Keratosis lichenoides chronica with mucous membrane involvement and ocular pseudo-pemphigus. Follow-up study over 18 years. Retinoid therapy
Schnitzler, L.; Bouteiller, G.; Bechetoille, A.; Verret, J.L.
Annales de Dermatologie et de Venereologie 108(4): 371-379
1981
ISSN/ISBN: 0151-9638 PMID: 7258990 Document Number: 171341
An uncommon ocular and mucocutaneous syndrome is reported. Cutaneous manifestations include keratotic plugs occasionally disposed into verrucous plaques, lichenoid lesions on face, neck, back of hands and nails. Histologically, 3 types of lesions are observed: marked hyperkeratosis, lichenoid lesions, and follicular pustulosis. Mucous membrane alterations including stomatitis, chronic balanitis with ulcerative and keratotic lesions leading to synechiae were present for nearly 20 yr. Ocular abnormalities have progressed into lesions similar to those of cicatricial pemphigoid. Direct immunofluorescence study in the vicinity of a buccal erosion showed a complement deposit on basement membrane. Direct and indirect immunofluorescence, studies on involved and uninvolved skin, showed negative results. Atypical Reiter's syndrome was considered but a diagnosis of keratosis lichenoides chronica with severe mucous cicatricial lesions,was made. Clear improvement of keratotic lesions was noted with oral retinoid therapy.