Systemic Sclerosis in Males-Deciphering the Enigma of Erasmus Syndrome.

Background: Systemic sclerosis is an autoimmune connective tissue disease characterized by fibrosis in skin and internal organs. Chronic exposure to silica may not only lead to silicosis of lungs but also systemic sclerosis. Systemic sclerosis is relatively commoner in females; however, occupational exposure to silica in males makes them vulnerable to silica--associated systemic sclerosis (Erasmus syndrome). Objective: To describe the clinico-epidemiological aspects of systemic sclerosis in males in a retrospective cohort study. Materials and Methods: The data were analysed retrospectively for demographic profile and clinical characteristics including examination findings, laboratory investigations, and treatments of all male patients diagnosed with systemic sclerosis with or without silica exposure, managed from January 2018 to December 2021. Results: Eight out of twelve patients were having silica exposure in the form of stone cutting, cement exposure, and working with concrete. The average age was 55 ± 10.72 years with average smoking exposure of around 24.4 ± 12.8 pack years. Skin thickening was reported by all patients with an average modified Rodnan score of 18.33/51 in diffuse and 7/51 in limited cutaneous systemic sclerosis. Raynaud's phenomenon and sclerodactyly were universal findings, while 9 (75%) patients had digital pitted scars. Antinuclear antibodies were present in all patients and specific antibodies substantiated the clinical assessment in almost all patients. Interstitial lung disease was the most common systemic finding present in 11 (91%) patients and tuberculosis was diagnosed in 2 (25%) cases with silica exposure. Gastrointestinal and cardiac system involvement was seen in 5 (41.6%) and 4 (33.3%) patients, respectively. Conclusion: Systemic sclerosis in males against the gender predilection indicates the role of occupational exposure. Silicosis and systemic sclerosis synergistically add to lung damage, and at the same time, these patients are more prone to infections like tuberculosis.

The Changing Trend of Syphilis: Is It a Sign of Impending Epidemic?

Background: Syphilis is a treatable bacterial infection caused by Treponema pallidum. There has been a change in incidence of syphilis in various nations over the years. Aim: To study the epidemiological trends, demographic profile, high-risk behaviour, clinical pattern, and stage of syphilis over the last ten years in patients presenting to an STD clinic in a tertiary care hospital. Material and Methods: This was a retrospective observational study over ten years. Records of all confirmed syphilis cases were analysed in relation to demography and clinical profile. Results: There were a total of 3,110 STD patients among whom 31 cases (accounting for 0.99%) of confirmed syphilis were seen. There was a significant increase in cases in the last five years of study, especially in the last year. An increase in primary (PS) and secondary syphilis (SS) was observed. Males outnumbered females (3:1). Mean age of patients was 35.0 ± 11.53 years. Professionals were most common (22.6%) having syphilis followed by farmers (19.35%). A significant proportion (45.1%) of our patients were at least graduates. Unprotected sex was seen in all the patients followed by extramarital/premarital sex (71.35%). There were 16.12% of cases who had a history of paid sex and 9.7% were homosexuals. SS and latent syphilis were more common (38.7% each) than PS (19.35%). In PS single chancre and in SS truncal asymptomatic rash was the commonest clinical presentation. Limitation: Single-centre study, including only self-reported patients leading to a small sample size, is the major limitation of the study. Conclusion: The increased trend of primary and secondary syphilis in recent years highlights that there is a risk of an impending epidemic.

Experience of Rituximab Therapy in Pemphigus: A Three-Year Retrospective Study from a Sub-Himalayan State.

Background: Pemphigus is a group of auto-immune blistering disorders, characterised clinically by mucocutaneous blisters and erosions and histopathologically by intra-epidermal acantholysis. It was traditionally associated with high morbidity and mortality. The use of rituximab has brought upon a new dawn in the treatment of pemphigus. Aim: A retrospective analysis to ascertain the efficacy, tolerance, adverse effect profile, remission, and relapse with the use of rituximab. Material and Methods: A retrospective analysis of all diagnosed pemphigus patients who received rituximab therapy over a period of 3 years was performed. The patient's baseline characteristics, disease duration, clinical presentations, mucosal involvement, disease-severity assessment, and adverse events with rituximab were noted. The outcomes were evaluated based on the definitions of the disease-outcome parameters as early and late endpoints. Results: Of the 17 pemphigus patients, there were 14 females (82.4%) and three males (17.6%) with a mean age of 35.9 ± 16.5 years (range: 9-65 years). Pemphigus vulgaris (PV) was the predominant type in 11 (64.7%) patients. After rituximab infusion, the 17 patients attained the end of consolidation phase (ECP) within 15 days to 3 months, and the mean duration was 1.24 months. The complete remission (CR on/off) ranged from 0.5 to 35 months, and the mean duration of remission was 21.7 months. Within a median time of 4.2 months, almost 80% patients achieved CR on therapy. Nine (53%) patients were in CR without any therapy till the end of the study period, and eight (47%) were in remission while on minimal therapy. Conclusion: Rituximab is an efficacious therapeutic agent for pemphigus and is better tolerated and safer to all the previous medications used in the treatment.

Delineating Injectable Triamcinolone-Induced Cutaneous Atrophy and Therapeutic Options in 24 Patients-A Retrospective Study.

Background: Steroids being the strongest anti-inflammatory agents are used in innumerable disorders in various formulations with excellent results and seemingly known side effects as well. Triamcinolone acetonide used as intralesional injections is seen to be associated with localized atrophy in some patients. Aim: To describe the cases of steroid-induced localized atrophy/lipoatrophy after intralesional triamcinolone over various parts of the body in a retrospective study. Materials and Methods: All patients, with localized atrophy/lipoatrophy with a history of intralesional triamcinolone, were evaluated clinically and histopathologically over the last 3 years. Patients with localized atrophy/lipoatrophy without a history of intralesional steroids were excluded from the study. Patients were evaluated for number, duration, sites, size, shape, and morphology of lesions and response to treatment. Results: There were 24 patients (13 females and 11 males) who had intralesional steroid-induced atrophy/lipoatrophy.All but one patient (4-year-old male child) were adults. Buttock (50%) was the most common site involved followed by wrist (25%), scalp (16.6%), malleolus, and neck (4.1%) each. The most common presentation was asymptomatic depigmented atrophic single oval or ameboid plaque with radial extensions. Histopathology was done in 10 patients showing diminished subcutaneous fat lobules with minimal inflammatory cells. Sixteen patients (66.6%) improved with medications (tacrolimus, platelet-rich plasma, and saline injections), and seven were lost to follow-up. Conclusion: Corticosteroids act as a double-edged sword so should be used cautiously. Depigmentation/atrophy is a peculiar side effect of intralesional triamcinolone. Depigmented lesions with minimal clinical atrophy respond well to topical tacrolimus, while normal saline injections appear to have promising results in steroid-induced lipoatrophy.

A Clinicoepidemiological Study of Psychiatric Co-Morbidity in Hansen's Disease.

INTRODUCTION: Leprosy is a chronic disease caused by mycobacterium leprae. Chronicity of the disease leads to various psychiatric co-morbidities, which are often undiagnosed and untreated. The main objectives of this study were to evaluate the frequency and nature of psychiatric co-morbidity in patients of Hansen's disease. MATERIALS AND METHODS: Seventy confirmed cases of Hansen's disease between the ages of 18 and 60 years attending the department of dermatology of Indira Gandhi Medical College and Hospital, Shimla were evaluated for various manifestations of Hansen's disease and screened for psychiatric co-morbidity using GHQ-12 and diagnosed using ICD-10 checklist. Severity of depression and anxiety was assessed with HAM-D and HAM-A scales, respectively. RESULTS: The prevalence of psychiatric co-morbidity was found in 27.14% of enrolled patients. Depression was most prevalent in (20%) mental disorder; followed by anxiety disorder (7.14%). Moderate depressive episode was the commonest seen in 11.42% patients whereas 5.71% patients had mild depression. Generalized anxiety disorder was seen in 1.42% whereas 5.71% patients had mixed anxiety disorder. Patients with higher education status, positive family history of leprosy, lepromatous leprosy, and hand deformities were significantly associated with higher psychiatric co-morbidity. CONCLUSION: Patients suffering from Hansen's disease have significantly high prevalence of psychiatric co-morbidity complicating the Hansen's disease. High index of suspicion is required to diagnose and treat it.

Hypokalaemic quadriparesis with respiratory failure due to latent Sjogren syndrome.

Sjogren's syndrome (SS) is an autoimmune disease with involvement of multiple organs, including both glandular and extraglandular organs. Usually involvement of glandular organs manifests before the extraglandular ones, but when the sequence is reversed, diagnosis may be missed. Hypokalaemic quadriparesis in SS is not uncommon. Respiratory failure in hypokalaemia is not usually seen, but in SS, it has been reported. We report a case of a 55-year-old woman who presented with sudden onset flaccid quadriparesis and respiratory muscle paralysis secondary to severe hypokalaemia. On detailed investigation, she was detected to have distal renal tubular acidosis secondary to clinically inapparent and asymptomatic SS.

Post Herpetic Neuralgia: A Retrospective Study to Evaluate Response to Modified Jaipur Block with Increased Concentration of Dexamethasone.

BACKGROUND: Postherpetic neuralgia (PHN) is a complication of herpes zoster characterized by persistent dermatomal pain. It has a negative impact on the quality of life. There is no gold standard therapy for PHN, and various local and systemic treatments have been tried. There are studies reporting the use of combination of steroids and local anesthetics but there is no standardized method. AIM: To evaluate the response of modified Jaipur block with increased concentration of dexamethasone. METHODS: We conducted a retrospective study in patients who were given Jaipur block. The patients age, sex, duration of PHN, type and severity of pain were observed. A combination of 2% lignocaine and 0.5% bupivacaine and dexamethasone was injected subcutaneously. The pain was scored using visual analogue scale at the baseline, and 1 month after 1st, 2nd, and 3rd session of block and follow up after 6 months and 1 year. RESULTS: The mean age of our patient was 63.33 ± 9.5 years. The males outnumbered females. Thoracic dermatomes were more commonly involved. The mean duration of PHN was 11.58 ± 12.76 months; stimulus evoked PHN was the commonest type of pain seen. The mean visual analogue score (VAS) decreased progressively after each session of the block. Maximum patients (50%) had excellent response, whereas 1.9% did not respond to the block. Relapse of pain was seen in 5.6% of the patients. There was no significant side effect noted. LIMITATIONS: There was no objective method used to assess pain. CONCLUSION: PHN is chronic neuropathic pain. Response to modified Jaipur block is good, but if the duration of PHN is more, the recurrence rate is higher. Modified Jaipur block is an effective and safe treatment for PHN.

Linear perioral atrophoderma along blaschko's lines- a retrospective study describing 14 cases of atrophia maculosa varioliformis cutis.

BACKGROUND: Spontaneous atrophic scarring is characterised by an idiopathic, non-inflammatory macular atrophy that typically occurs on the face and presents as shallow atrophic scars having sharp margins and may be linear, rectangular or varioliform. AIM: To describe the cases of spontaneous atrophic scarring over perioral region of face having specific feline band pattern in a retrospective study. MATERIALS AND METHODS: All patients with facial atrophoderma (perioral region) were evaluated clinically and histopathologically in tertiary care centres over 3 years. Patients with facial atrophoderma but no perioral involvement and secondary atrophoderma were excluded from the study. Patients were evaluated for number, sites, size and shape of lesions and were confirmed histopathologically. RESULTS: There were 14 patients (10 females) with facial atrophoderma particularly over the perioral region. Three patients had perioral involvement with a few lesions on the cheeks and forehead. All patients developed atrophoderma spontaneously without preceding inflammation. Most of the patients were asymptomatic except for mild pain or burning at the time of development of atrophic lesions; however, none of the patients were symptomatic at the time of presentation. Histopathology in 5 patients showed epidermal and upper dermal atrophy with no/minimal signs of inflammation. CONCLUSION: Idiopathic atrophodermas over the face can be due to spontaneous atrophia maculosa varioliformis cutis or atrophoderma of Moulin.

A Study to Evaluate the Role of Intradermal and Intralesional Measles, Mumps, Rubella (MMR) Vaccine in Treatment of Common Warts.

BACKGROUND: Warts are common cutaneous viral infection with a wide range of therapeutic modalities. Various agents have been tried for immunotherapy in warts. OBJECTIVES: Determine the role of intralesional and intradermal measles, mumps, rubella (MMR) vaccine in the treatment of common warts; to compare the efficacy of intralesional versus intradermal MMR vaccine. METHODS AND MATERIALS: Patients diagnosed with verruca vulgaris were divided into two groups. In study group A, the individuals were injected with an intralesional MMR vaccine of 0.3 mL in the representative wart (largest) once in 3 weeks till there is complete clearance or maximum of four injections whichever is earlier, while in study group B, the individuals were injected with an intradermal MMR vaccine of 0.3 mL over the unilateral deltoid muscle area at similar intervals. RESULTS: There were 33 patients in each group. In group A, 10 (30.3%) patients showed complete, 9 (27.3%) marked, 6 (18.2%) moderate, 3 (9.1%) mild, and 5 (15.2%) no response. In group B, seven (21.2%) patients showed complete, one (3.0%) marked, one (3.0%) moderate, four (12.1%) mild, and 20 (60.6%) no response. There were minimal side effects in the form of pain, erythema, itching at the injection site in a few patients, only one patient had syncope. CONCLUSION: We conclude that the MMR vaccine is an effective and safe modality of treatment for verruca vulgaris without any serious adverse effects. Also, the intralesional route showed better results in comparison to the intradermal route when we consider the treatment of a representative wart.

Spectrum of Cutaneous Adverse Drug Reactions to Anti-tubercular Drugs and Safe Therapy after Re-challenge - A Retrospective Study.

BACKGROUND: Tuberculosis (TB) is a major global health problem and leading cause of death. Anti-tubercular therapy (ATT) can lead to various adverse effects including cutaneous reactions. Re-challenge remains the only option to restart the safe therapy with limited number of most efficient primary ATT drugs. OBJECTIVES: To study the demographic profile, identify the spectrum of cutaneous eruptions, offending drug and the reinstitution of safe ATT. MATERIALS AND METHODS: This was a retrospective study with inclusion of the indoor patients with cutaneous adverse drug reaction secondary to ATT. Hospital records were analyzed regarding demographic characteristics, type of TB, ATT regimen, pattern of drug rash, offending drugs, laboratory parameters, and reinstitution of ATT after re-challenge. RESULTS: All the cases (40 patients) were reported in adults with male to female ratio of 1:1.2 and mean age of 50 years. Pulmonary TB was the most common type of TB observed in 24 (60%) patients followed by extra-pulmonary in 16 (40%) patients. Maculopapular rash was the most common (42.5%) type of cutaneous eruptions and ethambutol, the most common (45%) offending drug followed by other first line anti-tubercular drugs. Ten (25%) patients developed multiple drug hypersensitivity on re-challenging. Multiple drug hypersensitivity was seen in 10 (25%) patients. CONCLUSION: Drug reaction to ATT is like a double-edged sword as stopping ATT and starting treatment of reaction with systemic steroids can further aggravate the condition with increased risk of disseminated and multidrug resistant tuberculosis. Re-challenge with ATT not only find out the culprit drug but also helps to restart a safer alternate ATT regimen. LIMITATIONS: Small sample size, lack of proper hospital records due to which some patients were missed and the fact that re-challenge was not performred in mild lichenoid type rash.

Diffuse normolipemic plane xanthoma with underlying photosensitive dermatoses.

Diffuse plane xanthomatosis is a normolipemic non-Langerhan cell histiocytosis. Clinically, patient presents as asymptomatic yellowish plaques involving eyelids, neck, upper trunk and flexures. Usually, there is an associated haematological or lymphoproliferative malignancy which may precede or follow dermatological manifestations by many years. Rarely inflammatory dermatosis like atopic eczema, photosensitive dermatosis, erythroderma, previous scars and lymphoedema have been found to be associated with these xanthomas. We are reporting a 46-year-old male patient who had a history of photosensitivity for the last 15 years and subsequently developed diffuse plane normolipemic xanthomas.

Idiopathic Localized Involutional Lipoatrophy: A Retrospective Study of 12 Cases.

BACKGROUND: Idiopathic localized involutional lipoatrophy (ILIL) is focal loss of subcutaneous tissue without any clinical or histopathological inflammation with spontaneous regression. OBJECTIVE: To retrospectively study clinical features and evolution of lesions in patients diagnosed with idiopathic localized lipoatrophy presenting to the department of dermatology of two district hospitals of Himachal Pradesh. MATERIALS AND METHODS: A retrospective study of clinical patterns and evolution of ILIL was done in patients presenting with this condition in two district hospitals in the past 4 years (October 2013-September 2017). All clinically suspected and histopathologically confirmed cases of idiopathic localized lipoatrophy were included in the study. All cases with history of antecedent injections, vaccination, or medications before the development of lesion and inflammatory lipoatrophy on histopathology were excluded. RESULTS: We found a total of 12 patients with ILIL. About 66% were children (8/12), 3 (25%) young females, and 1 (8%) young adult male. The most common site involved was buttock in 9 (75%) cases followed by a single case each (8%) with lesion on arm, face, and lower back. Two children and one adult were having bilateral involvement (25%), whereas the remaining had unilateral lesions. Lipoatrophy in 8 (66%) patients decreased spontaneously (with placebo) within 4-12 weeks duration whereas 4 required treatment. None required surgical or cosmetic interventions. LIMITATIONS: Small sample size and nonavailability of immunohistochemistry reports in all patients. CONCLUSION: ILIL is a rare form of lipoatrophy with specific loss of adipose tissue without any inflammatory changes. We concluded that ILIL is an underreported entity, probably due to its spontaneous resolution.

A Study to Compare the Efficacy of Platelet-rich Plasma and Minoxidil Therapy for the Treatment of Androgenetic Alopecia.

BACKGROUND: Androgenetic alopecia (AGA) is the most common cause of hair loss in men with limited treatment options. Platelet-rich plasma (PRP) therapy is one of the newer treatment options in the management of AGA which has shown promising results. AIMS AND OBJECTIVES: This study was aimed at comparing the clinical efficacy of PRP therapy with minoxidil therapy. MATERIALS AND METHODS: In the study, patients were randomized into two groups - Group A (given PRP therapy) and Group B (given minoxidil therapy). Both groups were followed up over a period of 6 months, and final analysis was done with the help of global photography, hair pull test, standardized hair growth questionnaire, patient satisfaction score; in addition, a comparison of platelet counts in PRP was done, to know that if a clinical correlation exists between platelet concentration and clinical improvement. A total of 40 patients clinically diagnosed with AGA were enrolled in the study with 20 patients in each group. Four patients from Group A (PRP) and six patients from Group B (minoxidil) could not complete the treatment for 6 months and were eventually excluded. RESULTS: At the end of 6 months, 30 patients were evaluated to compare the efficacy of intradermal PRP and topical minoxidil therapy. On global photography, Group A (PRP) was found to have a comparatively better outcome than Group B (minoxidil). In hair pull test, hair growth questionnaire, and patient satisfaction score, Group A was found to be better than Group B. Mean platelet count at baseline was 3.07 ± 0.5 lac/mm, 3 while platelet count in final PRP prepared was 12.4 ± 1.7 lac/mm, and patients with a higher platelet count in PRP had a much better clinical improvement compared to patients with a low platelet count in PRP. Side effects with PRP therapy were minimal with better results which may improve the compliance of the patient. CONCLUSION: PRP therapy can be a valuable alternative to topical minoxidil therapy in the treatment of AGA.

Parkes Weber syndrome presenting as Stewart-Bluefarb acroangiodermatitis.

Parkes Weber syndrome (PWS) is a rare disorder characterised by arteriovenous (AV) fistula, along with capillary, lymphatic, venous malformations and limb hypertrophy. Stewart-Bluefarb syndrome is a variant of acroangiodermatitis, which is associated with congenital AV malformation/fistulas. It usually begins early in life, unilaterally over lower extremities presenting as violaceous to dusky coloured macules, papules or plaques with tendency to ulcerate. We are reporting a case of AV malformation fulfilling the triad of PWS and presenting with acroangiodermatitis.

Giant and Extensive Localized Xanthoma Associated with Primary Lymphedema.

Lymphedema is extravasation of lymphatic fluid into extracellular spaces and may be primary or secondary. Primary lymphedema may be caused by truncular lymphatic malformation. Long-standing lymphedema may result in various complications due to increased porosity of lymphatics, defective valves, and increased lymphatic pressure. There are few reports of normolipidemic xanthoma in association with lymphedema. We report a case of giant xanthoma associated with primary lymphedema.

Aggressive Digital Papillary Adenocarcinoma at Atypical Site.

Aggressive digital papillary adenocarcinoma (ADPA) is a rare tumour of eccrine origin. They present as asymptomatic nodules with flattened and thickened surface skin, most commonly on fingers and toes. It mimics many benign entities and tumours, hence leading to a delay in diagnosis. It has a high metastasizing potential, but is slowly progressive. Histopathologically, it has both a nodular and a cystic component with papillary projections into cystic spaces. We present a 54-year man with a tumorous mass on right heel, which was diagnosed by histopathology and immunohistochemistry as ADPA. Our case had ADPA lesion, a rarely reported malignancy on an uncommon site with distant metastasis.