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Nodular cephalic form of xanthoma disseminatum
*For correspondence: drntbhari@gmail.com
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Received: ,
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This article was originally published by Wolters Kluwer - Medknow and was migrated to Scientific Scholar after the change of Publisher.
A 48 yr old female† presented to the department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India, in March 2019, with yellowish papules and nodules over the periorbital areas for two years (Figure A). She also noticed similar lesions over the neck for the last six months (Figure B). There was no history of weight loss, bone pain, shortness of breath, polyuria or polydypsia. Skin biopsy showed diffuse infiltrate of histiocytes, foamy histiocytes and lymphocytes with Touton giant cells (Figure C and D). Areas of necrobiosis were not appreciated. Immunohistochemistry revealed a positive staining with CD68 with negative CD1a and S100. Systemic evaluation and investigations including those for monoclonal gammopathy and diabetes insipidus were within normal limits. A final diagnosis of nodular cephalic form of xanthoma disseminatum was made based on clinicopathological correlation.
- (A) Periorbital yellowish papules and nodules resulting in obstruction of vision. (B) Similar confluent lesions over the neck. (C and D) Skin biopsy showed diffuse infiltration of histiocytes, lymphocytes and Touton giant cells (blue arrow) (H and E, ×100, ×400). (E and F) Flattening of skin lesions after four infusions of cladribine.
For the first three months, she was treated with dexamethasone pulse with daily cyclophosphamide without much improvement. Four monthly cycles of cladribine infusion (0.14 mg/kg for five days) showed 50-60 per cent improvement in her periorbital lesions and 80-90 per cent improvement in her neck lesions (Figure E and F). She was planned for two more monthly infusions.
Conflicts of Interest: None.
