1.Cutaneous Rosai-Dorfman disease in a 40-year-old female: A case report
Joland A. San Juan ; Juan Antonio D. Cervantes ; Johannes F. Dayrit ; Ricky H. Hipolito ; Ma. Teresita G. Gabriel
Journal of the Philippine Dermatological Society 2021;30(1):53-56
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.
Lymphadenopathy
2.Primary cutaneous anaplastic large cell lymphoma in a 73-year-old Filipino male
Juan Antonio D. Cervantes ; Eunice Kaye M. Rayos-Lopez ; Ma. Teresita G. Gabriel ; Reynaldo L. Ugalde ; Johannes F. Dayrit
Journal of the Philippine Dermatological Society 2019;28(2):55-57
Introduction:
Primary cutaneous anaplastic large cell lymphoma (PCALCL) is an uncommonly encountered subtype
of cutaneous lymphoma under the classification of CD30-positive lymphoproliferative disorders which presents
histologically as large atypical lymphocytes with pleomorphic and anaplastic cytology that localizes to the dermis.
Although recurrent, PCALCL usually carries a good prognosis, with 5-year survival rates ranging from 85% to 95%.
Case Summary:
We report a 73-year-old elderly male who consulted at our out-patient department with a 3-year
and 6-month history of multifocal, gradually enlarging, erythematous nodules with dry, necrotic areas on the scalp,
right auricular area, left axillary area, right forearm, and right thigh, accompanied by loss of appetite and nontender
cervical, left axillary, and right inguinal lymphadenopathy. Previous skin punch biopsy and immunohistochemical
stain done by the patient’s preceding dermatologist was signed out as “suggestive” of pseudolymphoma. However,
management with intralesional corticosteroid injections provided no improvement. Skin punch biopsy done at our
institution revealed ALK negative (-) anaplastic large cell lymphoma. Patient was then referred to an oncologist,
however, the patient was lost to follow-up and succumbed to community acquired pneumonia.
Conclusion
This case highlights the importance of a thorough diagnostic assessment as recent studies indicate a
poorer prognosis of ALK (-) cases, with overall 5-year survival rates consistently below 50%.
Lymphoma
;
Lymphoma, Large-Cell, Anaplastic