AIDS-associated Kaposi sarcoma: A case series in the Philippine setting

Introduction@#Acquired immunodeficiency syndrome-Kaposi sarcoma (AIDS-KS) has unique clinical characteristics, often dis- seminated on presentation, a rapidly progressive course, and often fatal outcome. Describing the epidemiology and clinical characteristics of AIDS-KS in the Philippines may lead to early recognition, diagnosis, and management of this condition, which are the keys to preventing significant complications.@*Case Series@#AIDS-KS in 11 Filipino MSM patients with a mean age of 36.55 years (SD 11.54) was described. Violaceous plaques and nodules were present for an average of 5.1 months prior to diagnosis confirmed by biopsy. Histopathologic findings from all pa- tients were consistent with KS. The median CD4+ count of patients was 44 cells/microliter (range, 4 to 181). Six patients presented with opportunistic infections (OI)/AIDS-related conditions (ARC). The most common OIs observed were pulmonary tuberculosis, oropharyngeal candidiasis, and Pneumocystis jiroveci pneumonia. Nine patients improved with highly active antiretroviral therapy (HAART). One patient required modification on his HAART regimen, which was shifted to 2 NRTI and ritonavir-boosted protease inhibitor, and one patient died due to AIDS-related complications.@*Conclusion@#This series of 11 cases of AIDS-KS showed similar demographic, clinical and histopathologic characteristics to pre- viously published studies. Findings suggest the need for earlier recognition and diagnosis. While HAART afforded clinical improve- ment in a majority of patients, other treatment options such as chemotherapy should be considered for appropriate patients.

Cutaneous Rosai-Dorfman disease in a 40-year-old female: A case report

Introduction@#Rosai-Dorfman disease is a rare disease that manifests with painless cervical lymphadenopathy, fever, anemia, an elevated erythrocyte sedimentation rate (ESR), and hypergammaglobulinemia. Extranodal lesions occur in 1/3 of patients, and the skin is involved in more than 10% of cases. Purely cutaneous disease is uncommon and only about more than 100 cases have been reported. Cutaneous Rosai-Dorfman Disease (CRDD) appears to be a distinct entity with different age and race predilection from cases with lymph node involvement.@*Case report@#This is a case of a 40-year-old Filipino female who presented with multiple erythematous papules and plaques with pustules on the cheeks. Skin punch biopsy showed a dense dermal infiltrate of polygonal histiocytes with abundant cytoplasm and vesicular nuclei. Emperipolesis was also present. The histiocytes were highlighted by the immunohistochemical stains S-100 and CD68 and was CD1a negative. Complete blood count and ESR were normal. Cervical lymphadenopathy was absent. Findings were consistent with Cutaneous Rosai-Dorfman disease. The patient was started on methotrexate at 15mg/week with folic acid supplementation. Mild soap, benzoyl peroxide 5% gel and tretinoin 0.05% cream once daily were maintained during the treatment course. There was significant decrease in erythema and size of existing lesions after 2 months. The patient was referred to a hematologist for monitoring of possible future systemic involvement.@*Conclusion@#Because of its rarity, clinicopathological correlation is always mandatory to establish a diagnosis of CRDD. Immu- nohistochemical stains are required to differentiate this entity form other forms of Langerhans cell histiocytosis. Multidisci- plinary referral is required to rule out concomitant systemic involvement.

Dermatitis herpetiformis-like Linear IgA bullous disease in a Filipino

@#INTRODUCTION: Linear IgA bullous disease (LABD) is a rare autoimmune blistering disease characterized by subepithelial bullae and linear IgA deposition along the basement membrane zone of the epidermis. Lesions present as pruritic papulovesicles and tense bullae which may coalesce forming annular or polycyclic urticarial plaques with blistering on the edge of the lesions forming the classic “string of pearls” sign. Lesions may affect the face, trunk, and extensor extremities. Incidence rates range from 0.5 to 2.3 cases per million individuals per year. Due to its rare occurrence, there are only a fewdocumented reports on cases of LABD, particularly in the Filipino population. CASE REPORT: A 33 year-old Filipino female consulted because of a 3-week history of severely pruritic vesicles and crusts on the face, trunk, and arms. Patient noted no gastrointestinal symptoms on consultation. Skin punch biopsy revealed subepidermal blisters with collection of neutrophils at the dermal papillae. Direct immunofluorescence showed strong (+2) deposits of linear IgA at the dermo- epidermal junction in perilesional skin thus validating the diagnosis. The patient’s serum was negative for IgA anti-tissue transglutaminase and IgA antiendomysial antibodies. Patient was treated with topical corticosteroids and Dapsone 50 mgs OD with remarkable improvement. CONCLUSION: Linear IgA bullous disease has very few reported cases especially in the Philippine setting. Dapsone is considered the first-line treatment. The disease may persist for a decade or longer, and relapses may occur. Careful history-taking accompanied by the histological, immunofluorescence, and serological findings must be done to ensure proper treatment and good prognosis.