Case Report
Year: 2017 | Month: February | Volume: 7 | Issue: 2 | Pages: 348-352
Lichen Scleromyxoedema: Rare Disorder, Excellent Response to Steroid Pulse and PUVA Therapy: A Case Report
Dr Ghanshyam Verma1, Dr. Jatin Sharma2, Dr G.R. Tegta3, Dr Ajeet Negi4
1Associate Professor, 2Ex Resident, 3Professor and Head, 4Senior Resident,
Department of Dermatology, Venereology and Leprosy, Indira Gandhi Medical College, Shimla.
Corresponding Author: Dr Ghanshyam Verma
ABSTRACT
Lichen is a rare, chronic, progressive, fibro-mucinous disorder of unknown etiology characterized by numerous waxy lichenoid papuloplaques with extensive thickening and hardening of skin in the absence of thyroid disease. It involves the skin of face, trunk, forearms and hands. Characteristic cutaneous involvement is associated with fibroblast proliferation and mucin deposition in upper dermis, monoclonal gammopathy and systemic multi-organ involvement and can be fatal. Despite anecdotal reports of success with various agents, no satisfactory treatment is currently available. We report a 41 year old male with Lichen scleromyxoedema who responded excellently to weekly dexamethasone pulse and PUVA therapy.
Key words: Lichen, scleromyxoedema, mucinosis, monoclonal gammopathy, dexamethasone pulse, PUVA.