Mastocytosis is a rare myeloid neoplasm characterized by abnormal proliferation and accumulation of mast cells in one or more organ systems including the skin, bone marrow, liver, spleen, lymph nodes and gastrointestinal tract. An infant presenting with bullous lesions is an even rarer clinical presentation of cutaneous mastocytosis. The symptoms and complications are mostly in proportion to the mast cell degranulation in tissues. Management is focused on preventing and treating this event. We report a three-month-old infant with bullous mastocytosis to enhance awareness about this rare diagnosis.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL; α/β T-cell subtype) is a distinct variantof cutaneous T-cell lymphomas, which presents as inflammatory subcutaneous nodules. A 17-year-old male presented with recurrent fever with concomitant facial swelling, pedal edema, hepatosplenomegaly, and mildly tender subcutaneous plaques in generalized distribution along with patches of scarring alopecia on scalp. There were features of macrophage activation syndrome in the form of hemophagocytosis in the bone marrow, pancytopenia, high serum lactate dehydrogenase levels, low fibrinogen clotting activity, prolonged activated prothrombine time (aPTT), increased serum ferritin, hypoalbuminemia, and hypertriglyceridemia. Histopathology showed lobular panniculitis-like infiltration by atypical lymphocytes rimming the adipocytes. Immunohistochemistry revealed positive CD3 and CD8 markers, whereas CD4, CD56, and CD20 were negative, consistent with the diagnosis of α/β type of SPTCL. Treatment with oral prednisolone (1mg/kg/day) and cyclosporine (2mg/kg/day; 100 mg) led to rapid subsidence of fever, plaques, and abnormal hematological parameters within a few weeks.
<p>Dermatophytosis, a superficial fungal infection has attained significant extents among Indian population. Its clinical presentation is diverse, often in terms of morphology, severity type and involvement of all age groups. Management of dermatophytosis has become an important public health issue in India. Cases of steroid modified dermatophytosis are being encountered frequently, mostly caused due to the inadvertent use of steroid in combination with topical antifungal agents. This combination is available over the counter and is often used for the management of inflammation and pruritis associated with the disease. Current treatment recommendations must be reviewed as per the current clinical scenario of the disease. Thus, a topical formulation of an anti-itch agent like pramoxine and antifungal agent with an anti-inflammatory property like eberconazole holds a promising treatment approach for dermatophytosis. This article focuses on the challenges encountered in the management of dermatophytosis and strategies for optimizing treatment for better patient outcomes. An expert’s panel discussion was conducted involving fifteen dermatologists all over India, during which modified Delphi method was executed for a set of nine statements. Agreement of more than 75% was set to reach the consensus. This consensus document was developed to review the available evidence and make recommendation based on the expert group’s opinion for the use of pramoxine containing topical formulation of eberconazole, as it provides the benefit of having an anti-inflammatory and anti-pruritic activity in a single formulation, where pramoxine can be utilised as an excipient to combat pruritis associated with dermatophytosis.</p><p><strong> </strong></p>
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