Rosai-dorfman syndrome

Rosai-Dorfman syndrome is characterized by sinus histiocytosis with massive lymphadenopathy. A 25-year-old woman presented with multiple erythematous and yellowish papules on the forehead, cheeks, chin and thigh. She had massive generalized, firm, non-tender and non-matted lymphadenopathy and mild hepatomegaly. Her hemogram was normal. A skin biopsy showed collections of histiocytes with emperipolesis and plenty of plasma cells. A lymph node biopsy showed partial loss of architecture, dilated sinuses filled with histiocytes, which showed lymphocytophagocytosis, anisonucleosis and a clear cytoplasm. She was referred to an oncologist for further management

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

Syphilis of fungal world: Novel skin manifestations of histoplasmosis in an immunocompetent host

A case of chronic disseminated cutaneous histoplasmosis with unusual skin manifestations in an immunocompetent host is reported. Presence of cutaneous ulcers, linear erythematous plaques, skin coloured atrophic plaques and recurrent self-limiting oral ulcers in a single patient has not been documented in literature so far. Diagnosis was established by identifying small intracellular yeast-like cells of Histoplasma in tissue smear and skin biopsy. Leishman stained tissue smear proves to be an easy and simple procedure for diagnosis of histoplasmosis

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

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