Severe Generalized Ulcerative Lichen Planus

Abstract

Sir,Ulcerative lichen planus (ULP), a rare variant of lichenplanus, is characterized by chronic painful ulcers on thefeet (1). We report a patient who presented after PUVAtherapy with generalized ulcerations on an erythema-tous base, and with natural remission within a month.CASE REPORTA 67-year-old man was referred to our hospital inMarch 2000 for evaluation of poikiloderma on theentire body surface. The lesions started as an itchingrash on the buttock in 1965 and had gradually spread,especially during summer months. Erosions and/or ulcershad formed on the trunk and limbs a few times peryear since 1968. These lesions healed within a monthwithout treatment, leaving either hypo- or hyper-pigmented macules, and developed to poikilodermawhen he was 64 years of age. Simultaneously, hegradually lost the hair on his scalp. His medical historyincluded atrial fibrillation without medication since theage of 50. He had not used any kinds of drugshabitually.Physical examination revealed diffuse erythema withfine scales, based on reticulate pigmentation and tele-angiectasia on the trunk, limbs and head. Hyperker-atosis was seen on the palms and soles. The scalp hairwas sparse (Fig. 1). The finger and toe nails showedpachyonychia with yellowish discoloration. There wereno lesions on the mucous membranes.The results of routine laboratory examinations, inclu-ding liver function tests, were within normal limits. Theresults of the following laboratory studies were withinnormal limits or negative: anti-HTLV-1 antibody, anti-hepatitis C virus antibody, hepatitis B surface antigen,LE test and uroporphyrine.A skin biopsy from the erythema on the thighshowed wedge-shaped hypergranulosis, irregularacanthosis, damage to the basal cell layer, and band-like dermal lymphocytes. Interestingly, fatty tissues,reaching to the upper dermis, were seen beneath theband-like zone of inflammation. Below the fatty tissuethere were thick collagen deposits through the deepdermis and a few hair follicles.Direct immunofluorescence showed no specific depo-sits. The findings were highly suggestive of lichen planus,but a provisional diagnosis of parapsoriasis lichenoideswasmadebecauseofthegeneralizedlesions.Topicalappli-cations of diflucortolone valerate and tacalcitol, andtopical PUVA therapy to the back, were initiated inMarch 2000 and had a moderate effect.In April, the patient had a sudden onset of high feverwith numerous small vesicles and erosions on the back.These symptoms led us to discontinuation of PUVAtherapy.Aciclovirwasgivenwithouteffectandantibodiesto herpes simplex and varicella zoster viruses were nega-tive. Direct immunofluorescence of the vesicular skinshowed no linear deposits of IgG or C3 in the basalmembrane zone, with positive immunostaining forlaminin on the floor of the cleft (Fig. 2). Furthermore,immunoblot analysis demonstrated no specific anti-bodies. Ulcerative lichen planus was diagnosed on thebasis of these data.A spiking fever continued and the erosions developedrapidly into irregular-shaped ulcers, widespread overthe whole body. The patient was treated with sympto-matic therapies and the ulcers began to re-epithelize onthe upper trunk. In mid-May, the high fever graduallysubsided and the ulcers cleared almost completely.Several erosions and vesicles were noted occasionally,but they did not spread widely during the subsequent2 months. The patient suffered from recurrent episodesof widespread vesicular lesions in mid-July. In this epi-sode, the patient became bed-ridden due to pain fromthe ulcers, and had worsening decubitus on the sacralregion. The ulcers and general condition improved withunchanged symptomatic therapies in September. How-ever, his condition progressively deteriorated due to