A case of recurrent steroid-dependent severe type 2 lepra reaction treated successfully with colchicine.

Type 2 lepra reaction (or erythema nodosum leprosum [ENL]) is an immune complex-mediated reaction which has been reported to occur for up to 8 years after successful completion of multibacillary multidrug therapy. The management of ENL is a challenge with the patient often becoming steroid dependent; predisposing to recurrent episodes in 39%-77% of cases on attempted steroid withdrawal. Here, we present a case of recalcitrant recurrent type 2 reaction which responded promptly to treatment with colchicine.

Case Report: Rapid Response to Low-Dose Thalidomide in a Case of Severe Steroid Recalcitrant Erythema Nodosum Leprosum.

Erythema nodosum leprosum (ENL), or type 2 lepra reaction, presents with crops of evanescent, tender erythematous nodules accompanied by fever, arthralgia, malaise and organ-specific manifestations and is seen in borderline and lepromatous leprosy. The drugs approved for ENL include nonsteroidal anti-inflammatory drugs, systemic steroids, thalidomide and clofazimine. The management of ENL is challenging because long-term steroid use leads to steroid dependence. The present patient had severe steroid recalcitrant ENL with vesicular and pustular lesions mimicking Sweet's syndrome and was treated effectively with a low-dose thalidomide regimen (100 mg/d) as opposed to high dose (400 mg/d) recommended in literature. We discuss the patho-mechanics and clinical utility of a low-dose thalidomide regimen as an effective treatment option for ENL.

Multidrug resistance as a cause of steroid-nonresponsive downgrading type I reaction in Hansen's disease.

While Type 1 reaction in Hansen's disease is commonly encountered, the triggers and reasons for its persistence are not well understood even though the immunological milieu and cytokine interplay have been studied. Herein, we present a case of Type 1 downgrading reaction in which multidrug resistance was the probable cause of steroid-nonresponsiveness and which responded promptly on starting alternate antileprosy treatment.

A cavernous sinus lesion clinically responsive to steroids.

Tolosa Hunt syndrome (THS) is characterized by painful ophthalmoplegia secondary to idiopathic granulomatous inflammation of the cavernous sinus. The characteristic finding on MRI is an enhancing T1 isointense and T2 hypo- or hyperintense cavernous sinus mass lesion, which may result in focal narrowing of the ipsilateral internal carotid artery. Although the incidence is quite rare, it is a common diagnostic consideration in cases that present with multiple cranial neuropathies. However, the differential diagnosis for a unilateral cavernous sinus lesion in adults is broad and includes neoplastic, inflammatory (such as sarcoidosis and immunoglobulin G4-related disease [IgG4-RD]), infectious etiologies (such as syphilis and leprosy), as well as vascular lesions. We describe a patient presenting with neurologic symptoms referable to a persistent unilateral cavernous sinus MRI abnormality, initially thought to be consistent with Tolosa-Hunt syndrome, that was clinically but not radiographically responsive to steroids. Following reevaluation due to the presence of new symptoms, pathology revealed that the abnormality was most consistent with chordoma, a rare skull based tumor. In patients with a presumed diagnosis of Tolosa-Hunt syndrome, close clinical and radiographic follow-up is imperative, with early consideration for biopsy in patients that fail to respond to treatment both clinically and radiographically.

Hansen's Disease and Rheumatoid Arthritis Crossover of Clinical Symptoms: A Case Series of 18 Patients in the United States.

Hansen's Disease (HD) is a rare, chronic granulomatous infection of the skin and peripheral nerves caused by the noncultivable organism Mycobacterium leprae. Arthritis is the third most common symptom of HD. Subjects with both confirmed HD on skin biopsy and chronic arthritis were identified at the National Hansen's Disease Program (NHDP). We conducted a series of medical chart reviews and extracted and logged personally deidentified data into a database and carried out descriptive analyses. Eighteen of 261 subjects presented to the NDHP with both HD and chronic arthritis between 2001 and 2015. Among these, 16 were male, 16 were white, and 15 were residents of Louisiana. The median age at diagnosis of HD was 67 years. Ten of these subjects were diagnosed with borderline lepromatous leprosy, seven were diagnosed with lepromatous, and one was diagnosed with borderline tuberculoid leprosy. Patients were symptomatic with arthritis for a median of 5.3 years before HD diagnosis. Sixty-two percent of patients (11) were diagnosed with rheumatoid arthritis (RA) before HD diagnosis, and 10 of which were seronegative RA. Hands, feet, wrists, and elbows were most commonly reported as affected joints. Over half of the patients (61%) had completed HD multidrug therapy at the time of review, and 73% of these subjects had persistent joint pain requiring steroids or methotrexate for symptomatic control. Chronic arthritis in HD patients is present in a series of US-acquired cases of HD. Arthritis did not resolve with successful treatment of HD in most cases.

Efficacy of Steroid and Physiotherapy in Early Reported Lagophthalmos of Patient Affected with Leprosy. A Report from Trbal Dominated Leprosy Endemic State of India.

Lagophthalmos is one of the well-known complications of leprosy due to involvement of the facial nerve. In the present study an attempt has been made to elucidate the role of early intervention with steroids and adjunct physiotherapy in early reported lagophthalmos in patients affected with leprosy at a tertiary referral institute under the Disability Prevention and Medical rehabilitation (DPMR) programme. During April 2008 to March 2014, 62 patients affected with leprosy reported to Regional leprosy Training and Research Institute with lagophthalmos. Cases reporting within six months of difficulty in closure were categorized as early reporting group. These were either referred from a peripheral health centre (63%) or self-reported (37%). These patients were examined clinically and details were noted in a pretested Performa. The standard dosages of the steroids were given to patients as per NLEP guidelines. Lid gaps on direct gaze and with both gentle and forced closure were assessed using standard measuring technique by a physiotherapist. During the follow-up period the patients were imparted active and passive physiotherapy and any change in the lid gap was recorded. The data was analysed and appropriate test of significance was applied. Out of 62 lagophthalmos patients, 49(79.1%) were males and 13 (20.9%) were females. 56 (90.3%) cases were from MB category and 6 (9.7%) cases were from PB category. 53(85.4%) patients presented with unilateral eye involvement, while 9 (14.6%) had bilateral lagophthalmos. 53 (74.6%) of the eyes achieved complete lid closure, while the remaining 18 (25.4%) eyes had gap on gentle closure. During six month follow-up the amount of recession of lid gap among the early reported lagophthalmos was 4.15 mm with standard steroid regimen and physiotherapy. With the use of the steroid and regular physiotherapy lagophthalmos diagnosed and treated in initial stages shows significant improvement in the lid gap reduction.

Mononeuritis multiplex and painful ulcers as the initial manifestation of hepatitis B infection.

Hepatitis B virus infection leads to multisystem manifestations owing to involvement of kidney, skin, vasculature, haematopoietic and nervous system. The hepatitis B infection can cause neuropathy either to vasculitis associated with polyarteritis nodosa or immune-mediated neural damage. In this submission, we report a young woman, who presented with mononeuritis multiplex and painful ulcerations as the first manifestation of chronic hepatitis B virus infection. The antiviral therapy along with steroids led to remarkable recovery. The clinical settings of hepatitis B virus infection should not be ignored in the presentation of mononeuritis multiplex with ulcers, although the commonest cause is leprosy in the Indian sub-continent.

Behçet's disease in India: a dermatological perspective.

BACKGROUND: Behcet's disease (BD) is a chronic, recurrent, multi-system inflammatory disorder involving mucocutaneous (MC), ocular, intestinal, articular, vascular, urogenital and neurologic systems. BD occurs with a high prevalence in the Mediterranean population. There is scarcity of clinical data on BD from India with only three case series in the last two decades. AIMS: To study demographic profile, clinical manifestations and treatment outcome of patients with BD presenting to the dermatologic clinic in a tertiary hospital in north India. METHODS: Prospective analysis of all patients diagnosed to have BD between 1997 to 2011. RESULT: Twenty nine patients were diagnosed to have BD. The disease had a female preponderance (M:F = 1:3.8) with a mean age of disease onset of 27.4 (range 16-61) years. The prevalence of various MC and systemic manifestations are as follows: oral aphthae (100%), genital aphthae (93.1%), erythema nodosum (62%), papulopustular and acneiform lesions (31%), articular involvement (68.9%), ocular involvement (31%) and gastrointestinal (GI) involvement (3.4%) . Pathergy test positivity was observed in 31%. The treatment comprised of colchicine (16/29 patients), dapsone (7/29), dapsone with pentoxiphylline (3/29), systemic steroid (2/29), systemic steroid with methotrexate (1/29). Colchicine was effective and well tolerated in all patients. CONCLUSION: The disease occurs in a much milder form in India and is primarily mucocutaneous and arthritic. A high index of suspicion in patients with MC lesions may result in early diagnosis, management and prevention of complications of BD. We suggest colchicine as an effective and safe therapeutic option for MC and joint involvement.

Decompression of peripheral nerve trunks in leprosy prevents the development and progression of deformities?

Peripheral nerve involvement results in deformities formation in leprosy. High doses of (40-60 mg) steroids along with the anti-leprosy drugs is preferred even though the 70-75% cases develop deformity with the above treatment. 772 ulnar nerves, 120 median nerves and 108 posterior tibial nerves not responding to above medical treatment in 12 weeks, were undertaken for external and internal nerve trunk decompression. These cases were followed-up for 5-20 years at various intervals. The pain in nerves (neuritis) recovered in all cases of ulnar, median and posterior tibial nerves. Full sensory recovery with pin prick/feather or cotton wool touch was seen in 50% cases of all the three nerves. 20% cases maintain the pre-operative levels of sensory status. Plantar ulcers healed within 6 months after decompression of posterior tibial nerve. Only 6 cases showed reoccurrence. Overall motor recovery in ulnar nerve was seen 89% and 70% in median nerve. The sensory recovery restores protective sensation which prevents secondary injuries. The improvement of motor power gave better functional hands and improved the appearance which in absence of surgical intervention was not possible.

A study of standardized regimens of steroid treatment in reactions in leprosy at a referral centre.

It is a well known fact that reactions and nerve function impairment (NFI) account for majority of disabilities and morbidities in leprosy. Steroids are the principal agents administered for treatment of reactions and NFI. In this study, we compared the efficacy of two regimens namely high dose (60 mg) regimen tapered over 28 weeks and low dose (40 mg) regimen tapered over 22 weeks in treatment of reactions and early NFI as regards to incidence of recurrence of reactions in 209 patients. Concerns about the side-effects of steroids were also addressed byfollowing a strict pre-steroid investigation protocol. We observed that the low dose regimen was associated with a higher incidence of recurrence (48.3%) as compared to high dose regimen (16%) signifying the efficacy of longer duration of therapy with a higher starting dose. No patient developed side-effects which necessitated withdrawal of steroids. Early detection with prompt and adequate therapy with proper dose and duration is the key to reduce recurrence of reactions and to minimize deformities due to reactions and NFI in leprosy.

Hand eczema: an update.

Eczema, the commonest disorders afflicting the hands, is also the commonest occupational skin disease (OSD). In the dermatology outpatient departments, only the severe cases are diagnosed since patients rarely report with early hand dermatitis. Mild forms are picked up only during occupational screening. Hand eczema (HE) can evolve into a chronic condition with persistent disease even after avoiding contact with the incriminated allergen / irritant. The important risk factors for hand eczema are atopy (especially the presence of dermatitis), wet work, and contact allergy. The higher prevalence in women as compared to men in most studies is related to environmental factors and is mainly applicable to younger women in their twenties. Preventive measures play a very important role in therapy as they enable the affected individuals to retain their employment and livelihood. This article reviews established preventive and therapeutic options and newer drugs like alitretinoin in hand eczema with a mention on the etiology and morphology. Identifying the etiological factors is of paramount importance as avoiding or minimizing these factors play an important role in treatment.

Acroangiodermatitis of mali: a rare vascular phenomenon.

Acroangiodermatitis (synonym pseudo-Kaposi sarcoma) is an unusual, benign condition which clinically presents as purple-colored patches, plaques or nodules, mostly on the extensor surfaces of lower extremities in patients with chronic venous insufficiency and arteriovenous malformations. It resembles aggressive conditions like Kaposi's sarcoma and requires histopathological examination for its diagnosis. We report two such cases of acroangiodermatitis. Histopathology of both the cases showed dilated capillaries in the dermis with extravasated red blood corpuscles (RBCs), hemosiderin deposits, and hyperplastic granulation tissue. Both were treated with oral antibiotics and topical steroids. The ulcers showed a good response within 2 months of treatment.

Multicentric reticulohistiocytosis.

A 50-year-old male presented with fever, joint pain and skin lesions since eight months. Examination showed multiple papules and nodules with periarticular predisposition. Swelling of knees and elbows with flexion deformity of distal interphalangeal joints was present. Investigations revealed anemia and raised ESR. Histopathology was pathognomonic of multicentric reticulohistiocytosis. Patient was treated with bisphosphonates along with systemic steroids and methotrexate to which he responded well.