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.

Delineating the Boundaries of Superficial Lymphangitis: A Retrospective Study of 11 Cases with a Review of Literature.

Background: Lymphangitis is an inflammation of lymphatic channels caused by infectious or non-infectious agents, presenting with characteristic linear erythematous streaks draining toward regional lymph nodes. Objective: To describe the clinical characteristics and etiological factors involved in acute superficial lymphangitis in a retrospective descriptive study. Materials and Methods: Records of patients were analyzed retrospectively who presented with linear erythematous streaks, diagnosed as superficial lymphangitis, in the outpatient department of dermatology during the last 5 years (January 2018-December 2022) in a tertiary care hospital. Patients were evaluated for their demographic profile, detailed history, complete physical examination, and standard blood tests (if necessary). Results: A total of 11 patients were found, out of which 7 (63%) were males and 4 (37%) were females. The mean/median age of these patients was 30 years (range 9-52 years). The minimum duration of development of lymphangitis was within minutes in the case of a mosquito bite reaction and around 72 hours in the case of trauma or infection induced, with a median interval of 48 hours. The site most commonly involved was the upper extremity in 8 (72%) patients, followed by the trunk in 2 (18%) and the lower extremity in 1 (9%). Arthropod bite reactions (63%) were the most common etiological agent. All patients presented with linear erythematous streaks extending towards draining lymph nodes. Conclusion: Lymphangitis is often considered to be a bacterial infection and is mostly treated with antibiotics; however, non-bacterial and non-infectious causes should be kept in mind while treating superficial lymphangitis to make judicious use of systemic antibiotics.

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.

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.

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.

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.

Erasmus Syndrome: Association of Silicosis and Systemic Sclerosis.

Silicosis is an inflammatory disease of the lung characterized by irreversible lung fibrosis which develops from prolonged pulmonary inhalation and retention of crystalline silica and immune reaction. It mainly appears as an occupational hazard in persons involved in stone-quarrying, mining, and sand blasting. Crystalline silica is not only known to be responsible for silicosis but also for other autoimmune diseases including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA)-Caplan syndrome, systemic sclerosis (SSc), and antineutrophil cytoplasmic antibody (ANCA)-related vasculitis. Erasmus syndrome is the association of silica exposure and subsequent development of SSc. The limited numbers of cases reported in the literature were miners and only sporadically involved in other professionals. Here, we report a case of a 52 -year-old stone cutter who developed silicosis and SSc after 25 years of exposure.