Vidya Kharkar

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 histoplasmosis in an immunocompetent adult.

Histoplasmosis, a systemic mycosis caused by the dimorphic fungus Histoplasma capsulatum var capsulatum and Histoplasma capsulatum var duboisii is endemic to many parts of the world. The clinical manifestations range from acute or chronic pulmonary infection to a progressive disseminated disease. After initial exposure to the fungus, the infection is self-limited and restricted to the lungs in 99% of healthy individuals. The remaining 1%, however, progress to either disseminated or chronic disease involving the lungs, liver, spleen, lymph nodes, bone marrow or rarely, the skin and mucous membranes. Mucocutaneous histoplasmosis is frequently reported in patients with acquired immune deficiency syndrome (AIDS), but it is rare in immunocompetent hosts. A 60-year-old male presented with asymptomatic swelling of the hard palate and crusted papules and nodules over the extremities, face and trunk. Clinically, the diagnoses of cutaneous cryptococcosis versus histoplasmosis was considered in this patient. A chest X-ray revealed hilar lymphadenopathy. Enzyme-linked immunosorbent assay (ELISA) for human immunodeficiency virus (HIV) was nonreactive. Skin biopsy revealed multiple tiny intracellular round yeast forms with a halo in the mid-dermis. Culture of the skin biopsy in Sabourauds dextrose agar showed colonies of Histoplasma capsulatum. Despite an investigation including no evidence of underlying immunosuppression was found, he was started on IV amphotericin-B (0.5 mg/kg/day). However, the patient succumbed to his disease 2 days after presentation. We report a rare case of disseminated cutaneous histoplasmosis in an immunocompetent individual.

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.

Keratotic vascular papules over the feet: a case of Waldenström's macroglobulinaemia-associated cutaneous macroglobulinosis

Waldenström’s macroglobulinaemia (WM) is a plasma‐cell dyscrasia characterized by the monoclonal proliferation of lymphoplasmacytes. A 48‐year‐old man presented with a 4‐year history of multiple painful, hyperkeratotic deep‐seated papules over the pressure areas of both soles. He had a 1‐year history of Raynaud’s phenomenon, intermittent epistaxis, recurrent vomiting, tingling and numbness, and visual disturbances. Histological examination of a skin biopsy found amyloid‐like deposits in the upper and mid dermis involving dermal blood vessels, but apart from periodic‐acid–Schiff, various stains gave negative results for amyloid. Direct immunofluorescence was positive for IgM antibody. Hence, a diagnosis of WM with cutaneous macroglobulinosis was made. Immunoelectrophoresis found monoclonal IgM kappa antibody, and bone‐marrow examination revealed a lymphoplasmacytoid malignancy. The patient’s systemic systems were attributed to hyperviscosity syndrome associated with WM and the cutaneous papules were identified as deposits of excess IgM antibodies. The patient received five cycles of chemotherapy, resulting in nearly complete resolution of the skin lesions and systemic symptoms.

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.

Diamond necklace appearance in superficial porokeratosis.

CLINICAL CHALLENGE Superficial varieties of porokeratosis may be difficult to diagnose because of imperceptible or incomplete ridges at the edge of the lesions. Moreover, biopsy sections may sometimes miss the typical cornoid lamella, making the diagnosis more difficult. Herein, we present a new finding on ultraviolet (UV) dermoscopy in superficial porokeratosis that the keratotic ridge in porokeratosis glows in UV light with a ‘‘diamond necklace’’ appearance. A 21-year-old woman presented to our outpatient department with asymptomatic lesions of 15 years’ duration on both her upper and lower extremities. The physical examination revealed the presence of multiple, well defined, barely elevated papules with a slightly atrophic center at these sites (Fig 1). The mucosae and nails were spared.

The angiotensin-converting enzyme gene insertion/deletion polymorphism in Indian patients with vitiligo: a case-control study and meta-analysis.

Background Vitiligo is a common, acquired, idiopathic depigmenting skin disorder. Although the exact pathogenesis remains unknown, genetic susceptibility and autoimmune responses play a role in vitiligo development. Previous studies have suggested that the D allele of the insertion/deletion (I/D) polymorphism of the angiotensin‐converting enzyme (ACE) gene is associated with vitiligo in Indians and Koreans. Furthermore, significantly higher serum ACE levels have been demonstrated in patients with some autoimmune and autoinflammatory disorders.

Trichorrhexis nodosa with nail dystrophy: diagnosis by dermoscopy.

A 25-year-old male, born of non-consanguineous marriage presented with complaints of sparse and thin hairs over scalp and dystrophy of nails since childhood. This case highlights the association of trichorrhexis nodosa with nail dystrophy and the use of trichoscopy as a noninvasive method for diagnosis of hair disorders.