Neurolymphomatosis in Primary Cutaneous CD4+ Pleomorphic Small/Medium-sized T-cell Lymphoma Mimicking Hansen's Disease.

Neurolymphomatosis (NL) refers to nerve infiltration by neurotropic neoplastic cells in the setting of a known or an unknown hematological malignancy. It typically presents as painful or painless peripheral mononeuropathy, mononeuritis multiplex, polyneuropathy, polyradiculopathy, or cranial neuropathy. A 32-year-old male presented with a hyperpigmented hypoesthetic plaque over the anterolateral aspect of the right leg with thickening of the right common peroneal nerve and foot drop clinically diagnosed as Hansen's disease. Biopsy taken from skin showed infiltrates of pleomorphic small and medium sized lymphocytes in the dermis and subcutis. On immunohistochemistry, the cells were positive for CD3, CD4 and negative for CD8, CD20, and CD30. Ultrasonography-guided fine-needle aspiration of the thickened nerve showed infiltrates of atypical lymphoid cells. Based on these findings, a diagnosis of NL in primary cutaneous CD4+ pleomorphic small/medium-sized T-cell lymphoma was made. The disease responded to systemic chemotherapy and localized radiotherapy with no evidence of relapse during 3 years follow-up. NL in primary cutaneous CD4+ pleomorphic small/medium-sized T-cell lymphoma presenting with manifestations redolent of Hansen's disease is not described in available literature. This case also demonstrates the utility of fine needle aspiration of nerve, a minimally invasive procedure in the diagnosis of NL.

Severe cutaneous adverse drug reactions: a clinicoepidemiological study.

BACKGROUND: Drug eruptions range from transient erythema to the life threatening severe cutaneous adverse reactions (SCAR) that encompass Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), acute generalized exanthematous pustulosis (AGEP) and drug reaction with eosinophilia and systemic symptoms complex (DRESS). AIMS AND OBJECTIVES: To study the clinical and epidemiological aspects of cutaneous adverse drug reactions (CADR). MATERIALS AND METHODS: Ethical clearance was obtained from the institutional ethics committee. All patients admitted in the Dermatology ward of our tertiary care hospital with CADR (those who fit in the category of probable or possible drug reaction as per WHO casuality assessment) from first September 2011 to 31(st) August 2012 were included in this cross sectional study after obtaining written informed consent. The drug reaction patterns observed in the study population were determined and the common offending drugs were identified. RESULTS: In the study, population of males outnumbered females and the majority were between 46 and 60 years of age. The commonest reaction pattern observed was SJS- TEN spectrum of illness and aromatic anticonvulsants were the common offending drugs. Prompt withdrawal of the culprit drug and administration of systemic steroids with or without I/V Ig reverted the adverse reaction in all except one. CONCLUSION: Severe drug reactions predominated as the study population was comprised of inpatients of a tertiary referral centre. Though; previous authors had reported a mortality rate of up to 20% in DRESS, all our patients with this reaction pattern, responded well to treatment. The mortality rate among TEN cases was much lower than the previous reports. Early diagnosis, prompt withdrawal of the suspected drug, careful monitoring for development of complications and immediate intervention can improve the prognosis of CADR.

Multifaceted adult T-cell leukemia/lymphoma in India: a case series.

BACKGROUND: Adult T-cell leukemia/lymphoma (ATL) is caused by human T-cell lymphotropic virus type-1 (HTLV-1). India is considered as a nonendemic region for HTLV-1. Recent upsurge of cases have been noted in southern parts of India. AIMS AND OBJECTIVES: The objective was to describe skin manifestations in various types of ATL. MATERIALS AND METHODS: Clinical examination, blood investigations, skin biopsies, lymph node biopsies, and immunohistochemistry were performed in five patients. Flow cytometry was performed in two cases. RESULTS: Serological testing was positive for HTLV-1 in all patients. All patients presented with skin lesions. Rare presentations of molluscum contagiosum like papules, purpuric macules and plaques, hypopigmented macules and verrucous papules were seen. Dermatophytic infections occurred in two patients. Mucosal lesion was seen in one patient. Histological features include dermal lymphoid infiltrate with or without epidermotropism. Presence of epidermotropism did not correlate with the severity of disease. All patients except one succumbed to illness within few months to 1 year period. CONCLUSIONS: ATL manifest in myriad presentations and skin lesions are often the earliest manifestation. Cutaneous manifestations of ATL vary from subtle hypopigmented macules to florid nodular lesions, and HTLV-1 screening need to be carried out in all doubtful cases.

Malignant pyoderma gangrenosum eroding the parotid gland successfully treated with dexamethasone pulse therapy.

BACKGROUND: Malignant pyoderma gangrenosum is a rare ulcerative variant of pyoderma gangrenosum (PG) involving the head and neck. Early intervention is crucial to prevent an aggressive outcome. OBJECTIVES: We report a patient with malignant PG eroding into the parotid gland treated with dexamethasone pulse therapy. METHODS: A 47-year-old man presented with necrotic ulcers, predominantly over the face and neck, of one month's duration. Findings of a thorough systemic evaluation were within normal limits. A Mantoux test and pathergy test were negative. Tissue specimens for acid-fast bacilli and fungal cultures were negative. A biopsy showed epidermal necrosis along with perivascular and periappendageal neutrophilic infiltrate without granuloma or vasculitis, consistent with PG. Over the next two weeks, the ulcers progressed in depth and width. The ulcer overlying the right parotid gland continuously discharged clear fluid. The invasive nature of the ulcers and histopathological findings confirmed the diagnosis of malignant PG. Dexamethasone pulse therapy was administered as 100 mg dexamethasone in 500 ml 5% dextrose infused over 3-4 hours on three consecutive days. RESULTS: This treatment resulted in complete healing of the ulcers within six weeks. The patient subsequently received nine similar pulses administered at 28-day intervals and has shown no evidence of relapse over the past year. CONCLUSIONS: In the present patient, parenteral antibiotics and daily steroids, colchicine, and oral minocycline had failed to halt the progression of ulcers. However, dexamethasone pulse therapy resulted in complete healing without sequelae and also proved cost-effective.

Drug reaction with eosinophilia and systemic symptoms: observations from a tertiary care institution.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe drug reaction which can mimic a viral infection, an autoimmune disease or a neoplastic disease. AIMS: To study the clinical and epidemiological aspects of DRESS and to identify the precipitating drugs. METHODS: All patients admitted to the dermatology ward of our tertiary care hospital from 1 st October 2010 to 30 th September 2013 with probable or definite DRESS as per the RegiSCAR scoring system were included in this prospective study. The clinical manifestations observed in the study population were studied and the common offending drugs were identified. RESULTS: During the 3 year study period, 26 patients fulfilled criteria for probable or definite DRESS. In more than 50% of cases, the culprit drug was phenytoin. Most common symptoms observed were fever, rash and facial edema. Liver was the most common internal organ affected. Most of the patients responded to withdrawal of the drug and administration of steroids for 3-6 weeks. One patient with dapsone-induced DRESS died. CONCLUSIONS: Intense facial erythema and edema and an elevated eosinophil count were not found to be bad prognostic factors. In most instances the flare ups during the course of the disease could be managed with a slower tapering of steroids. More prospective studies on DRESS are required to assess the prognostic factors and to formulate better diagnostic criteria.

Adult T-cell leukemia/lymphoma: a retroviral malady.

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive leukemia/lymphoma of mature T-lymphocytes caused by human T-cell lymphotropic virus type 1 (HTLV-1). At a tertiary healthcare center in South India, a 58-year-old female presented with multiple erythematous, crusted, and umbilicated papules over the body along with cervical lymphadenopathy. The skin biopsy was consistent with cutaneous T-cell lymphoma. Although she responded initially to chemotherapy, the disease relapsed after 3 months, and she developed disseminated infiltrated skin lesions, generalized lymphadenopathy, and leukemia. Due to the unusual clinical findings we did HTLV-1 Enzyme-linked immunosorbent assay (ELISA), which turned out to be positive in high titers. Her mother had died at an early age from a hematological malignancy and her daughter was also found to be seropositive. To the best of our knowledge, this is the first case to be reported from India of the chronic type of ATLL associated with mother-to-child transmission of HTLV-1 in two generations. This case also emphasizes that the chronic type of ATLL can occur in nonendemic areas like India and should be suspected in nonresponding cases of mycosis fungoides. It should be kept in mind that the chronic type often presents without hypercalcemia or the characteristic 'flower cells' in the peripheral smear.

Postpartum pemphigoid gestationis.

Pemphigoid gestationis (PG) or herpes gestationis is a rare autoimmune subepidermal blistering disorder associated with pregnancy. The condition typically develops during the second or third trimester of pregnancy, but has been rarely reported in the first trimester and postpartum period. Here, we report a case of PG that presented for the first time in the postpartum period, associated with a low birth weight baby.

Localized psoriasis herpeticum: Case report and review of literature.

Kaposi's varicelliform eruption (KVE) is a widespread cutaneous eruption caused by viruses, especially herpes simplex virus in patients with pre-existing dermatoses. "Psoriasis herpeticum" refers to the rare occurrence of KVE in patients with psoriasis. We report a case of KVE localized to the face in a patient with exfoliative dermatitis secondary to psoriasis. This case is being reported to make the treating clinician aware of the possibility of KVE in patients with psoriatic erythroderma.

Purpuric macules with vesiculobullous lesions: a novel manifestation of Chikungunya.

BACKGROUND: Chikungunya (CHIK) is an emerging viral disease with a myriad of cutaneous manifestations. AIMS: The aim of our study was to document the morphology and evolution of skin lesions in cases presenting with fever, purpuric macules and vesiculobullous lesions, to confirm its causative relationship with CHIK, and to investigate further in order to delineate possible mechanisms of bulla formation in these cases. MATERIALS AND METHODS: A prospective, descriptive hospital-based study was carried out at a tertiary health care centre in Kerala. A total of 10 patients were enrolled in the study and investigated. RESULTS: All cases had morbilliform eruption prior to onset of purpuric macules. Eight cases developed vesiculobullous lesions that arose either de novo or over a part or whole of the purpuric macules. Skin lesions resolved within an average of 7.6 days leaving post-inflammatory hypopigmentation. IgM CHIK enzyme-linked immunosorbent assay (ELISA) was positive in all 10 patients. Tzanck smear from the bullae showed lymphocytes in most cases along with acantholytic cells, necrotic keratinocytes or occasional neutrophils. Skin biopsy showed intraepidermal or subepidermal bullae. Immunohistochemistry revealed predominantly CD8 positive T lymphocytes in the infiltrate. The prognosis was good with supportive management alone. DISCUSSION: The clinical features in our cases are comparable to the 3 previous reports of vesiculobullous lesions in CHIK affected infants. Based on the current evidence, we hypothesize that at least 2 mechanisms are at play for these skin lesions; CHIK virus induced keratinocyte necrosis followed by a cytotoxic immune response, and possible modulation of rash by drugs. CONCLUSION: With severe epidemics of CHIK spreading from Asia and Africa to the Western hemisphere, we must consider bullous CHIK as a differential diagnosis in cases with fever and purpuric and vesiculobullous lesions.

Sub-polar lepromatous leprosy localized to the face.

Leprosy is an infectious disease characterized by a wide spectrum of clinical manifestations, ranging from tuberculoid to lepromatous disease with immunologically unstable borderline forms in between. In clinical practice cases often do not conform to a classical textbook description, which may lead to misdiagnosis if not properly investigated. A 22-year-old patient presented to us with erythematous plaques localized to the face. Slit skin smear for Mycobacterium leprae was positive from lesional as well as non-lesional skin. A biopsy from a plaque showed diffuse atrophy of the epidermis with a subepidermal cell free zone (grenz zone). The cellular infiltrate was composed of foamy macrophages admixed with lymphocytes in the dermis. Fite-Faraco staining revealed clumps of acid-fast bacilli within the macrophages. Based on the skin smear and histopathology findings, a diagnosis of sub-polar lepromatous leprosy was made and the patient was started on multidrug therapy. The exact pathogenesis of localized multibacillary disease is not known. Our case highlights the importance of skin smear and biopsy in all suspected cases of Hansen disease. We conclude that routine skin smear in all new leprosy cases is mandatory to differentiate localized multibacillary cases from paucibacillary cases for the purpose of accurate categorization and treatment.

Idiopathic scoliosis in Singapore schoolchildren: a prevalence study 15 years into the screening program.

STUDY DESIGN: A point prevalence survey of 72,699 schoolchildren in four age groups was performed. OBJECTIVES: To determine the prevalence rates of idiopathic scoliosis and to compare with a previous prevalence study done 15 years earlier. SUMMARY OF BACKGROUND DATA: Prevalence rates for idiopathic scoliosis of 5 degrees or more in schoolchildren were established in a study performed in 1982. There have been no previous data on prevalence rate changes over time. METHODS: A total of 35,558 boys and 37,141 girls from randomly selected schools were screened for scoliosis. Those with scoliometer readings of more than 5 degrees underwent radiographic evaluation. Prevalence rates were calculated for scoliosis at a predefined Cobb angle of 10 degrees and 5 degrees , the latter for comparison with the previous prevalence study. Curve type and distribution, pubertal status, and symptoms were correlated with the prevalence data. RESULTS: Prevalence rates were 0.05% for girls and 0.02% for boys at 6 to 7 years of age, 0.24% for girls and 0.15% for boys at 9 to 10 years of age, 1.37% for girls and 0.21% for boys at 11 to 12 years of age, and 2.22% and 0.66%, respectively, for girls and boys at 13 to 14 years of age. The ratio of girls to boys increased from 1.6 at 9 to 10 years of age to 6.4 at 11 to 12 years of age. Thoracolumbar curves were the most common (40.1%), followed by thoracic curves (33.3%), double/triple curves (18.7%), and lumbar curves (7.9%). Older children had greater proportions of larger curves. Compared with the previous prevalence study in 1982, there was a significant increase in the prevalence rate in girls 11 to 12 years of age. Screening of 11- to 12- and 13- to 14-year-old girls detected curves in the range suitable for bracing, with nearly 96% and 32% of the age groups, respectively, still amenarche or within a year of menarche, and 57% and 34% of the age groups, respectively, having low Risser grades of 0, 1, and 2. CONCLUSIONS: The overall prevalence rate of idiopathic scoliosis in our school population in 1997 was 0.93% in girls and 0.25% in boys. The prevalence rates were low at 6 to 7 and 9 to 10 years of age but increased rapidly to 1.37% and 2.22% for girls at 11 to 12 and 13 to 14 years of age, respectively. The prevalence rate increased significantly in 11- to 12-year-old girls over a 15-year period from 1982 to 1997. Screening of 11- to 12- and 13- to 14-year-old girls identified a significant number who could benefit from brace treatment.

A randomized trial of rigid gas permeable contact lenses to reduce progression of children's myopia.

PURPOSE: To test whether rigid gas permeable (RGP) contact lens wear can reduced the rate of myopia progression in school age children. DESIGN: Randomized clinical trial. SETTING: Single clinical center. STUDY POPULATION: Both eyes of 428 Singaporean children. INCLUSION CRITERIA: 6 through 12 years of age with myopia between -1 and -4 diopters, astigmatism