lgA pemphigus, intraepidermal neutrophillic type in an 8-year old Filipino female: A case report

INTRODUCTION@#lgA pemphigus is a rare, chronic, relapsing, benign group of autoimmune intraepidermal blistering dermatosis with an unknown etiology. It is characterized by significantly pruritic, vesiculopustular lesions that occur mainly on the trunk and proximal extremities. Histopathologic and immunofluorescence studies show intraepidermal blisters and deposition of immunoglobulin A in the intercellular spaces of the epidermis, respectively.@*CASE REPORT@#To our knowledge, we present the first reported pediatric case of lgA pemphigus, intraepidermal neutrophilic type, in an 8-year old Filipino female with a 2-year history of generalized papules and flaccid pustules, some forming an annular pattern. Diagnosis was confirmed by histopathology and direct immunofluorescence. Enzyme-linked immunosorbent assay for Desmoglein 1 was negative. Complete clearance of lesions was achieved with dapsone, colchicine and prednisone.

Primary cutaneous anaplastic large-cell lymphoma with metastases in an 81-year-old Filipino female

Introduction@#Primary Cutaneous Anaplastic Large Cell Lymphoma (PC-ALCL) is a rare Non-Hodgkin lymphoma (NHL) representing approximately 9% of all cutaneous lymphomas.3 It usually manifests as a slow-growing, solitary tumor which has a tendency to regress spontaneously. However, metastasis is reported in 5-10% of cases.2@*Case Summary@#Our case is an 81-year-old female with a four-month history of an enlarging nodule on the left upper back. The initial impression was a malignant cutaneous tumor. Histopathology revealed epidermotropic large atypical lymphocytes and diffuse infiltrates of hyperchromatic, pleomorphic mononuclear cells and lymphocytes. Immunohistochemistry showed CD3+, CD20-, CD30+, epithelial membrane antigen (EMA)- and anaplastic lymphoma kinase (ALK)- consistent with PC-ALCL. Metastatic work-up revealed pulmonary involvement. The patient underwent two cycles of multi-agent chemotherapy with marked improvement. However, patient declined further treatment and expired five months after the diagnosis.@*Conclusion@#This case emphasizes that although PC-ALCL commonly presents with an indolent course, extracutaneous spread can occur. Prompt treatment with chemotherapy increases survival significantly with a cure rate of 92%.2