Kawasaki disease (KD) is a systemic vasculitis that mostly affects children below the age of 5. The vasculitis involves arteries of medium size, especially the coronaries. Various etiologies have been proposed including association with micro-organisms, bacterial superantigens, and genetic factors, however, the exact cause remains unknown. There is no specific laboratory test for KD. Diagnosis is clinical and depends upon the presence of fever for ≥5 days and 4 or more of five principal features, viz. polymorphous exanthem, extremity changes, mucosal changes involving the lips and oral cavity, bilateral bulbar conjunctival injection, and unilateral cervical lymphadenopathy. The term “incomplete KD” refers to the presence of fever and less than four principal clinical features. Recognition of this group of patients is important because it is usually seen in infants and risk of coronary abnormalities is increased probably because of delays in diagnosis. However, what appears to be “incomplete” at a given point of time may not actually be so because some of the features may have already subsided and others may evolve over time. Hence, a detailed dermatological examination is warranted in all cases, especially in incomplete KD, to ensure timely diagnosis. Although KD is a self-limiting disease in most patients, coronary artery abnormalities (CAAs) including coronary dilatations and aneurysms may develop in up to 25% of untreated patients. CAAs are the most common cause of morbidity and mortality in patients with KD. Treatment is aimed at reducing inflammation and consists of intravenous immunoglobulin (IVIG) along with aspirin. Despite treatment, some patients may still develop CAAs, and hence, long-term follow up is of utmost importance.

On the basis of current evidence derived from hospital-based studies, mostly from North India, the prevalence of psoriasis in adults varies from 0.44 to 2.8%, with a much lower prevalence in children. The peak age at onset in adults is in the third and fourth decade of life, with a slight male preponderance. It is recommended that population-based large epidemiologic studies should be undertaken in different parts of the country for estimating the correct prevalence of psoriasis in general population. Chronic plaque-type psoriasis is the most common morphologic presentation of psoriasis, accounting for more than 90% of all cases. Other morphologic variants that deserve special mention include palmoplantar psoriasis, pustular psoriasis, and recalcitrant psoriasis.For epidemiologic purposes, psoriasis can be classified into early and late onset psoriasis. Psoriasis can be classified on the basis of morphology and extent of involvement into localized and widespread disease.For the purpose of clinical trials, psoriasis may be classified as mild psoriasis, moderate psoriasis, and severe psoriasis. The literature shows that there is a significant risk of psoriatic arthritis (7–48%) in patients with plaque-type psoriasis. Hence, it is recommended to evaluate for its presence by detailed history taking and clinical examination, and if necessary, by appropriate radiological investigations. Evidence on the association between plaque-type psoriasis and cardiovascular disease risk factors and ischemic heart disease isinconsistent.On the basis ofavailable evidence, it is prudent to proactively look for metabolic syndrome, dyslipidemia, and obesity, especially in patientswith severe psoriasis (Level 1+ evidence based on systematic reviews and meta-analysis). Based on the current evidence, the psoriasis area severity index appears to be the most valid and reproducible clinical severity score in the management of adult patients with plaque-type psoriasis.

Psoriasis generally does not affect survival but has significant detrimental effect on quality of life (QOL), which may be comparable to that of ischemic heart disease, diabetes, depression, and cancer. The foremost important thing in the management of psoriasis is counseling of the patient. The clinician needs to be empathetic and spend adequate time with the patient and educating the patient about psoriasis. Clinicians should make it clear to the patient that the primary goal of treatment is control of the disease rather than cure. Eating a balanced and low glycemic diet could be an important adjuvant factor in the prevention and treatment of moderate nonpustular psoriasis. Obese people are more likely to have severe psoriasis and psoriatic arthritis than people with an average body mass index. Dietary supplementation with oily fish, rich in n-3 fatty acids, in psoriasis had shown mixed results in trials. Promising results have been documented for parenteral application of n-3 fatty acid, but not with oral supplementation. Increased smoking or alcohol abuse increases the risk of developing psoriasis and may influence disease severity, and hence must be avoided. Soaking in warm water with bath oil can be done in extensive psoriasis for hydration and emollient effect, and bland soaps or soap substitutes should be used; antiseptics should be avoided as they may irritate the skin. Relatively small, localized patches of psoriasis may improve with occlusion, i.e., waterproof adhesive dressings. The use of emollients is an internationally accepted standard adjunctive to the treatment of psoriasis. Dermatology Life Quality Index is a psychometrically sound and responsive measure of psoriasis-specific outcomes and most comprehensively captures the impact of clinical signs and symptoms on patient's well-being.

The biologics currently in use for psoriasis in India are etanercept, infliximab and recently introduced itolizumab and secukinumab. Biosimilars, expected to play a significant role in psoriasis management in future, have also been available for the last few years. Patients with psoriasis may be considered eligible to receive treatment with any of the licensed biologic interventions when they fulfill the eligibility criteria. The decision to proceed with treatment must be made in collaboration with the patient and include a careful assessment of the associated risks and benefits. Etanercept is indicated in moderate to severe psoriasis and moderate to severe psoriatic arthritis with a dose of 25 mg or 50 mg twice weekly. Methotrexate may be recommended as co-medication in certain clinical circumstances, e.g., where it is required for associated arthropathy, or to improve efficacy. Infliximab is indicated in severe psoriasis and moderate to severe psoriatic arthritis. Infliximab therapy should be initiated at a dose of 5 mg/kg at weeks 0, 2 and 6 and disease response assessed at 3 months.In patients who respond, subsequent infusions (5 mg/kg) should be given at 8-week intervals to maintain disease control although long-term data are available only up to 1 year.Interrupted therapy should be avoided given the associated increased risk of infusion reactions and poorer disease control. Itolizumab is indicated in moderate to severe plaque psoriasis. It is given in a dose of 1.6mg/kg iv infusions every 2 weeks for 12 weeks initially and then 1.6mg/kg every 4 weeks up to 24 weeks. Long term data are unavailable. Secukinumab is indicated in moderate to severe plaque psoriasis and psoriatic arthritis.An initial loading dosing regimen of 300 mg secukinumab should be given by subcutaneous injection at weeks 0, 1, 2 and 3 followed by maintenance dose of 300 mg every 4 weeks starting at week 4. To exclude tuberculosis (TB) before anti TNF alfa therapy and therapy with itolizumab, pretreatment chest X-ray and Mantoux skin test currently remain the preferred screening tests in patients not on immunosuppression. During treatment, and for 6 months following discontinuation, a high index of suspicion for TB should be maintained. The effect of secukinumab on TB reactivation is as yet poorly understood, hence, in the Indian scenario, it is better to follow the same guidelines for ruling out latent TB

Introduction: The term facial acanthosis nigricans (FAN) lacks definition of precise clinical and histopathological features. We present a descriptive study of patients with FAN to define pigmentary patterns and estimate the prevalence of obesity and insulin resistance in these cases. Materials and Methods: It is a prospective study that included all patients with classical AN of the neck and/or other areas with facial acanthosis nigricans described as brown-to-black macular pigmentation with blurred ill-defined margins, found on the zygomatic and malar areas. The body mass index (BMI) and waist circumference (WC) of the included patients were used as parameters of obesity.Homeostatic Model of Assessment of Insulin Resistance (HOMA2 IR) was used as a parameter to evaluate insulin resistance. Histopathological features of the 6 skin biopsies that were possible were reviewed. Results: Among the 102 included individuals, the patterns of facial pigmentation seen in addition to the classic pattern involving zygomatic and malar areas were a hyperpigmented band on the forehead in 59.80%, periorbital darkening in 17.64%, perioral darkening in 12.74%, and generalized darkening in 9.8% of cases. 85.29% of the males and 100% of the females were found to be obese. Varying degrees of insulin resistance was noted in 82.34% of the individuals. Six biopsies available for evaluation showed changes such as mild epidermal hyperplasia with prominent basal melanin, however, without the typical papillomatosis seen in AN of the flexures. Conclusion: We document an increased prevalence of obesity and insulin resistance in patients presenting with FAN and its presentations in addition to the classical description. We propose that FAN can be considered a cutaneous marker of insulin resistance and that HOMA2 IR can serve as a parameter of insulin resistance in such cases.

An elderly patient presented with two clusters of asymptomatic fleshy and pedunculated papules. Biopsy of the papules was consistent with neurofibromas. Decades prior she had undergone a surgery for the excision of a large schwannoma. Given her lack of other neurofibromatosis findings, the patient was diagnosed with multisegmental neurofibromatosis (multi-SN) with deep schwannoma, a possible new phenotype of SN. Because this entity may be associated with internal malignancy, it is important to screen and educate these patients as well as to provide regular follow-up.

Griscelli syndrome (GS) is a rare autosomal recessive multisystem disorder of pigmentary dilution of skin, silver gray hair, variable immunodeficiency, neurological impairment, and abnormal accumulation of melanosomes in melanocytes. GS type 3 is characterized by hypomelanosis with no immunological and neurological manifestation. Prognosis is very good in type 3 GS and usually require no active intervention, as opposed to type 1 and 2 where early diagnosis and treatment plays a crucial role in patient's survival. The characteristic phenotypic appearance, especially the pigment dilution of the patient's hair, is emphasized here.

Acute skin graft-versus-host disease (GVHD) classically presents as a pruritic erythematous maculopapular rash. We describe a patient who underwent allogeneic hematopoietic stem cell transplantation and presented with a hand foot and mouth disease like clinical presentation. Histopathology was suggestive of acute GVHD. This case is being reported to make dermatologists aware of this unusual presentation of GVHD.

Cowden's syndrome is an autosomal dominant genodermatosis with variable orofacial and systemic manifestations. Here we present one such classical case of Cowden's syndrome in a 45-year-old female patient with features such as multiple cutaneous papillomatosis, oral fibromas, and fibromas involving multiple organs such as gastrointestinal tract (multiple polyps), thyroid disorders, and breast cancer.

Progressive nodular histiocytosis (PNH) represents a very rare type of non-Langerhans cell histiocytosis. It is characterized by progressive appearance of papules and nodules without spontaneous resolution. We report a 60-year-old patient with novel clinical features in the form of extensive noduloulcerative lesions, ichthyotic patches, and laryngeal involvement culminating in fatal outcome prior to therapeutic intervention. Although the presenting features were baffling, histopathology and immunohistochemistry clinched the diagnosis of PNH.

Keratosis lichenoides chronica also known as Nekam's disease is a rare mucocutaneous disorder, characterized clinically by asymptomatic violaceous keratotic papules arranged in linear, reticular, or plaque form usually on the trunk and extremities and histologically by interface dermatitis. The disease is considered rare with only 128 cases being reported in the literature till date and very few from India. We report a case of a 40-year-old man who presented with constellation of features of lichen planus, seborrheic dermatitis, and apthous ulcers, which upon workup was found to be Nekam's disease.

Haberland syndrome or Fishman syndrome also known as encephalocraniocutaneous lipomatosis (ECCL) is a rare, congenital neurocutaneous disorder. It is characterized by unilateral involvement of skin, eyes and central nervous system. We report the case of a 28-year-old woman who presented with soft lipomatous swelling over right temporal area with nonscarring alopecia of part of frontal and parietal region. The patient had a history of seizures and ipsilateral scleral dermoid. Computed tomography scan findings were suggestive of lipomas and calcification of falx. Magnetic resonance imaging showed right-sided hemi atrophy and two intracranial cysts. We report this case because of its rarity.

Primary cutaneous anaplastic large cell lymphoma (pcALCL) forms 9% of the cutaneous T-cell lymphomas. It usually presents as solitary reddish brown ulcerating nodule or indurated plaque. Sometimes, it mimics other dermatological diseases such as eczema, pyoderma gangrenosum, pyogenic granuloma, morphea, and squamous cell carcinoma. Our case presented with large pyogenic granuloma like lesion with regional lymphadenopathy. Since pcALCL is rare, one can misdiagnose such cases and therefore high index of suspicion is necessary.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL; α/β T-cell subtype) is a distinct variantof cutaneous T-cell lymphomas, which presents as inflammatory subcutaneous nodules. A 17-year-old male presented with recurrent fever with concomitant facial swelling, pedal edema, hepatosplenomegaly, and mildly tender subcutaneous plaques in generalized distribution along with patches of scarring alopecia on scalp. There were features of macrophage activation syndrome in the form of hemophagocytosis in the bone marrow, pancytopenia, high serum lactate dehydrogenase levels, low fibrinogen clotting activity, prolonged activated prothrombine time (aPTT), increased serum ferritin, hypoalbuminemia, and hypertriglyceridemia. Histopathology showed lobular panniculitis-like infiltration by atypical lymphocytes rimming the adipocytes. Immunohistochemistry revealed positive CD3 and CD8 markers, whereas CD4, CD56, and CD20 were negative, consistent with the diagnosis of α/β type of SPTCL. Treatment with oral prednisolone (1mg/kg/day) and cyclosporine (2mg/kg/day; 100 mg) led to rapid subsidence of fever, plaques, and abnormal hematological parameters within a few weeks.

Eccrine angiomatous hamartoma (EAH) is an exceedingly rare benign tumor-like lesion prevalent in childhood, which can produce pain and marked sweating. Histological features include proliferation of eccrine sweat glands and angiomatous capillary channels. It may be rarely associated with underlying pathological conditions. A 15-year-old female patient presented with multiple tender reddish papules and nodules coalescing to form plaques of 10 × 8 cm over the anterior aspect of the right lower thigh since birth. It was associated with hypertrichosis, hyperhidrosis, pain, and occasional bleeding on trauma. Histopathological examination of the lesion showed increased proliferation of both eccrine and angiomatous channels. Ultrasonography and Color Doppler of the right thigh showed dilatation of the great saphenous vein (GSV) above the right knee, with evidence of grade 3 reflux at saphenofemoral junction. Magnetic resonance imaging revealed large dilated GSV with slow flow and venous malformation in the anterior part of the right knee