Severe generalized ulcerative lichen planus.

A 67-year-old man was referred to our hospital in March 2000 for evaluation of poikiloderma on the entire body surface. The lesions started as an itching rash on the buttock in 1965 and had gradually spread, especially during summer months. Erosions and/or ulcers had formed on the trunk and limbs a few times per year since 1968. These lesions healed within a month without treatment, leaving either hypoor hyperpigmented macules, and developed to poikiloderma when he was 64 years of age. Simultaneously, he gradually lost the hair on his scalp. His medical history included atrial fibrillation without medication since the age of 50. He had not used any kinds of drugs habitually. Physical examination revealed diffuse erythema with fine scales, based on reticulate pigmentation and teleangiectasia on the trunk, limbs and head. Hyperkeratosis was seen on the palms and soles. The scalp hair was sparse (Fig. 1). The finger and toe nails showed pachyonychia with yellowish discoloration. There were no lesions on the mucous membranes. The results of routine laboratory examinations, including liver function tests, were within normal limits. The results of the following laboratory studies were within normal limits or negative: anti-HTLV-1 antibody, antihepatitis C virus antibody, hepatitis B surface antigen, LE test and uroporphyrine. A skin biopsy from the erythema on the thigh showed wedge-shaped hypergranulosis, irregular acanthosis, damage to the basal cell layer, and bandlike dermal lymphocytes. Interestingly, fatty tissues, reaching to the upper dermis, were seen beneath the band-like zone of inflammation. Below the fatty tissue there were thick collagen deposits through the deep dermis and a few hair follicles. Direct immunofluorescence showed no specific deposits. The findings were highly suggestive of lichen planus, but a provisional diagnosis of parapsoriasis lichenoides was made because of the generalized lesions. Topical applications of diflucortolone valerate and tacalcitol, and topical PUVA therapy to the back, were initiated in March 2000 and had a moderate effect. In April, the patient had a sudden onset of high fever with numerous small vesicles and erosions on the back. These symptoms led us to discontinuation of PUVA therapy. Aciclovir was given without effect and antibodies to herpes simplex and varicella zoster viruses were negative. Direct immunofluorescence of the vesicular skin showed no linear deposits of IgG or C3 in the basal membrane zone, with positive immunostaining for laminin on the floor of the cleft (Fig. 2). Furthermore, immunoblot analysis demonstrated no specific antibodies. Ulcerative lichen planus was diagnosed on the basis of these data. A spiking fever continued and the erosions developed rapidly into irregular-shaped ulcers, widespread over the whole body. The patient was treated with symptomatic therapies and the ulcers began to re-epithelize on the upper trunk. In mid-May, the high fever gradually subsided and the ulcers cleared almost completely. Several erosions and vesicles were noted occasionally, but they did not spread widely during the subsequent 2 months. The patient suffered from recurrent episodes of widespread vesicular lesions in mid-July. In this episode, the patient became bed-ridden due to pain from the ulcers, and had worsening decubitus on the sacral region. The ulcers and general condition improved with unchanged symptomatic therapies in September. However, his condition progressively deteriorated due to