(Last Updated On: October 29, 2018)

Authors: Diana Marie M. Lazaro-Nisce, MD, Maria Jasmin J. Jamora, MD, FPDS

Abstract

Introduction: Lymphomatoid papulosis (LyP) is a recurrent, self-healing papulonodular skin eruption, belonging to the group of cutaneous CD30+ lymphoproliferative disorders. It is rare worldwide and in the Philippines with only 11 cases recorded from 2011- 2018. Here we describe a case of LyP who was later diagnosed with limited cutaneous systemic sclerosis (lcSSc), an autoimmune inflammatory condition where the skin becomes hard and thickened and the internal organs develop fibrosis and vascular abnormalities. Only 10 cases of this have been recorded from 2011-2018 in the PDS His.

Case Summary: JD is a 17-year old Filipino who consulted due to red rashes and fluid-filled bumps on the arms and legs 2 years prior. He was diagnosed with Rubella and given supportive treatment. During the interim, noted recurrence of similar lesions on the arms and legs hence consulted at Skin Clinic. Biopsy showed superficial and deep dermatitis with leukocytoclastic vasculitis, consistent with LyP. (+) CD3 and (+) CD30 further confirmed the diagnosis. Patient underwent narrowband UVB treatment, starting at 200 MJ, 2x a week for 10 sessions. Lesions resolved with only 1-2 lesions occasionally. Phototherapy was reduced to once weekly. During treatment, patient noted persistence of a whitish firm plaque on the left upper arm which he first noticed one year prior. Biopsy revealed localized scleroderma. Additional laboratories showed (+) ANA, (+) SCL 70 antibody, (-) RF, (-) anti-centromere B antibody. Chest x-ray and spirometry were normal. The patient was treated as a case of lcSSc. He has completed narrowband UVB for 10 sessions, with the plaque noted to become less woody.

Conclusion: This is a case of an adolescent Filipino male presenting with LyP and lcSSc, both of which responded well to treatment with phototherapy.

 

Citation

 

Keywords

Lymphomatoid papulosis, systemic scleroderma, limited cutaneous systemic sclerosis, narrowband UVB, phototherapy

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