of Sporothrix. The diagnosis was reached with the help of periodic acid-Schiff positive yeast cells and cigar shaped bodies seen in skin biopsy along with the therapeutic response to potassium iodide.     

Psoriasis in children: an insight

Onset of psoriasis in childhood is quite common. Chronicity, inflammation and hyperproliferation are the cardinal features by which the condition establishes its uniqueness. Clearance of disease may be farfetched in most patients and relapse is frequent. Early recognition and management of psoriasis in children and adolescents is vital in therapy in children.     

A clinical and histopathological profile of patients with cutaneous tuberculosis

Extrapulmonary tuberculosis constitutes about 10% of all cases of tuberculosis, and cutaneous tuberculosis makes up only a small proportion of these cases. Despite prevention programs, tuberculosis is still progressing endemically in developing countries. Commonest clinical variant of cutaneous tuberculosis in our study was lupus vulgaris seen in 55% patients followed by scrufuloderma seen in 25% patients followed by orificial tuberculosis, tuberculosis verrucosa cutis, papulonecrotic tuberculid, and erythema induratum seen in 5% each. The commonest site of involvement was limbs seen in 50% patients followed by neck seen in 25% patients, face in 15%, and trunk in 10% patients. Maximum percentage of patients (55%) had duration of cutaneous tuberculosis between 6-12 months followed by 35% between 13-24 months, 5% had duration of cutaneous tuberculosis less than 6 months, and the rest 5% had duration more than 24 months. The commonest histopathological feature in our study was tuberculoid granuloma with epitheloid cell and Langhans giant cells seen in 70% patients, hyperkeratosis was seen in 15% patients and AFB bacilli were seen in 5% patients.     

Clinical evaluation of patients patch tested with plant series: a prospective study

Background:

Plants are ubiquitous in nature and are essential for human life, though some do have detrimental effects. In India, there are various indigenous and foreign plants that are well known to cause dermatitis, Parthenium hysterophorus being the foremost.

Aims of the Study:

To study the clinical features of patients presenting with plant dermatitis and evaluate plant allergen series patch test results.

Materials and Methods:

50 patients with suspected plant dermatitis were evaluated regarding evolution of dermatitis, precipitating factors and contact with various plants. 31 patients (62%) complained of photo sensitivity and 24 (48%) had involvement of sun-exposed areas. Our provisional diagnoses included allergic contact dermatitis in 16, air borne contact dermatitis in 9, phytophotodermatitis in 7, photodermatitis in 6, airborne contact dermatitis to other causes in 4, phytodermatitis in 3. Patients were patch tested with Indian standard series of allergens as well as plant allergen series consisting of common allergenic plants of local flora in and around Manipal and also with plants strongly suspected by the patient. 35 tested positive with maximum positives (26 patients, i.e., 52%) to Parthenium hysterophorus (Compositae).

Results:

The result of patch testing was correlated with the history and clinical examination and relevance established.

Conclusions:

Majority of the patients with clinically suspected plant dermatitis belonged to 3^rd or 4^th decade and Parthenium was the leading plant allergen Sensitization to Leguminosae plants and Hopea ponga, seen in 9 cases, have not been reported earlier in literature.     

Pilot study of sexual dysfunction in patients with psoriasis: influence of biologic therapy

Background:

Psoriasis is a chronic skin disease that affects 1 to 3% of the population in most industrialized countries. It is commonly associated with a variety of psychological problems including low self-esteem, depression, suicidal thoughts, and sexual dysfunction.

Materials and Methods:

We have performed a pilot study in which we have tried to assess the impact on sexual dysfunction in patients with psoriasis who have started treatment with biological therapy using validated indexes in Spanish: International Index of Erectile Function for men and female sexual function index in women.

Results:

Considering the men and women from our study, an improvement in FSFI by an average of 9.5 and 6.3 points is observed, respectively.

Conclusion:

We considered our series as a first step for a more detailed approach to the study of sexual function in patients with psoriasis.     

Zosteriform skin metastases: clue to an undiagnosed breast cancer

Cancer metastases represent the most devastating aspect of malignancy, since the mortality of cancer patients is mainly related to the metastatic behavior of the primary neoplasm. Skin metastases are usually late events in the course of tumor progression. Excluding melanoma, the most common tumor to metastasize to the skin is breast cancer. Patients who develop cutaneous metastases rarely present with a zosteriform distribution. Herein, we present a 60-year-old female, an undiagnosed case of breast cancer, with zosteriform metastases along her right T2-T3 dermatome.     

Skin tags and acanthosis nigricans in patients with hepatitis C infection in relation to insulin resistance and insulin like growth factor-1 levels

Background:

Skin tags (ST) are papillomas commonly found in the neck, axillae of middle-aged and elderly people

Aim:

Insulin and insulin-like growth factor (IGF-1) levels are affected by hepatitis C virus (HCV) infection and both of them may be implicated in the etiopathogenesis of ST and acanthosis nigricans (AN) through their proliferative and differentiating properties. So, the aim of this work was to evaluate the impact of HCV infection on ST and AN through the estimation of insulin resistance and IGF-1.

Materials and Methods:

Participants were arranged into four groups: (ST +ve / HCV +ve) 23 subjects, (ST+ / HCV -ve) 19 subjects, (HCV -ve / ST-ve) 20 subjects and (ST-ve /HCV +ve) 22 subjects. Age, ST size, color, number, AN, fasting glucose, fasting insulin, insulin resistance, IGF-1, HCV-antibodies (Ab) were recorded.

Results:

The mean number of ST in Group 1 was half the number of ST in Group 2 (11.0±9.3 / 22.3±14.0) (P=0.005). The difference in insulin resistance between the same groups was non-significant (13.1±10.6 / 9.0±5.5) (P=0.441) while the difference in IGF-1 was statistically significant (218.6±46.2 /285.4±32.8) (P=0.002). The multivariate logistic regression for the variables revealed that insulin resistance is the only factor affecting the occurrence of ST (OR=1.096, P=0.023). Multivariate regression analysis for the variables showed that HCV was borderline but not a significant factor affecting the number of ST (Beta=-0.409, P=0.053). The number of patients with AN was doubled in Group 2 in comparison to Group 1 but this was non significant 3(13%) / 6(32%) (P=0.2800).

Conclusion:

HCV is associated with a significant decrease in the ST number and in the serum level of IGF-1 together with an obvious decrease in the occurrence of AN. Our results may point to the entrant effect of insulin resistance and IGF-1 in ST and AN development. The current study suggests the evaluation of IGF-1-lowering agents in the control of ST and AN especially in the females with polycystic ovary and in the prevention of the recurrence of ST after surgical removal.