DRESS, the maverick among SCARS: A case series-based review of literature.

DRESS is a potentially life-threatening severe cutaneous adverse reaction (SCAR). Historically, it was most frequently linked with phenytoin and was initially described as phenytoin hypersensitivity syndrome; however, it was later found to be caused by various other medications, with the commonest been aromatic anticonvulsants, allopurinol and sulfonamides. The severity of this entity is related to systemic involvement, which can result in multiorgan failure and death. The diagnosis of DRESS, especially in the early stages, remains challenging and elusive due to its heterogeneous clinical presentation and the complex course of the disease with different patterns depending on the causal drug. The most important step in the management of DRESS is early diagnosis and immediate cessation of the suspected offending drug along with oral steroids or immunosuppressants to control the disease. We describe the varying presentation and management of six adults with DRESS from a tertiary care hospital, observed over a two-year period with a brief review of the literature.

Awareness of Leprosy in an urban slum of Western Maharashtra Post 35 Years of the National Leprosy Eradication Program (NLEP).

Background: Even after 35 years of the National Leprosy Eradication Program (NLEP) and 15 years post-elimination, leprosy continues to be a public health challenge in India. This paper discusses the current awareness of leprosy among people living in urban slums of western Maharashtra. Methods: The study was conducted in an urban slum of western Maharashtra with 400 participants. A closed-ended questionnaire regarding the knowledge, attitude, practices, and stigma existing among the people was administered, followed by a small awareness talk and screening for leprosy. Results: Of the total 400 participants, 205 (51.25) were females and 195 (48.75) were males. Only 154/400 (38.5%) people were aware of leprosy. 130/400 (32.5%) people thought that it is treatable; however, 71/130 (54.6) of them thought that it would recur even after completing the treatment. Only 103/400 (25.75) said that they would marry a person with leprosy, denoting prevalent stigma in the society, and 79/400 (19.75) were aware of government services for leprosy and NLEP. Screening of all the participants surveyed did not reveal any new or doubtful cases of leprosy. Conclusion: The present study shows a lack of awareness and knowledge of leprosy among the target population. With only 20% of them being aware of government services and the NLEP, combined with an extremely low knowledge about the disease; it shows the need to further augment the government programs. There is also an increasing need to educate people to accomplish a positive attitude of the community towards leprosy patients.

An observational study on ocular manifestations in patients of psoriasis in a tertiary care hospital in western India.

Background: The aim of the study is to observe the ocular manifestation in patients of psoriasis. Methods: All the diagnosed cases of Psoriasis by the dermatology department of this tertiary care hospital were included in this study. Relevant details of the history pertaining to disease duration, type of psoriasis, and treatment undertaken including ocular symptoms were obtained. Disease severity was quantified using the PASI score. Complete ocular examination including intraocular pressure, Schirmer I and II tests, Tear Film Breakup Tme (TBUT); was carried out for all the patients. Results: Of 126 patients of psoriasis, ocular manifestations were seen in 76 patients (60.3%). Dry eyes (27%) and blepharitis (15.9%) were the most common ocular manifestations. Uveitis was seen in 3.2% of the patients of which 75% patients were HA B27-positive psoriatic arthritis, which was statistically significant (p = 0.001). There was no statistical correlation between duration of the disease and ocular manifestations (p value is 0.077 using chi square test). The ocular manifestations were more common in patients with PASI score 10 when compared with the patients with PASI score 10 (p value = 0.028) which was statistically significant. Conclusions: In our study, prevalence of ocular manifestation was 60.3% which increased with the increasing PASI score. Dry eyes and blepharitis were the most common manifestations. Hence, routine ocular examination is recommended in patients with psoriasis.

A case of neurofibromatosis type 2 (NF2) with classic imaging and skin findings.

Neurofibromatosis is a neurocutaneous, tumor predisposing, inheritable disorder characterized by tumors of the brain and spine and the presence of skin lesions. The most important tumors associated with neurofibromatosis are vestibular nerve schwannomas, with others being meningiomas and ependymomas. The cord is also affected by tumors, ependymoma being the commonest, besides meningiomas and schwannomas. We present a case with café au lait macules, neurofibromas in the skin, bilateral VIII cranial nerve schwannomas, multiple meningiomas in the brain and spine, ependymomas in the brain and spine, and schwannomas in the cauda equina nerve roots.

Hyper-Immunoglobulin E Syndrome: Case Series of 6 Children from India.

Hyper-immunoglobulin E syndrome is a rare primary immunodeficiency syndrome characterized by severe atopic dermatitis, recurrent pulmonary and staphylococcal skin infections. Its diagnosis requires a high degree of suspicion, typical clinical features, and not mere rise in serum IgE levels. Genetic studies are not always possible in a resource poor setting in developing countries. In this case series, all children had recurrent eczematoid rash, secondary infections, multiple episodes of hospitalization for pulmonary infection and raised serum IgE levels. Diagnostic genetic study was feasible in only one of the cases which revealed pathogenic homozygous deletions of exons 15 to 18 (Transcript: NM_203447) in DOCK8 gene. The main goal of management of hyper-immunoglobulin E syndrome is aggressive treatment of infections and optimum skin care. Our case series highlights various characteristic, presentations, and management of this rare syndrome childhood cases. Awareness of these manifestations may facilitate early identification and contribute to optimal care of patients as representative data on the same is limited in literature.

Pulseless Systemic Lupus Erythematosus: A Rare Presentation.

Vascular disease is frequent in patients with systemic lupus erythematosus, which can be related to the disease process, or can develop as an accompanying co-morbidity and represents the most frequent cause of death in established disease. However, at times the presentations can be uncommon and subtle, and warrants a thorough examination both clinically and radiologically. We report a case of a young female with photosensitive malar rash, oral ulcers, intermittent fever with joint pains, history of two abortions, and unilateral absent radial and brachial artery pulses on clinical examination. The evaluation revealed positive antinuclear antibody (4+), anti-Smith antibody (2+), direct Coomb's test (2+), and antiphospholipid antibody panel was negative. Color doppler flow imaging of right upper limb (arterial) revealed irregular wall thickening with a narrow lumen and mildly reduced peak systolic volume. Computed tomography aortogram revealed wall thickening and luminal narrowing involving the entire length of the right brachial and radial artery. We report this case for its rarity and unique presentation of medium vessel vasculopathy.

Lepromatous leprosy in a case of epidermodysplasia verruciformis - A coincidence or a result of defective cell-mediated immunity?

Epidermodysplasia verruciformis (EDV) is a rare, autosomal recessive, genetic disorder of immune dysregulation characterized by increased susceptibility to cutaneous human papilloma virus infections. It was first described by Lewandowsky and Lutz in 1922 as a form of epidermal nevus. Its association with skin cancers was proposed by Sullivan and Ellis in 1939. Initial lesions often occur in childhood and are of two types; lifelong eruptions of pityriasis versicolor like lesions and flat wart like papules. The latter is characterized by malignant transformation in 35%-50% of patients especially after 40-50 years of age. Bowen disease is the most common tumor followed by invasive squamous cell carcinoma, however, metastasis is rarely encountered.

Cytomorphological Patterns of Nerve Aspirates in Pure Neuritic Leprosy-A Single Centre Cross-Sectional Observational Study.

BACKGROUND: Pure neuritic leprosy (PNL) poses a diagnostic challenge because of absence of skin patches, inconclusive skin biopsies and nerve conduction studies. Nerve biopsy though the diagnostic gold standard, is invasive, requires expertise, and may not be feasible in all cases. Fine needle aspiration cytology (FNAC) of accessible thickened nerves can be utilized as a minimally invasive diagnostic modality in PNL. This study was carried out to describe cytomorphological patterns of nerve aspirates in patients of PNL for diagnosis and classification of leprosy and study its advantage, if any, over skin biopsy. METHODS: Twenty-seven treatment naive clinically diagnosed patients of PNL were included in this cross-sectional study carried out from January 2017 to December 2018 at a tertiary care centre in Western India. FNAC was done from a clinically involved nerve and aspirates were evaluated for cytomorphological characteristics and the presence of Acid-Fast Lepra bacilli. RESULTS: Nerve aspirates were diagnostic in 10 (37%) patients while 17 (63%) aspirates showed non-specific or no inflammation. Of the diagnostic aspirates, six (22.2%) were classified as tuberculoid leprosy, three (11.1%) as lepromatous and one (3.7%) as borderline leprosy. Mycobacterium leprae were demonstrated among three (11.1%) of these aspirates. In comparison, only three (11.1%) skin biopsies were diagnostic of leprosy with features of indeterminate spectrum. Remaining 24 skin biopsies showed normal histology in 20 (74.1%) cases to perivascular lymphocytic infiltrate in four (14.8%) cases. CONCLUSION: Our study demonstrates that FNAC of clinically thickened nerves has a better diagnostic yield than skin biopsy in PNL and shows all spectrums of leprosy. It also offers the advantage of sampling major nerve trunks without the fear of residual neurological deficit. However, most of the smears were paucicellular and a negative aspirate does not rule out leprosy.

A Rare Presentation of Concurrent Onset and Coexistence of Generalized Lichen Planus and Psoriasis in a Child.

Independently, psoriasis and lichen planus (LP) are common inflammatory skin conditions affecting around 2-3% and 1% of the population, respectively. However, the coexistence of both diseases is rare. Common immunological mechanisms with altered T-cell-mediated autoimmunity, cytokine milieu, and Koebner phenomenon play a role in their pathogenesis. As reviewed in the literature, psoriasis may independently be associated with other autoimmune conditions like vitiligo, alopecia areata, LP, and discoid lupus erythematosus. A rare co-occurrence of these two or three autoimmune diseases has been reported. Here, we report a case of a 10-year-old boy who presented with persistent nonpruritic multiple hyperpigmented scaly papules and plaques over the body since 6 months of age. Histopathology revealed features suggestive of both LP and psoriasis in the same section. A diagnosis of psoriasis-LP overlap was made, and the patient was managed with tab acitretin 25 mg daily and narrowband ultraviolet B (NBUVB) phototherapy with which he showed satisfactory clearance of the lesions.

Pseudoxanthomatous or xanthelasmoid mastocytosis: Reporting a rare entity.

Mastocytosis is a disease characterized by abnormal and pathologic increase in mast cells in the cutaneous tissue and extracutaneous organs such as the bone marrow, liver, spleen, lymph node and gastrointestinal tract. Cutaneous mastocytosis comprises of four major clinical variants: solitary and multiple mastocytomas, urticaria pigmentosa, diffuse cutaneous mastocytosis and telangiectasia macularis eruptiva perstans. Cutaneous mastocytosis of the xanthelasmoid type is a rare variant of diffuse mastocytosis. It is clinically characterized by the typical yellowish hue and is accompanied histologically by mast cells infiltrating far into the lower dermis. Here we report one such rare case.

Anagen Effluvium after Methotrexate: An Idiosyncratic Reaction.

Low-dose methotrexate (MTX) is the backbone of treatment for extensive psoriasis vulgaris not responding to topical therapy. Since its induction for the treatment of psoriasis in the 1970, its short- and long-term side effect profile has been extensively studied. Hair loss with MTX has been reported mostly with high-dose MTX used in chemotherapy. Here, we report a case of guttate psoriasis who presented with acute-onset anagen effluvium following the administration of only two doses of MTX 7.5 mg per week.

A Rare Case of Poikilodermatous Mycosis Fungoides.

Poikilodermatous mycosis fungoides (PMF) is a rare clinical variant of early-stage mycosis fungoides with peculiar histological features and with low risk of disease progression. Since poikiloderma can coexist with classical mycosis fungoides lesions, PMF can only be considered when poikilodermatous lesions are predominant (>50% of lesions). We here report one such rare case of PMF with poikilodermatous lesions covering almost 70% of the body surface and with characteristic clinical, histopathological, dermoscopic, and immunohistochemical findings.

A Rare Case of Lichenoid Sarcoidosis Mimicking Papular Amyloidosis.

Cutaneous lesions in sarcoidosis are seen in nearly 20-30% and include lesions like papules, nodules, plaques, infiltrated scars, and erythema nodosum. Lichenoid variants of cutaneous sarcoidosis, a type of papular variant is quite rare. Herein we report a case of lichenoid sarcoidosis in a 40-year-old male who presented with raised itchy lesions over his legs and lower back of 8 years duration.

A Case Report of Early Disseminated Lyme Disease.

Lyme disease is a multi-organ infectious disease caused by the spirochete "Borrelia burgdorferi," and transmitted by the "Ixodes" tick. Early disseminated Lyme disease can have varied central nervous system manifestations ranging from meningitis to radiculopathy and cranial neuropathy. If not suspected, misdiagnosis or delayed diagnosis can prove to be fatal. Erythema migrans is the most common clinical presentation, thereby, making dermatological examination extremely crucial in early diagnosis and treatment.

Use of Transient Elastography in Detection of Liver Fibrosis in Psoriasis Patients - A Cross- Sectional Study.

BACKGROUND AND AIMS: The risk of liver damage in psoriasis increases with increase in cumulative dose of methotrexate and guidelines suggest use of liver biopsy for risk mitigation. Recently, transient elastography (TE) has been used for detection of liver fibrosis. Most studies for TE are in hepatitis B and C patients. However, psoriasis patients have risk factors like metabolic syndrome which predisposes them to increased risk of liver damage due to methotrexate. This underlying liver disease may change the TE values in patients with psoriasis. The aim of this study is to determine utility of transient elastography in detection of liver fibrosis in patients with psoriasis. METHODS: 82 patients with chronic plaque psoriasis requiring systemic therapy or already on methotrexate were included in the study. Clinical examinationand biochemical investigations were conducted. Data were analysed using STATA 12.1 (Texas, USA). Univariate analysis using Chi-square and independent't-test' was carried out to evaluate the association between categorical variables and outcomes. RESULTS: Patients consists of 62 males and 20 females. TE value >7 kPa (kilopascal) were seen in 23 patients and <7 kPa were seen in 59 patients. Value of >7 kPa was significantly associated with age, waist circumference, diastolic blood pressure, fasting and post prandial blood sugar, AST, PASI and presence of metabolic syndrome. Cumulative methotrexate dose was not significantly associated with high TE value. Mean TE value in patients with metabolic syndrome was significantly higher. LIMITATIONS: Small sample size and inability to confirm TE findings on liver biopsy. CONCLUSION: TE is a non-invasive tool for detection of liver fibrosis. Value of >7 kPa correlates with liver fibrosis in most chronic liver diseases. However, high prevalence of metabolic syndrome in psoriasis patientsmayconfound utility of TE for monitoring of methotrexate toxicity.

A Case of Rhupus-reporting of a Rare Entity.

Rhupus syndrome, a rare entity, is the co-existence of systemic lupus erythematosus (SLE) with rheumatoid arthritis (RA). It manifests as more RA and less SLE related damages. The duration of the disease is longer than typical RA or SLE. Controversies exist regarding the definition of Rhupus. The incidence of Rhupus in patients with arthritis is 0.01%-0.2% and <2% in patients with connective tissue diseases. However, we report a rare case of rhupus in a 55-year-old lady with polyarthritis and joint deformities of 6 year duration, presenting with features of lupus erythematosus.

Immune-mediated tuberculous uveitis - A rare association with papulonecrotic tuberculid.

The tuberculids are a group of distinct clinicopathological form of skin lesions representing hypersensitivity reaction to hematogenous dissemination of Mycobacterium tuberculosis or its antigen from an underlying active or a silent focus of tuberculosis elsewhere in the body in an individual with a strong antituberculous cell-mediated immunity and by definition do not show bacilli on special stains and are culture-negative. Ocular involvement can occur in tuberculosis, both due to direct invasion by the bacilli as well as an immune-mediated reaction; however, immune-mediated tuberculous uveitis occurring as a hypersensitivity response in association with PNT has hardly been reported in the literature. Here we report one such rare case.