Pustular psoriasis: A distinct aetiopathogenic and clinical entity.

Pustular psoriasis is a distinct subset of psoriasis that presents with involvement of the skin in the form of sterile pustules along with systemic manifestations. Though it has been conventionally grouped under the umbrella of psoriasis, recent research has shed light on its pathogenetic mechanisms associated with the IL-36 pathway, which is distinct from conventional psoriasis. Pustular psoriasis in itself is a heterogeneous entity consisting of various subtypes, including generalised, localised, acute, and chronic forms. There is confusion regarding its current classification as entities like deficiency of IL-36 antagonist (DITRA) which are closely related to pustular psoriasis both in their pathogenetic mechanism and its clinical manifestations, are not included under pustular psoriasis. Entities like palmoplantar pustulosis, which presents with similar clinical features but is pathogenetically distinct from other forms of pustular psoriasis, are included under this condition. Management of pustular psoriasis depends upon its severity; while some of the localised variants can be managed with topical therapy alone, the generalised variants like Von Zumbusch disease and impetigo herpetiformis may need intensive care unit admission and tailor-made treatment protocols. The advent of newer biologics and better insight into the pathogenesis of pustular psoriasis has opened the way for newer therapies, including tumour necrosis factor-alpha inhibitors, interleukin-1 inhibitors, interleukin-17 inhibitors, and granulocyte monocyte apheresis. It continues to be an enigma whether pustular psoriasis is actually a variant of psoriasis or an entirely different disease entity, though we feel that it is an entirely different disease process.

Unconventional treatment options in psoriasis: A review.

Psoriasis is a common skin disease that affects 1-3% of the general population. The treatment depends on body surface area involved, quality of life impairment and associated comorbidities. The treatment options include topical therapy, phototherapy, conventional systemic therapy (methotrexate, cyclosporine and acitretin), biologics and oral small molecules (apremilast and tofacitinib). Despite the availability of newer therapies such as biologics and oral small molecules, many a time, there is a paucity of treatment options due to the chronic nature of the disease, end-organ toxicity of the conventional drugs or high cost of newer drugs. In these scenarios, unconventional treatment options may be utilized as stand-alone or adjuvant therapy. In this review, we have discussed these uncommonly used treatment options in the management of psoriasis.

A rare case of scrub typhus vasculitis presenting as acute coronary syndrome diagnosed by skin manifestations.

A 48-year-old diabetic man presented with complaints of acute onset chest heaviness with palpitations, anxiety and headache. He had raised troponin-T level and electrocardiogram showed ST elevation myocardial infarction. There was a prior history of fever of 4 days duration with associated abdominal pain. He later developed skin rash and neurological symptoms following admission to the hospital. Dermatological examination revealed purpura and a livedo-like rash. Investigations revealed deranged liver and renal function tests and positive serological tests for scrub typhus. Coronary angiography revealed no evidence of atherosclerosis or any other pathology. He was therefore diagnosed as a case of scrub typhus-induced vasculitis with coronary manifestations and was managed with oral doxycycline. Scrub typhus presenting like an acute coronary syndrome has been reported very rarely previously. In addition, patient had gastrointestinal, central nervous system and hematological involvement which added to the rarity of the case.

Porokeratosis: An enigma beginning to unravel.

Porokeratosis is a keratinization disorder with unclear etiopathogenesis, varied clinical presentation and characteristic histopathology, and is usually unresponsive to current therapeutic options. Until now, it was considered to be a clonal disorder with immunity, ultra violet radiation and other factors playing important roles in etiopathogenesis. It is now known that abnormalities in the mevalonate pathway are responsible for this clonal keratinization abnormality. New variants of porokeratosis like eruptive bullous, pruriginous, lichen planus like, follicular variants and porokeratoma have been described. While the cornoid lamella is the classical histopathologic feature, dermoscopy and reflectance confocal microscopy make the diagnosis clearer. Development of malignancy in a few variants is a concern. Linear, disseminated superficial actinic and giant lesions are most prone to developing malignancies. Bowen's disease, squamous cell carcinoma, basal cell carcinoma and even melanoma have been reported in cases of long-standing porokeratosis. Newer modalities of therapy such as photodynamic therapy, ingenol mebutate and HMGCoA inhibitors may play a role in the future.

Dermatology practice in the times of the COVID-19 pandemic.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is implicated in the ongoing pandemic across the globe since December 2019. It was first notified by China from Wuhan on 31 December 2020 and transmission to healthcare workers was first reported on 20 January 2020. Human-to-human transmission is mainly by droplet infection. At present no effective vaccine is available. Our speciality needs to collectively address the urgent issue of risk of transmission in dermatology practice. A case series of Coronavirus Disease 2019 (COVID-19) from Wuhan described that 41.3% of their patients may have acquired the infection from the hospital. Of all the infected health care workers, 77.5% worked in general wards and departments. These data highlight the significant risk of nosocomial transmission of COVID-19 and also the higher risk in general wards and departments compared to the emergency room or intensive care unit. Dermatology patients are generally seen in clinics and in outpatient departments in hospitals. Patients wait together in the waiting area, intermingle and then are seen by the physician in their chamber. This can cause transmission of the pathogen among patients and from patient to physician. Social distancing, hand hygiene and the use of personal protective equipment are important for preventing the spread of infection and dermatology practices also have to incorporate these aspects. Telemedicine is becoming an important tool for the management of dermatology patients in these times. At-risk patients in dermatology also need to be given priority care. Protocols for the use of immunosuppressants and biologics in dermatology during the pandemic are being developed.

Cytokine gene polymorphisms in type I and type II reactions in Hansen's disease.

INTRODUCTION: Leprosy or Hansen's disease is a chronic debilitating disease caused by Mycobacterium leprae. Host genetics are believed to strongly influence the course of the disease. It is known that cytokines play an important role in leprosy and cytokine gene polymorphisms probably influence the course of the disease. METHODS: In the present study, we evaluated 70 patients with leprosy and 243 controls. DNA was extracted from the peripheral blood and genotyping was done for the following polymorphisms: IL-1 RA intron 2, IL-1ß-511 C/T and TNF-α A/G. RESULTS: A strong association of TNF-α-308 G/A polymorphism with Hansen's disease with both genotypes and alleles was found. However, no correlation was identified between the other two polymorphisms and Hansen's disease. A strong association between the IL-1ß gene polymorphisms and the type of reactions seen in leprosy was found. In contrast, the other two polymorphisms did not show any such association. LIMITATIONS: Genetic polymorphisms are association studies. They are not a direct reflection of the transcriptome or proteome and this is a major limitation of this study. CONCLUSION: In conclusion, cytokine gene polymorphisms appear to influence the susceptibility and course of Hansen's disease. An evaluation of the cytokine levels in the skin during lepra reactions would confirm this observation. Possibly, in future, this would be a guide to therapeutic decisions in cases of lepra reactions.

Livedoid vasculopathy: A review of pathogenesis and principles of management.

Livedoid vasculopathy is a rare cutaneous disease manifesting as recurrent ulcers on the lower extremities. The ulceration results in atrophic, porcelain white scars termed as atrophie blanche. The pathogenesis is yet to be understood with the main mechanism being hypercoagulability and inflammation playing a secondary role. The important procoagulant factors include protein C and S deficiency, factor V Leiden mutation, antithrombin III deficiency, prothrombin gene mutation and hyperhomocysteinemia. Histopathology of livedoid vasculopathy is characterized by intraluminal thrombosis, proliferation of the endothelium and segmental hyalinization of dermal vessels. The treatment is multipronged with anti-thrombotic measures such as anti-platelet drugs, systemic anticoagulants and fibrinolytic therapy taking precedence over anti-inflammatory agents. Colchicine, hydroxychloroquine, vasodilators, intravenous immunoglobulin, folic acid, immunosuppressive therapy and supportive measures are also of some benefit. A multidisciplinary approach would go a long way in the management of these patients resulting in relief from pain and physical as well as psychological scarring.