Amyloidosis Cutis Dyschromica in a 16-Year-Old Filipino Girl: A Case Report.

Amyloidosis cutis dyschromica is a rare variant of primary cutaneous amyloidosis characterized by hyper- and hypopigmented macules. In this paper, we reported a case of a 16-year-old Filipino girl with hyper- and hypopigmented to depigmented macules on the upper and lower extremities, which started when she was 9 years of age.

Fibroblastic rheumatism: a report of 4 cases with potential therapeutic implications.

BACKGROUND: Fibroblastic rheumatism is a rare dermatoarthropathy characterized by the sudden onset of cutaneous nodules, flexion contractures, and polyarthritis. Histopathology in the correct clinical context confirms the diagnosis. Treatment is based on observational data from single case reports. OBJECTIVE: We describe 4 cases, review histologic findings, and discuss therapeutic responses. METHODS: Cases coded as fibroblastic rheumatism were retrieved from institutional and consultation files. Medical charts and biopsy specimens were reviewed. Elastic stains and immunostains for smooth muscle actin, S100, CD34, desmin, and epithelial membrane antigen were performed on selected cases. RESULTS: Four cases were identified. Patients displayed cutaneous nodules and arthralgias. Flexion contractures/decreased motion were present in two patients; one patient had associated Raynaud phenomenon and erosive joint disease. Biopsy specimens demonstrated a fibroblastic proliferation associated with a collagenous stroma. Growth patterns varied from cellular fascicles to paucicellular randomly arranged spindle cells. Elastic fibers were absent in all cases tested (3/3). Immunohistochemical stains demonstrated immunoreactivity for smooth muscle actin in one of 3 cases in a myofibroblastic pattern. Other stains were negative. One patient had complete resolution of disease with methotrexate. One patient partially responded to interferon-alfa and ribavirin and was subsequently treated with methotrexate with additional improvement. One patient had limited response to all therapies attempted. One patient was lost to follow-up. LIMITATIONS: Small sample size (n = 4) is a limitation. CONCLUSION: Our data expand the clinical, histologic, and therapeutic response data on fibroblastic rheumatism. Correlation with clinical history is critical to avoid misdiagnosis as other fibrosing lesions. Methotrexate and interferon-alfa are potential therapies.

A rare case of superficial polyfibromatosis in a Filipino male adolescent

The superficial fibramatoses are a rare group of mainly benign disorders that clinically manifest as slow progressive dermal hypertrophies. This bewildering group of dermal matrix proliferation is composed of fibrous tissue, fibroblastic cells or spindle stromal cells with varying degrees of cellularity. Skin lesions are usually solitary or localized to a specific site of predilection. This is a case of a 15-year-old Filipino boy, presenting with asymptomatic firm, skin colored to erythematous papules and nodules on his limbs and nose. The lesions slowly worsened, resulting in flexion deformity and limited range of motion of both hands.

Generalized asymptomatic red nodules in a 21-year old Filipino male

A 21 year old Filipino male presented with swelling of the second right hand digit unresponsive to antibiotics. Amputation revealed chronic inflammation and negative cultures. He developed sterile conjunctivitis and a generalized eruption of asymptomatic red papules and nodules. First skin biopsy revealed a diffuse infiltrate of epithelioid and foamy histiocytes, diagnosed as "juvenile xanthogranuloma." The second biopsy revealed large histiocytes with a "ground-glass" eosinophilic cytoplasm, multinucleated giant cells, and mixed cell infiltrate. Immunohistochemistry showed histiocytes staining with (+)S100 and (+)CD68, and (-)CD1a. Final diagnosis was "multicentric reticulohistocytosis." Despite treatment with oral prednisone, methotrexate and alendronate, lesions were progressive. CONCLUSION: This fascinating case manifests with overlapping features of both juvenile xanthogranuloma and multicentric reticulohistiocytosis, and lead the authors to suggest considering the spectrum of diseases called the non-Langerhans cells histiocytosis when presented with a generalized nodular eruption.