Psoriasis is resistant to treatment and it shows frequent relapse; systemic treatment is often associated with toxicities, and long-term safety data are lacking for most of the newer drugs like biologics. Moreover, some body areas such as hands, feet, intertriginous areas, scalp, and nails are even more resistant. Frequently, systemic treatments are necessary considering the higher psychological impact on the patient. There is a lack of agreement on the best therapeutic modalities in the management of psoriasis involving difficult-to-treat locations. At present, there are no Indian guidelines for these conditions. Available literature has been reviewed extensively on the treatment of psoriasis involving difficult-to-treat locations; level of evidence has been evaluated as per the Oxford Centre for Evidence-Based Medicine 2011 guideline, and therapeutic suggestions have been developed. Best care has been employed to consider socioeconomic, cultural, genetic, and ethnic factors to prepare a therapeutic suggestion that is appropriate and logical to be used among Indian population and people of similar ethnic and socioeconomic background.

In observational studies, as well as in interventional ones, it is frequently necessary to estimate risk that is the association between an observed outcome or event and exposure to one or more factors that may be contributing to the event. Understanding incidence and prevalence are the starting point in any discussion of risk assessment. Incidence rate uses person-time as the denominator rather than a simple count. Ideally, rates and ratios estimated from samples should be presented with their corresponding 95% confidence intervals (CIs). To assess the importance of an individual risk factor, it is necessary to compare the risk of the outcome in the exposed group with that in the nonexposed group. A comparison between risks in different groups can be made by examining either their ratio or the difference between them. The 2 × 2 contingency table comes in handy in the calculation of ratios. Odds ratio (OR) is the ratio of the odds of an event in the exposed group, to the odds of the same event in the nonexposed group. It can range from zero to infinity. When the odds of an outcome in the two groups are identical, then the OR equals one. OR >1 indicates exposure increases risk while OR <1 indicates that exposure is protecting against risk. The OR should be presented with its 95% CI to enable more meaningful interpretation – if this interval includes 1, then even a relatively large OR will not carry much weight. The relative risk (RR) denotes the ratio of risk (probability) of event in exposed group to risk of same event in the nonexposed group. Its interpretation is similar (but not identical) to the OR. If the event in question is relatively uncommon, values of OR and RR tend to be similar. Absolute risk reduction (ARR) is a measure of the effectiveness of an intervention with respect to a dichotomous event. It is calculated as proportion experiencing the event in control group minus the proportion experiencing the event in treated group. It is often used to denote the benefit to the individual. The reciprocal of ARR is the number needed to treat (NNT), and it denotes the number of subjects who would need to be treated to obtain one more success than that obtained with a control treatment. Alternatively, this could also denote the number that would need to be treated to prevent one additional adverse outcome as compared to control treatment. Extended to toxicity, the NNT becomes a measure of harm and is then known as the number needed to harm (NNH). NNT and NNH are important concepts from the policy makers perspective and ideally should be calculated in all trials of therapeutic or prophylactic intervention.

As researchers, we often collect data on a clinical record form or a questionnaire. It is an important part of study design. If the questionnaire is not well designed, the data collected will not be useful. In this section of the module, we have discussed some practical aspects of designing a questionnaire. It is useful to make a list of all the variables that will be assessed in the study before preparing the questionnaire. The researcher should review all the existing questionnaires. It may be efficient to use an existing standardized questionnaire or scale. Many of these scales are freely available and may be used with an appropriate reference. However, some may be under copyright protection and permissions may be required to use the same questionnaire. While designing their own questionnaire, researchers may use open- or close-ended questions. It is important to design the responses appropriately as the format of responses will influence the analysis. Sometimes, one can collect the same information in multiple ways - continuous or categorical response. Besides these, the researcher can also use visual analog scales or Likert's scale in the questionnaire. Some practical take-home points are: (1) Use specific language while framing the questions; (2) write detailed instructions in the questionnaire; (3) use mutually exclusive response categories; (4) use skip patterns; (5) avoid double-barreled questions; and (6) anchor the time period if required.

Mycosis fungoides is the most common primary cutaneous T-cell lymphoma. The approach to diagnosis and further follow-up is outlined. Evidence for interventions is based classically on a Tumor Node Metastasis Blood TNMB “stage-based” approach. The treatment options in India are limited. The options as per risk stratification and prognostic index are discussed. Early stages and low-risk patients can be managed with expectant policy or skin-directed therapies including topical steroids and phototherapy; intermediate-risk patients can be opted for interferons or retinoids or low dose methotrexate along with radiotherapy including total skin electron beam therapy while high-risk patients are managed most often with single agent or multiagent palliative chemotherapy. Patients who are intermediate- or high-risk need management by a multispecialty team at tertiary care centers.

Cutaneous T-cell lymphomas (CTCLs) represent a group of rare and heterogeneous diseases that are very difficult to treat at advanced stages. The development of monoclonal antibodies is a new hope for the treatment of these diseases. Alemtuzumab (Campath) is a humanized IgG1 kappa monoclonal antibody specific for CD52, an antigen expressed by most T and B lymphocytes. Alemtuzumab may frequently induce long-term remissions in patients with Sezary syndrome but high-dose treatments lead to severe cytopenia, immune depletion, and opportunistic infections. This treatment is less efficient in mycosis fungoides (MF). Brentuximab vedotin is a chimeric anti-CD30 monoclonal antibody conjugated to monomethyl auristatin E, a cytotoxic antitubulin agent. Brentuximab vedotin is a very interesting new treatment for advanced tumor MF, Sezary syndrome, and primary cutaneous CD30+ lymphoproliferative disorders. The main limiting adverse event is neurosensitive peripheral neuropathy. Mogamulizumab is a humanized anti-C-C chemokine receptor Type 4 monoclonal antibody with a defucosylated Fc region leading to increased antibody-dependent cellular cytotoxicity. Mogamulizumab is very efficient on aggressive peripheral T-cell lymphomas, particularly adult T-cell leukemia/lymphoma and CTCLs, especially on the blood component of tumor cells. The main limiting events are related to the concomitant depletion of regulatory T-cells. IPH4102 is a humanized monoclonal antibody that targets the immune receptor KIR3DL2/CD158k. Preclinical results with this antibody offer proofs of concept for the clinical development of IPH4102 to treat patients with advanced CTCL.

The term “Primary Cutaneous B-Cell Lymphoma” (PCBCL) comprehends a variety of lymphoproliferative disorders characterized by a clonal proliferation of B-cells primarily involving the skin. The absence of evident extra-cutaneous disease must be confirmed after six-month follow-up in order to exclude a nodal non-Hodgkin's lymphoma (NHL) with secondary cutaneous involvement, which may have a completely different clinical behavior and prognosis. In this article, we have summarized the clinico-pathological features of main types of PCBCL and we outline the guidelines for management based on a review of the available literature.

Background: Nonmycosis fungoides/Sézary syndrome (non-MF/SS) primary cutaneous lymphomas (PCL) are currently categorized under the 2005-World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for PCL. These differ in behavior from secondary cutaneous lymphomas (SCL) and to lymphomas limited to the oral cavity (primary oral lymphomas [POL]) both categorized under the 2016-WHO classification for lymphoid neoplasms. Aims: This study aims to report the first series of non-MF/SS PCL, SCL, and POL in a Mexican cohort, examine the applicability of current classification systems and compare our findings with those from foreign cohorts. Materials and Methods: Eighteen non-MF/SS PCL, four SCL, and two POL with available tissue for morphology and immunophenotypic assessment were reclassified according to the 2005-WHO/EORTC and 2016-WHO classifications. Results: Non-MF/SS PCLs were primarily of T-cell origin (61%) where CD30+ lymphoproliferative disorders predominated, followed by Epstein–Barr virus-induced lymphomas, and peripheral T-cell lymphomas, not otherwise specified. Primary cutaneous B-cell lymphomas (BCL) were primarily of follicle center cell origin followed by postgerminal lymphomas of the diffuse large BCL variety. Conclusions: Most non-MF/SS PCL, SCL, and POL can be adequately categorized according to the 2005-WHO/EORTC and 2016-WHO classification systems, even when dealing with clinically atypical cases. The relative frequencies in our cohort hold closer similarities to Asian registries than from those of Europe/USA, supporting the concept of individual and/or racial susceptibility, and the notion of geographical variances in the rate of lymphomas. In particular, such disparity may arise from viral-induced lymphomas which might show partial geographical restriction.

Context: Diabetes mellitus is associated with many skin manifestations including vitiligo. Vitiligo occurs more commonly in Type 1 diabetes mellitus. A few recent studies have shown its increased occurrence in Type 2 diabetes mellitus. Aims: This study aims to study the prevalence of vitiligo in Type 2 diabetic patients and to compare the prevalence of vitiligo in age- and sex-matched group of nondiabetic population. Settings and Design: The present study was a hospital-based cross-sectional study conducted in the Department of Dermatology in a tertiary care hospital. Subjects and Methods: Six hundred consecutive consenting patients of Type 2 diabetes were included in the study group and age- and sex-matched controls were healthy nondiabetic adult volunteers attending the Department of Dermatology. Fasting and postprandial blood sugar levels were done. A complete history, physical examination, and wood's lamp examination to detect vitiligo were conducted. In all those with vitiligo, the type of vitiligo was noted. Statistical Analysis Used: Data were analyzed using SPSS software version 20.0. Comparison between the presence of vitiligo in cases and controls was done using Chi-square test with P = 0.05 for significance. Results: Vitiligo was seen in 12% of cases and 6% of control group which was statistically significant (P < 0.01). There was no significant difference between cases and controls with respect to type of vitiligo. Conclusions: Vitiligo can occur in Type 2 diabetics as seen in our study and few other recent studies. The exact pathogenesis is not very clear and needs further consideration.

Background: Pemphigus is an acquired immunobullous disorder in which antibodies are directed against epidermal cadherins. Despite the commercial availability and less cost of enzyme-linked immunosorbent assays (ELISAs) to detect antidesmoglein 1 (Dsg1) and anti-Dsg3, immunofluorescence is still widely used for confirmation of diagnosis. Aims: (1) To compare the usefulness of indirect immunofluorescence (IIF) and ELISA tests in the diagnosis of pemphigus. (2) To find the clinical correlation between the tests and severity of the disease. Materials and Methods: Sixty-one patients (27 women and 34 men, age distribution from 20 to 75) were clinically diagnosed as pemphigus (pemphigus foliaceus - 11, pemphigus vulgaris - 50) and were recruited for the study. IIF and Dsg ELISA were performed and the findings were compared with each other and with the pemphigus area activity score. Data were entered in SPSS and were analyzed using Kruskal–Wallis test. Results: There was a moderate positive correlation between the cutaneous score and Dsg1 titer, and mucosal score and Dsg3 titer. The titer of IIF showed statistically significant positive correlation with the cutaneous score but not the mucosal score. Dsg ELISA showed higher sensitivity (90.2%) than IIF (75.4%) in the diagnosis of pemphigus. Conclusions: Dsg ELISA is a more sensitive method than IIF and shows more correlation with the disease severity.

Aims: This study aims to evaluate the knowledge and pattern of self-medication for acne among undergraduate medical students at a tertiary care teaching hospital. Materials and Methods: This cross-sectional study was conducted in II MBBS (Group A), III MBBS Part I (Group B), and III MBBS Part II (Group C) students. Prevalidated questionnaire about knowledge, attitude, and practice of self-medication were administered to participants. Data were analyzed using one-way analysis of variance and Chi-square test. Results: Out of 582 students who responded to questionnaire, 518 suffered from acne. Self-medication practice was observed in 59.2% students. Significantly higher number of female students practiced self-medication (P < 0.0001). Most common source of information was seniors/friends/family members (34.2%). The mildness of illness (42.3%) was the most common reason of self-medication. A total mean score of knowledge was significantly higher in Group C as compared to Group A (P < 0.001) and Group B (P < 0.05). Allopathic medication was preferred by 69.8% students. Seventy-five percentage students read leaflet/package insert/label instruction and expiry date of the medicines. Conclusions: The participating students lack the knowledge about self-medication for acne. Adequate knowledge and awareness about the appropriate use of medication will reduce the practice of self-medication and improve rational prescribing.

Background: Chronic alcohol intake impacts skin directly, through organ dysfunction or by modifying preexisting dermatoses. However, dermatoses afflicting chronic alcoholics figure in a few studies only. Aim: This study aims to correlate the spectrum of dermatoses in chronic alcoholics with the quantum/duration of alcohol intake and raised liver transaminases. Materials and Methods: Adult males, totaling 196, ascertained to fulfill the Royal College of Psychiatry criteria for chronic alcoholism by the de-addiction center and referred for dermatological consult were enrolled as cases, and similar number of age-/sex-matched teetotallers, as controls. Data emanating from detailed history, clinical examination, and routine liver functions tests were summarized and subsequently analyzed, including statistically using the Chi-square, independent “t” and Spearman's rank correlation tests, and compared with data from previous studies. Results: Majority (104) drank 41–50 units of alcohol/week since 3–40 (mean: 20.01 ± 9.322) years. Generalized pruritus (odds ratio [OR]: 31.15, P < 0.001), xerosis (OR: 3.62, P = 0.008), and seborrheic dermatitis (OR: 12.26, P < 0.001) were significantly more common in cases than controls. Infections (73; 37.2%), eczemas (45; 22.9%), and generalized hyperpigmentation (28; 14.2%) were the major presenting complaints. Spider nevi, gynecomastia, and pellagroid dermatitis were present in 34 (17.3%), 19 (9.7%), and 8 (4.1%) respectively exclusively in cases only. Commonly seen systemic abnormalities were an alcoholic liver disease (45; 22.9%), diabetes mellitus (23; 11.7%), and peripheral neuropathy (19; 9.7%). Conclusion: Knowledge of cutaneous manifestations of chronic alcoholism could prompt in-depth history taking of alcohol intake, lead to specialist referral and thereby enable timely de-addiction, hopefully before serious adversities in the chronic alcoholics.

Introduction: Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive potentially life-threatening condition, characterized by glucocorticoid deficiency, preserved aldosterone/renin secretion, and secondary rise in plasma adrenocorticotropic hormone level. This occurs due to either mutation in adrenocorticotropic receptor (25%, FGD Type-1) or in the MC2 receptor accessory protein (15%–20%). However, in about 50% patients, no identifiable mutations have been identified. Clinically, it manifests with weakness, fatigue, weight loss, anorexia, nausea, vomiting, diarrhea, abdominal pain, hypoglycemia, and hypothermia. Progressive mucocutaneous pigmentation is a conspicuous presentation. Repeated hypoglycemia may result in seizure, persistent neurological, severe mental disability, and even sudden death. Standard therapy is oral glucocorticoids (10–15 mg/m²). Patients and Results: Two familial cases of FGD were put on progressively increasing doses of oral glucocorticoids (10 mg, 15 mg, and 20 mg/m²/day, each for 6 weeks) to achieve the best response without any adverse effects. One patient had excellent improvement with 15 mg/m²/day, and another required 20 mg/m²/day. The latter patient had excellent overall improvement with only moderate improvement in pigmentation. Conclusion: Glucocorticoids replacement with optimum dose is necessary in FGD to promote physical and neurological growth and to prevent adrenal crises, hypotension, hypoglycemia, and sudden death. Higher dose than mentioned in literature (15 mg/m²/day) may be required in selected cases. Mucocutaneous pigmentation may require even higher dose than we used. More studies are required.

Psoriasis is a complex inflammatory disease that occurs in genetically susceptible individuals and presents with the development of erythematous scaly plaques on the skin. Interleukins (ILs) in the Th17 pathway play a pivotal role in the pathogenesis of psoriasis and have thus become targets for recent biologic drug development. Secukinumab is a human monoclonal IgG1k antibody that has been developed to target and block the actions of IL-17A. Secukinumab recently approved for use as first-line systemic therapy in a patient with moderate to severe psoriasis has been studied first in psoriasis before other diseases. Both Phase II and III clinical trials have demonstrated the effectiveness of secukinumab in the treatment of moderate-to-severe plaque psoriasis, and it has demonstrated superiority to other comparable biologics on the market, including the tumor necrosis factor inhibitor etanercept. Secukinumab has also shown superiority to ustekinumab, a relatively recent biologic introduced for the treatment of psoriasis. Besides demonstrating better efficacy compared to etanercept and ustekinumab, secukinumab has also demonstrated a greater impact of the quality of life of patients with a comparable safety profile. Secukinumab shows great promise in having a tremendous impact on the treatment of plaque psoriasis based on its ability to produce similar, if not better, clinical outcomes than other biologic antipsoriasis medications.

Cutaneous T-cell lymphoma (CTCL) commonly presents as mycosis fungoides or Sezary syndrome, both having CD4 positivity. A subset of CTCL which lacks CD4 surface marker is classified as cutaneous g and d–T-cell lymphoma (CGD-TCL). Because of its rarity and inability to study large number of patients, the impact of immunophenotype on the clinical outcome of primary CTCL in patients is limited. We report a case of primary CGD-TCL in a 71-year-old male because of this rarity and to emphasize its aggressive nature.

Mycosis fungoides (MF) is the most common type of primary cutaneous lymphomas. Several clinical variants of MF have been described. Purely, hypopigmented variant of MF (HMF) is rare. Phototherapy, especially photochemotherapy (Psoralen and ultraviolet), is the most widely used method and is recommended as the first-line treatment for HMF. However, there are no standard guidelines for phototherapy as the disease is uncommon. We, hereby, report a 30-year-old woman with HMF in whom clinical, histopathological, and immunohistochemical remission was achieved following narrow-band ultraviolet B therapy.

A 2-month-old boy was presented with widespread lateralized blue macules (nevus cesius), an extensive nevus flammeus, and large patches of cutis marmorata telangiectatica congenita. Moreover, he had macrocephaly, a coarse facial appearance with depressed nasal bridge, retinal abnormalities, septal defects of the heart, and obliteration of the left brachiocephalic vein and major veins of the left arm with pronounced collateralization. The multisystem disorder of this boy cannot be categorized within the present classification of distinct types of phacomatosis pigmentovascularis. Although some similar complex cases have previously been reported, it seems too early to give them a specific name. Rather, the present case should be included, so far, into the group of unclassifiable types of phacomatosis pigmentovascularis.

Juvenile hyaline fibromatosis (JHF) is a rare hereditary disease with an autosomal recessive transmission. JHF is characterized by papulonodular skin lesions, osteolytic bone lesions, flexural joint contractures, and gingival hyperplasia and usually diagnosed in infancy or early childhood. JHF is thought to be a disorder of collagen metabolism and characterized by homogenous amorphous eosinophilic material and fibrous tissue. We report the case of a 14-year-old male child with multiple papulonodular skin lesions, progressive flexion contractures of joints, and severe gingival hyperplasia, with a 10-year follow-up. Although the lesions were totally removed thrice during the last 10 years, they recurred rigorously.

Context: In vivo epidermal nuclear staining (ENS) can be found in patients with autoimmune connective tissue diseases (CNTDs) and other diseases. Aims: The aim of this study was to reveal the underlying diseases and direct immunofluorescence (DIF) characters of patients with in vivo ENS and association of in vivo ENS with circulating autoantibodies. Settings and Design: A retrospective analysis was conducted involving skin biopsy specimens submitted for DIF study at the Dermatoimmunology Laboratory at Siriraj Hospital between 2002 and 2012. Subjects and Methods: The findings of DIF study, clinical manifestations, and diagnosis of patients who had positive ENS were investigated. Statistical Analysis Used: The SPSS software version 18.0. Descriptive statistics were used to report demographic data, clinical characteristics, and laboratory investigation results. Moreover, Chi-squared test or Fisher's exact test were used to compare the categorical variables. Results: One hundred and thirty-eight out of 3735 submitted specimens (3.7%) showed positive ENS. The most common diagnosis was CNTD (79%) followed by vasculitis (10.1%). Lupus erythematosus was the most common diagnosis among CNTD (78%). No association between levels of serum antinuclear antibodies (ANA) titer and intensity of ENS (P = 0.660). However, we found that patients with positive in vivo ANA had lower prevalence of systemic involvement. Conclusions: Although lupus erythematosus was the most common diagnosis among patients with in vivo ENS, the presence of ENS does not indicate any specific diagnosis. However, patients with ENS tend to have less systemic involvement.

Context: Discoid lupus erythematosus (DLE) and human immunodeficiency virus (HIV) are both disorders of the immune system. The pathophysiology of these diseases varies greatly as DLE is characterized by an overactive immune system that attacks normal host cells, whereas HIV is characterized by an exogenous attack on the immune system that depletes it of key cell types. Although the reason is unknown, co-occurrence of DLE and HIV is rare. Aims: The goal of this study is to determine the prevalence of co-occurrence of DLE and HIV and to determine whether patients with both DLE and HIV share any clinical feature. Subjects and Methods: The medical records of all patients seen within a single academic health center over a 20-year period were reviewed to determine the prevalence of cutaneous lupus, HIV, and co-occurrence of these conditions. The charts of patients diagnosed with both conditions were further reviewed to determine similarities between them. Results: Of the 10,719 patients diagnosed with HIV and 182 patients diagnosed with cutaneous lupus, only 2 patients were diagnosed with both conditions. Both of these patients were diagnosed with DLE several years after being diagnosed with HIV. They had an undetectable HIV viral load, normal CD4 T-cell counts, and were on antiretroviral therapy when diagnosed with DLE. Conclusion: These results confirm that co-occurrence of DLE and HIV is rare. Although our study population was small, findings from these patients suggest that in HIV-positive patients, DLE manifestations occur when their HIV disease activity is minimal.