Sunanda Mahajan

Type I lepra reaction presenting as immune reconstitution inflammatory syndrome.

Immune reconstitution inflammatory syndrome (IRIS) is an unusual inflammatory reaction due to infectious and non-infectious causes occurring in human Immunodeficiency virus (HIV)-infected patients. IRIS occurs after the initiation of antiretroviral therapy. There are no reports of type I lepra reaction due to IRIS in published literature from India. We report two cases of HIV-infected males who presented with borderline tuberculoid leprosy in type 1 reaction after the initiation of highly active antiretroviral treatment (HAART). Case 1 presented with multiple, tender, erythematous and hypoesthetic plaques on the trunk and extremities after 3 months of antiretroviral therapy. In case 2, type I lepra reaction was observed 2 months after the initiation of HAART.

Disseminated cutaneous with nasopharyngeal rhinosporidiosis: Light microscopy changes following dapsone therapy.

We report a case of disseminated cutaneous and nasopharyngeal rhinosporidiosis in a 48‐year‐old man. The patient complained of a 6‐month history of six subcutaneous skin‐coloured swellings on the body and a 5‐year history of a pea‐sized swelling on the inner aspect of the left lower eyelid. Histopathological examination from one of these lesions showed multiple globular cysts packed with endospores that were typical of rhinosporidiosis. We report this case because of its rarity and to describe the morphological effects of dapsone in promotion of a host inflammatory response to the organism.

Multiple capillary hemangiomas: a distinctive lesion of multicentric Castleman's disease and POEMS syndrome.

A diagnosed case of Castlemans disease, proven by biopsy from enlarged inguinal lymph nodes, presented with multiple, asymptomatic, erythematous papules and nodules prevalent since nine years over the trunk and extremities. The lesions had been gradually increasing in number and size. The patient had had plasmacytoma of the lower thoracic vertebra 12 years ago, for which he was adequately treated with chemotherapy and local radiotherapy. Dermatological examination revealed erythematous papules and nodules on the face, trunk, and extremities that were diagnostic of capillary hemangiomas. Histopathology of the erythematous, soft papule was suggestive of capillary hemangioma. Contrast-enhanced computerized tomography of the abdomen and pelvis showed multiple retroperitoneal nodes suggestive of Castlemans disease along with multiple osteolytic lesions in the pelvic girdle and vertebrae. The patient was treated with injection rituximab and is currently under follow-up. We report this case to highlight a rare association between Castlemans disease and POEMS syndrome.

Granulomatous mycosis fungoides with hypohidrosis mimicking lepromatous leprosy

Granulomatous mycosis fungoides (GMF) is a rare type of cutaneous T cell lymphoma. A 38-year-old married male presented with decreased sweating all over the body for last 8 years, progressive redness and scaling over body for 2 years and multiple noduloulcerative lesions over the body for 1 year. Cutaneous examination revealed generalized erythema and scaling with poikilodermatous changes over chest and upper back along with multiple noduloulcerative lesions. Skin biopsy from a nodular lesion revealed dense granulomatous infiltrate of atypical lymphocytes with epidermotropism and sparing of appendages. Diagnosis of GMF was made. Computed tomographic scan of thorax, abdomen and pelvis revealed axillary and inguinal lymphadenopathy. Immunohistochemistry revealed leukocyte common antigen and CD3 positivity suggestive of T cell origin. Patient was started on CHOP (Cyclophosphamide, Hydroxydaunorubicin, Oncovin and Prednisolone) regimen of chemotherapy with marked improvement after three cycles of chemotherapy. This case had some clinical resemblance to lepromatous leprosy.

Cutaneous vasculitis as a presenting feature of multiple myeloma: A report of 2 cases

We report two male patients who presented with symmetrical, painful purpura that evolved into bullae and necrotic ulcers, predominantly on the extremities, over two months in spite of conventional therapy including oral steroids. Examination showed livedoid and purpuric patches with necrotic centers in starburst pattern over the extremities and buttocks. The first case also had similar lesions over the ears. The clinical presentation and the histopathological examination suggested a diagnosis of necrotizing leukocytoclastic vasculitis (LCV). Blood testing ruled out connective tissue disease, hepatitis B or C infection or streptococcal infection as underlying cause of vasculitis. Serum antinuclear factor, antineutrophilic cytoplasmic antibody and anticardiolipin anticoagulant were negative in both cases. Cryoglobulins were positive in case 2. An incidental finding was raised serum proteins and globulins in case 2. Further investigations revealed M band on electrophoresis and features of multiple myeloma on bone marrow biopsy in both cases. These cases emphasize the importance of simple investigations like serum proteins in the evaluation of LCV.

Primary cutaneous angiosarcoma of the nose.

Indian Journal of Dermatology, Venereology, and Leprology | July-August 2012 | Vol 78 | Issue 4 496 3. Fletcher CD, Beham A, Bekir S, Clarke AM, Marley NJ. Epithelioid angiosarcoma of deep soft tissue: A distinctive tumor readily mistaken for an epithelial neoplasm. Am J Surg Pathol 1991;15:915-24. 4. Bacchi CE, Silva TR, Zambrano E, Plaza J, Suster S, Luzar B, et al. Epithelioid angiosarcoma of the skin: A study of 18 cases with emphasis on its clinicopathologic spectrum and unusual morphologic features. Am J Surg Pathol. 2010;34:1334-43. 5. Prescott RJ, Banerjee SS, Eyden BP, Haboubi NY. Cutaneous epithelioid angiosarcoma: A clinicopathological study of four cases. Histopathology 1994;25:421-9. 6. Barber WA, Scriven P, Turner D, Hughes D, Wyld L. Epithelioid angiosarcoma: Use of angiographic embolisation and radiotherapy to control recurrent haemorrhage. JSCR 2010; 5:7-10.

Epidermodysplasia verruciformis: An unusual malignant transformation

Epidermodysplasia verruciformis (EV) is a rare, life-long heritable disease caused due to a unique susceptibility to human papilloma virus. The disseminated verrucous lesions and pityriasis versicolor-like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. Malignant transformation into syringoid eccrine carcinoma (SEC) has been reported only once so far. SEC is an extremely invasive, rare, locally destructive, slowly growing adnexal tumor. We hereby report the association of EV with SEC in a 29-year-old male.

Epidermotropic metastatic melanoma with perilesional depigmentation in an Indian male.

Melanoma is a rare form of cutaneous malignancy encountered in the dark skin population. Epidermotropic metastatic melanoma is a rare form of cutaneous metastatic melanoma which can mimic primary melanoma on histopathology. Hence its differentiation is of immense prognostic importance. The occurrence of rim of depigmentation around the primary cutaneous melanoma has previously been reported to portend a bad prognosis. The occurrence of vitiligo like lesions in patients with metastatic melanoma in comparison has a better prognosis. However the occurrence of depigmentation around the secondaries is rare and its importance is not well known. Hence we wish to report a case of epidermotropic metastatic melanoma with perilesional depigmentation in a 78 year old Indian male.

Successful treatment of multifocal cutaneous leishmaniasis with miltefosine

We report a 48-year-old immunocompetent male, resident of Central India, who presented with slowly progressive asymptomatic multiple red lesions on different parts of body. On enquiry, the patient gave history of travel to Middle East 6 months back. Examination showed 10 crusted erythematous indurated plaques and nodules over forearms, left leg, right index finger, left wrist and dorsa of both feet. Histopathological examination of tissue biopsy showed multiple intracellular as well as extracellular leishmania donovan bodies. Keeping in mind the higher rate of side effects to pentavalent antimony, we treated this patient with oral miltefosine 50 mg bid and the lesions showed complete resolution over 4 months of therapy.

Nodular amelanotic melanoma

We report a case of 65-year-old male patient who presented with multiple erythematous papules coalescing to form a nodular mass over posterior aspect of right thigh of six months duration. His general and systemic examinations were within normal range except for right inguinal lymphadenopathy. Biopsy from the lesion was done, which showed diffuse infiltrate of nests of atypical melanocytes extending upto reticular dermis. Malignant cells were positive for S100 and human melanin black 45(HMB 45). Hence, a diagnosis of amelanotic melanoma (AM)--Clarke level IV and TNM stage III was reached. MRI of involved leg showed fungating soft tissue mass in the posterolateral aspect of right thigh and metastatic right inguinal adenopathy. Fine needle aspiration cytology (FNAC) from the right inguinal nodes confirmed metastasis of melanoma. The patient was referred to oncosurgery department for further management.