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.

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.

Chronic idiopathic urticaria: comparison of clinical features with positive autologous serum skin test.

BACKGROUND: Chronic idiopathic urticaria (CIU), in its extremely severe form, can pose a therapeutic challenge to the treating physician. It has been noted that in one third of such patients, autoantibodies against the IgE receptor are seen and such patients have more severe and unremitting urticaria. AIM: To compare clinical features of autoimmune urticaria with those of other CIU patients. METHODS: We conducted a prospective study in an attempt to correlate the clinical features with autoantibodies, indirectly detected via the autologous serum skin test (ASST), which is the simplest and the best in vivo clinical test for detection of basophil histamine-releasing activity. DISCUSSION: Out of 100 patients with chronic idiopathic urticaria, 34 showed a positive reaction to the autologous serum skin test and it was found that the frequency and severity of attacks was higher in these patients. CONCLUSION: ASST may be used as a simple and cost-effective test for the classification of chronic urticaria, which has proven to be a therapeutic challenge to the treating physician.