Metastatic Vulvar Crohn's Disease—A Rare Case Report and Short Review of Literature

Metastatic Crohn''s disease (CD), a type of extraintestinal CD may present with gynecological manifestation which causes diagnostic dilemma and needs multidisciplinary approach. Vulvar lesions occur in very small number of cases with CD of which asymmetrical labial swelling and edema is the most common presentation. We report a case of hypertrophic exophytic variety of vulvar CD because of its rarity.     

Acquired Zinc Deficiency in an Adult Female

Acrodermatitis enteropathica is an autosomal recessive inherited disorder of zinc absorption. Acquired cases are reported occasionally in patients with eating disorders or Crohn''s disease. We report a 24-year-old housewife with acquired isolated severe zinc deficiency with no other comorbidities to highlight the rare occurrence of isolated nutritional zinc deficiency in an otherwise normal patient.     

Darier's Disease in Gastric Malignancy: An Unusual Paraneoplastic Phenomenon

Darier''s disease is an autosomal dominant genodermatosis resulting from ATP2A2 gene mutation. A 62-year-old male presented at our outpatient (OPD) with sudden-onset numerous dirty, warty papules over the head, neck, and back since 2 months. Histopathology of the skin lesions revealed acantholytic dyskeratosis suggestive of Darier''s disease. He was referred to the gastroenterology department for some gastrointestinal (GI) symptoms where he was diagnosed with adenocarcinoma of the stomach and was subsequently operated. On his next visit to our department for follow-up, we found a marked diminution of the skin lesions in the absence of any specific treatment. In view of the above finding, we concluded that paraneoplastic dermatosis in the form of Darier''s disease occurred in this patient. Paraneoplastic Darier''s disease with gastric adenocarcinoma is not yet described in dermatology literature and is hence reported here.     

BIOLOGICS: TARGET-SPECIFIC TREATMENT OF SYSTEMIC AND CUTANEOUS AUTOIMMUNE DISEASES

Biologics are becoming important in the treatment of systemic and cutaneous autoimmune diseases. They are designed to target specific components of immune system. As the new drugs are capable of targeting proteins in a more specific fashion, yet have lower risks of systemic side-effects, they have considerable advantages over the older immunomodulators. The development of TNF-alpha blockers in the treatment of psoriasis, psoriatic arthritis, rheumatoid arthritis, Crohn''s disease and ankylosing spondylitis have been major breakthroughs. Likewise, B-cell depletion has proved to be equally revolutionary for the treatment of lupus, pemphigus, certain vasculitides etc. But all said and done, the development of these molecules and their clinical usage are still at evolving stages. Consensus needs be formed to further categorize the clinical profiles of the patients in whom biologics are to be used in the future, given that the long-term safety profiles of these agents are very much unknown at present.     

RARE PRESENTATION OF KYRLE'S DISEASE IN SIBLINGS

Background:

Kyrle''s disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infective agents as well as exposure to chemicals. We present a rare case of this genodermatosis in two siblings.

Materials and Methods:

Two siblings of a non-consanguineous marriage came with generalized discrete papular lesions with a central keratotic plug. All biochemical and serological investigations were within normal limits. Serial sections of the biopsy revealed typical epidermal invaginations filled with parakeratotic debris and perforation into the dermis with accompanying granulomatous reaction.

Results and Conclusions:

A careful history, detailed routine investigations and serial sections of the skin biopsy are required to demonstrate the typical morphology and stages of evolution of Kyrle''s disease. This helps to differentiate the rare primary Kyrle''s disease from other primary and secondary keratotic lesions. Due to the familial occurrence, screening of relatives of an index case is recommended.     

A STUDY COMPARING CHEMICAL PEELING USING MODIFIED JESSNER'S SOLUTION AND 15%TRICHLOROACETIC ACID VERSUS 15% TRICHLOROACETIC ACID IN THE TREATMENT OF MELASMA

Background:

Melasma is a symmetric progressive hyperpigmentation of the facial skin that occurs in all races but has a predilection for darker skin phenotypes. Depigmenting agents, laser and chemical peeling as classic Jessner''s solution, modified Jessner''s solution and trichloroacetic acid have been used alone and in combination in the treatment of melasma.

Objectives:

The aim of the study was to compare the therapeutic effect of combined 15% Trichloroacetic acid (TCA) and modified Jessner''s solution with 15% TCA on melasma.

Materials and Methods:

Twenty married females with melasma (epidermal type), with a mean age of 38.25 years, were included in this study. All were of skin type III or IV. Fifteen percent TCA was applied to the whole face, with the exception of the left malar area to which combined TCA 15% and modified Jessner''s solution was applied.

Results:

Our results revealed statistically highly significant difference between MASI Score (Melasma Area and Severity Index) between the right malar area and the left malar area.

Conclusion:

Modified Jessner''s solution proved to be useful as an adjuvant treatment with TCA in the treatment of melasma, improving the results and minimizing postinflammatory hyperpigmentation.     

Pigmentary nevi on face have unique patterns and implications: The concept of Blaschko's lines for pigmentary nevi

Background:

Nevi are proposed to reflect the mosaicism and thus generally follow the different archetypal patterns of mosaicism. Blaschko''s lines are the most common pattern of mosaicism. There have been many attempts to elucidate the Blaschko''s lines on face from the distributional patterns of different nevi, but studies that evaluated exclusively pigmentary nevi are sparse. Aims: This study attempted to evaluate the patterns followed by different pigmentary nevi on face and utilized this to elucidate the pattern of embryological pigmentation on face.

Materials and Methods:

Spatial parameters like shape, orientation, and distribution patterns of different flat pigmentary nevi on face were analyzed and graphically drawn on human facial diagram. This was compared with existing facial Blaschko''s lines. All cases of palpable pigmented nevi like congenital and giant melanocytic nevi and nevus spilus were excluded.

Results:

A total of 68 cases of pigmentary nevi on face (male-39, female-29) were examined. The shape and distribution lines were found to have a close similarity with Blaschko''s lines on face with distinct differences.

Conclusions:

The concept of facial embryonic pigmentary ‘segment’, ‘unit’ and existence of separate Blaschko''s lines for facial pigmentary nevi is conceived. Some insight into the pathogenesis of Blaschko''s lines is also proposed in this study.     

Disseminated Kaposi's Sarcoma with the Involvement of Penis in the Setting of HIV Infection

Kaposi''s sarcoma (KS) is a malignant proliferation of the endothelial cells. It typically presents with several vascular nodules on the skin and other organs. The penile localization of KS, particularly on the shaft area, is exceptional. We report an HIV-positive 34-year-old man who had multiple purplish-black plaques on his extremities and several small violaceous macules on the glans and shaft of the penis. Kaposi''s sarcoma was diagnosed by histopathology.     

Biostatistics Series Module 4: Comparing Groups – Categorical Variables

Categorical variables are commonly represented as counts or frequencies. For analysis, such data are conveniently arranged in contingency tables. Conventionally, such tables are designated as r × c tables, with r denoting number of rows and c denoting number of columns. The Chi-square (χ²) probability distribution is particularly useful in analyzing categorical variables. A number of tests yield test statistics that fit, at least approximately, a χ² distribution and hence are referred to as χ² tests. Examples include Pearson''s χ² test (or simply the χ² test), McNemar''s χ² test, Mantel–Haenszel χ² test and others. The Pearson''s χ² test is the most commonly used test for assessing difference in distribution of a categorical variable between two or more independent groups. If the groups are ordered in some manner, the χ² test for trend should be used. The Fisher''s exact probability test is a test of the independence between two dichotomous categorical variables. It provides a better alternative to the χ² statistic to assess the difference between two independent proportions when numbers are small, but cannot be applied to a contingency table larger than a two-dimensional one. The McNemar''s χ² test assesses the difference between paired proportions. It is used when the frequencies in a 2 × 2 table represent paired samples or observations. The Cochran''s Q test is a generalization of the McNemar''s test that compares more than two related proportions. The P value from the χ² test or its counterparts does not indicate the strength of the difference or association between the categorical variables involved. This information can be obtained from the relative risk or the odds ratio statistic which is measures of dichotomous association obtained from 2 × 2 tables.     

Vaccines in Dermatology

A vaccine is a biological preparation that improves immunity to a specific disease. More than two centuries have passed since the first successful vaccine for smallpox was developed. We’ve come a long way since. Today''s vaccines are among the 21^st century''s most successful and cost-effective public health tools for preventing diseases.     

DEXAMETHASONE PULSE THERAPY IN PATIENTS OF SYSTEMIC SCLEROSIS: IS IT A VIABLE PROPOSITION? A STUDY FROM KASHMIR

Background:

Systemic sclerosis is a multisystemic autoimmune disorder. Intravenous dexamethasone pulse therapy has been used since 1998.

Aim:

The aim wasto report the beneficial effects of dexamethasone pulse in patients of systemic sclerosis vis-à-vis the side effects.

Materials and Methods:

Forty-seven patients of systemic sclerosis were included. After looking at the history and physical examination, the patients were submitted to various relevant investigations. Clinical scoring of the patient was done at baseline and 6-month interval according to Furst''s organ indices score.

Results:

A total of47 patients of systemic sclerosis were included (45 females, 2 males). In majority, acrosclerosis was seen. Severe sclerosis and contractures were seen in two patients. Moderate proteinuria, restrictive lung disease, dysphagia, and valvular heart involvement were seen.A total of13 patients on dexamethasone pulse therapy developed tuberculosis. Improvement in skin scoring and decreased severity of Raynaud''s phenomenon was seen. No improvement in dysphagia, severe vascular symptoms, or restrictive lung disease was seen.

Conclusion:

Thus, beneficial effects of dexamethasone pulse therapy seem to be merely cosmetic.     

Nodular Erythema Elevatum Diutinum Mimicking Kaposi's Sarcoma in a Human Immunodeficiency Virus Infected Patient

Erythema elevatum diutinum (EED) has been emerging as a specific Human Immunodeficiency Virus (HIV) associated dermatosis in recent times. It is an extremely rare chronic disease of unknown origin and part of the spectrum of leukocytoclastic vasculitis. We describe a case of EED simulating Kaposi''s sarcoma in a 52-year-old HIV infected female patient with no previous opportunistic infections and CD4+ count of 164/mm³. Therapy with oral dapsone (100 mg/day) for two weeks resulted in resolution of some lesions.     

A Case of Reticulate Acropigmentation of Kitamura: Dowling Degos Disease Overlap with Unusual Clinical Manifestations

Reticulate hyperpigmentary disorders are a group of rare genetic pigmentary abnormalities which includes reticulate acropigmentation of Kitamura (RAPK), Dowling-Degos disease (DD), reticulate acropigmentation of Dohi (RAPD), Haber''s syndrome, and Galli-Galli disease. A 25-year-old male presented with asymptomatic dark-colored lesions on his hands and feet with light-colored skin lesions involving the trunk since three years. Dermatological examination revealed hyperpigmented macules in a reticulate pattern involving the dorsa of the hands and feet, front and sides of the neck, axillae, periorbital region, and groin. Multiple pits were present over both palms, with breaks in dermatoglyphics. He also had multiple nonacne facial scars predominantly on the nose and malar areas. The patient had overlapping features of RAPK and DDD. In addition, he also had hypopigmented macules and acneiform facial scars. Such an overlap of features of reticulate pigmentation has not been previously reported in the literature.     

Biostatistics Series Module 3: Comparing Groups: Numerical Variables

Numerical data that are normally distributed can be analyzed with parametric tests, that is, tests which are based on the parameters that define a normal distribution curve. If the distribution is uncertain, the data can be plotted as a normal probability plot and visually inspected, or tested for normality using one of a number of goodness of fit tests, such as the Kolmogorov–Smirnov test. The widely used Student''s t-test has three variants. The one-sample t-test is used to assess if a sample mean (as an estimate of the population mean) differs significantly from a given population mean. The means of two independent samples may be compared for a statistically significant difference by the unpaired or independent samples t-test. If the data sets are related in some way, their means may be compared by the paired or dependent samples t-test. The t-test should not be used to compare the means of more than two groups. Although it is possible to compare groups in pairs, when there are more than two groups, this will increase the probability of a Type I error. The one-way analysis of variance (ANOVA) is employed to compare the means of three or more independent data sets that are normally distributed. Multiple measurements from the same set of subjects cannot be treated as separate, unrelated data sets. Comparison of means in such a situation requires repeated measures ANOVA. It is to be noted that while a multiple group comparison test such as ANOVA can point to a significant difference, it does not identify exactly between which two groups the difference lies. To do this, multiple group comparison needs to be followed up by an appropriate post hoc test. An example is the Tukey''s honestly significant difference test following ANOVA. If the assumptions for parametric tests are not met, there are nonparametric alternatives for comparing data sets. These include Mann–Whitney U-test as the nonparametric counterpart of the unpaired Student''s t-test, Wilcoxon signed-rank test as the counterpart of the paired Student''s t-test, Kruskal–Wallis test as the nonparametric equivalent of ANOVA and the Friedman''s test as the counterpart of repeated measures ANOVA.     

Hypnotherapy: A Useful Adjunctive Therapeutic Modality in Hansen's Disease

Hypnotherapy is a useful adjunctive psychotherapeutic procedure used in various conditions such as pain disorders, atopic dermatitis, and alopecia areata. However, it is less utilized in the field of dermatology. Only limited data exist on its role in the management of various skin diseases. There is dearth of literature on the role of hypnotherapy in Hansen''s disease (HD). We report two cases of HD, one with very resistant neuralgia and the other with recurrent erythema nodosum leprosum (ENL). Both the patients were assessed using hospital anxiety and depression scale, dermatology life quality index and the neuralgia was assessed using the visual analog scale. Three sessions of hypnotherapy were given to both the patients. There was dramatic improvement in the incidence of ENL and neuralgia and we could rapidly reduce the dose of drugs used for both conditions.     

Unusual Presentation of Idiopathic Sweet's Syndrome in a Photodistributed Pattern

A 40-year-old lady presented with history of multiple red raised painful lesions over her body of 10 days duration. Lesions spread from forearms to arms and back of trunk during the progress of the disease. Associated pain and burning sensation in the lesions was present while working in the sun. Mild to moderate grade fever, malaise, pain over large joints, decreased appetite, and redness of eyes was also present. There was no history of drug intake or other risk-factors. Dermatological examination revealed erythematous papules coalescing to form plaques with a pseudovesicular appearance over the extensor aspect of forearms and photo-exposed areas on the back of trunk. There was a sharp cut-off between the lesions and the photo-protected areas. Investigations revealed anemia, neutrophilic leukocytosis, raised erythrocyte sedimentation rate and positive C reactive protein. Skin biopsy showed characteristic features of Sweet''s syndrome. No evidence for any secondary etiology was found. She responded to a tapering course of oral steroids and topical broad spectrum photo-protection. This case is a very rare instance of idiopathic Sweets syndrome occurring in a photo-distributed pattern.     

Acquired Bilateral Nevus of ota-like Macules with Mucosal Involvement: A New Variant of Hori's Nevus

Acquired bilateral nevus of Ota-like macules (ABNOM) or Hori''s nevus, a rare form of acquired dermal melanocytoses, presents as bilateral facial blue-gray macules without ocular or mucosal involvement. This condition is mostly found in women of Asian descent and usually appears in the fourth or fifth decade of life. Pathogenesis is unknown, though few theories have been proposed. Effective treatment has been found to be achieved with pigment-specific lasers. Herein, we report a case of Hori''s nevus with mucosal involvement. A 42-year-old male patient, presented to us with blue-gray discoloration on either side of his face, both eyes, and in the mouth since the age of one year. Histopathological examination showed clusters and singly dispersed pigmented melanocytes within the upper and mid-dermis regions. Special staining of melanocytes using Masson-Fontana stain was positive. Diagnosis of Hori''s nevus was made by correlating clinical and histopathological findings. Patient was informed of his treatment options, but refused treatment. A similar case of Hori''s nevus with mucosal involvement has not been reported so far.     

CURRENT CONCEPTS IN THE PATHOGENESIS OF PSORIASIS

Psoriasis is a multi-factorial skin disease with a complex pathogenesis. Various factors which have been suggested to play a key role in the pathogenesis are T cells, antigen presenting cells (APC''s), keratinocytes, Langerhans'' cells, macrophages, natural killer cells, an array of Th1 type cytokines, certain growth factors like vascular endothelial growth factor (VEGF), keratinocyte growth factor (KGF), and others. It has been hypothesized that the disease starts with the activation of T cell by an unknown antigen, which leads to secretion of an array of cytokines by activated T cells, inflammatory cells, and keratinocytes. The characteristic lesion of psoriasis is due to the hyper-proliferation of the keratinocyte. Activated Langerhans'' cells migrate from skin to lymph nodes presenting the antigen to nodal naïve T cells (cells that have not been activated by antigen previously). The T cells activated by non-antigen-dependent mechanism may, however, become antigen-specific memory cells that react with a cross-reactive auto-antigen such as keratin (molecular mimicry). The genetic background of the disease may be suggested from the fact that concordance rate is 63–73% in monozygotic twins, as compared to 17–20% in dizygotic twins. Several disease susceptibility loci have been suggested as predisposing factors, PSORS1-PSORS9.     

Clinical Presentation and Evaluation of Dermatomyositis

Dermatomyositis (DM) is a chronic inflammatory disorder of the skin and muscles. Evidence supports that DM is an immune-mediated disease and 50–70% of patients have circulating myositis-specific auto-antibodies. Gene expression microarrays have demonstrated upregulation of interferon signaling in the muscle, blood, and skin of DM patients. Patients with classic DM typically present with symmetric, proximal muscle weakness, and skin lesions that demonstrate interface dermatitis on histopathology. Evaluation for muscle inflammation can include muscle enzymes, electromyogram, magnetic resonance imaging, and/or muscle biopsy. Classic skin manifestations of DM include the heliotrope rash, Gottron''s papules, Gottron''s sign, the V-sign, and shawl sign. Additional cutaneous lesions frequently observed in DM patients include periungual telangiectasias, cuticular overgrowth, “mechanic''s hands”, palmar papules overlying joint creases, poikiloderma, and calcinosis. Clinically amyopathic DM is a term used to describe patients who have classic cutaneous manifestations for more than 6 months, but no muscle weakness or elevation in muscle enzymes. Interstitial lung disease can affect 35–40% of patients with inflammatory myopathies and is often associated with the presence of an antisynthetase antibody. Other clinical manifestations that can occur in patients with DM include dysphagia, dysphonia, myalgias, Raynaud phenomenon, fevers, weight loss, fatigue, and a nonerosive inflammatory polyarthritis. Patients with DM have a three to eight times increased risk for developing an associated malignancy compared with the general population, and therefore all patients with DM should be evaluated at the time of diagnosis for the presence of an associated malignancy. This review summarizes the immunopathogenesis, clinical manifestations, and evaluation of patients with DM.