1/428. growth hormone insufficiency in a girl with the autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy. Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an inherited disease which may comprise many endocrine and non-endocrine components. GH insufficiency has not been recognised as a classical manifestation of this syndrome. We describe the case of a girl with APECED, who presented with four endocrine (hypoparathyroidism, Addison's disease, hypothyroidism, gonadal failure) and three non-endocrine components (candidiasis, ectodermal dystrophy and lichen ruber planus). In addition, growth failure was documented beginning at approximately 8 years; bone age was delayed and stimulated GH peaks after clonidine and arginine were 2.2 and 9.2 microg/l, respectively. A partial empty sella was found on a computed tomography scan of the hypothalamic-pituitary region. At 10.5 years rhGH therapy was started and height gain of 26 cm was observed after 2.7 years of treatment. puberty started at 11.2 years and menarche occurred at 12.7 years. At 13.25 years rhGH therapy was discontinued owing to frequent hypocalcemic crises; serum IGF-1 levels were persistently low in the following years (between 160 and 180 microg/l, normal range for age 250-600 microg/l). The patient attained a final height of 160.8 cm, which was appropriate for her target height. The presence of lichen ruber planus and GH insufficiency probably secondary to empty sella are two unusual findings in patients with APECED. ( info) |
| Linear lichen planus is a rare distinctive variant of lichen planus (LP) characterized by a pruritic eruption of lichenoid, violaceous papules in a linear distribution that sometimes assume a Blaschko line pattern. We describe a 33-year-old woman who presented with a 4-month history of a slightly pruritic unilateral linear array of papular lesions on the left side of her neck that were clinically and histologically consistent with linear LP. Two months after the onset of her skin disease she developed typical lesions with a lacy white pattern on the left lateral aspect of her tongue and the left buccal mucosa with a striking predominance for the left side. Clinically the lesions on the patient's neck were similar to lichen striatus or lichenoid epidermal naevus, a variant of linear verrucous epidermal naevus. However, the histological features and the fact that later in the course of her disease the patient developed typical LP of the oral mucosa suggest that this patient has the rare condition of linear LP with unilateral restriction. ( info) |
3/428. Erosive mucosal lichen planus: response to topical treatment with tacrolimus. Erosive mucosal lichen planus is a painful and disabling inflammatory skin disease that is highly resistant to topical treatment. We report on six patients with severe recalcitrant erosive mucosal lichen planus who benefited from topical application of tacrolimus ointment. After 4 weeks of treatment, complete resolution was observed in three cases, and substantial improvement was achieved in the other three patients. In these cases, prolonged treatment resulted either in further improvement or in complete healing. All patients reported rapid relief from pain and burning. No severe side-effects were observed. ( info) |
4/428. Demonstration of antibody to 230 kDa bullous pemphigoid antigen in lichen planus-like keratosis. We describe a 67-year-old man with lichen planus-like keratosis associated with anti-230 kDa bullous pemphigoid antigen (BPAG1) autoantibody. The patient had noticed solitary dark brown macule more than 6 years previously on his left chest. Histological findings showed hypergranulosis, irregular acanthosis, liquefaction degeneration of basal cells, band-like infiltration of lymphocytes at the subepidermal portion, and a cleft at the basement membrane zone (BMZ), resulting in the formation of subepidermal blisters. Direct immunofluorescence findings of perilesional skin showed a linear deposition of IgG at BMZ. On indirect immunofluorescent study using normal human skin, circulating IgG autoantibody to BMZ was present in the patient's serum at a titer of 1:80. The antigen located on the epidermal site of normal skin split by 1M NaCl was reacted with the patient's serum. Immunoblot analysis using epidermal extracts demonstrated the presence of IgG antibody directed to BPAG1 in the patient's serum. These observations suggest that the presence of an antibody to BPAG1 could be caused by the damage of basal cells following lichen planus-like keratosis. ( info) |
5/428. lichen planus associated with milia. The formation of milia is well recognized in both bullous and inflammatory dermatoses. There are several reports of milia developing in a rare variant of lichen planus pilaris known as lichen planus follicularis tumidus (LPFT), but the association of milia with other types of lichen planus (LP) has not been documented in the literature. We report five patients who developed milia during the course of either drug-induced or idiopathic LP and one in whom milia developed in a lichenoid tattoo reaction. Milia were noted to occur transiently during the resolving phase of LP. Most cases were severe enough to warrant treatment with systemic steroids. The association of milia with LP is not restricted to the rare clinical variant LPFT. We speculate that a severe lichenoid reaction with basal layer degeneration may precipitate the formation of milia in some cases of LP. ( info) |
6/428. stevens-johnson syndrome. Description of an unusual clinical case due to glucocorticoid therapy for oral lichen planus. erythema multiforme (EM) is an acute inflammatory disease with an autoimmune pathogenesis clinically expressing in a wide variety of mucocutaneous illnesses. It is usually described in a minor form (Von Hebra) characterized by classical cutaneous lesions, and in major form (Stevens-Johnson), involving mucosal damage, while a clinical type restricted to the oral mucosa is described in oral pathology. A considerable number of factors of different nature have been reported as etiologic agents of EM, but most of them are not well documented; however, a certain relationship with EM is recognized for different classes of systemic drugs. This paper describes a case of stevens-johnson syndrome with initial oral involvement, in which the precipitating factor was due to the administration of systemic glucocorticoids, prescribed for the therapeutic treatment of an erosive form of oral lichen planus. ( info) |
7/428. IgG autoantibodies from a lichen planus pemphigoides patient recognize the NC16A domain of the bullous pemphigoid antigen 180. lichen planus pemphigoides (LPP) most likely encompasses a heterogeneous group of subepidermal autoimmune blistering disorders occurring in association with lichen planus. We describe the case of a 49-year-old patient with features characteristic of LPP. Direct immunofluorescence microscopy studies demonstrated linear deposits of C3 along the cutaneous basement membrane, while circulating IgG autoantibodies directed against the epidermal side of skin separated by 1 M NaCl were detected. The patient's serum contained IgG autoantibodies immunoblotting a recombinant form of bullous pemphigoid antigen 180 (BP180), but not the COOH-terminus of BP230. By using deletion mutants, it was found that IgG reactivity was restricted to the NC16A domain of BP180, the region harboring the major antigenic sites targeted by IgG autoantibodies from patients with the bullous pemphigoid group of disorders. Our findings provide support to the idea that a subset of patients with LPP have a distinct form of bullous pemphigoid associated with lichen planus. ( info) |
8/428. lichen planus pemphigoides with IgG autoantibodies to the 180 kd bullous pemphigoid antigen (type XVII collagen). We describe a 75-year-old patient with pruritic papules on her trunk and extremities, typical of lichen planus, who later experienced subepidermal blisters. These clinical features are consistent with lichen planus pemphigoides. Immunofluorescence of perilesional skin showed linear deposits of C3 along the dermoepidermal junction. Circulating IgG autoantibodies were found to be directed against an epidermal component of the dermoepidermal junction because the patient's serum labeled the epidermal side of 1 mol/L NaCl-split skin. The patient's IgG autoantibodies were directed exclusively against the 180 kd bullous pemphigoid antigen (BPAg2, type XVII collagen) detected in human keratinocyte lysate by Western blot assay. No reactivity was found against the 230 kd bullous pemphigoid antigen, type VII collagen, or the laminin-5 subunits. This study demonstrates that BPAg2 is recognized, not only by bullous pemphigoid sera, but also by lichen planus pemphigoides sera. Our findings attest to the similarity of immunopathology in these two subepidermal blistering skin diseases. ( info) |
| PURPOSE: To report two cases of cicatrizing conjunctivitis associated with paraneoplastic lichen planus. methods: case reports. RESULTS: Two patients were examined because of redness and discomfort in both eyes. A 63-year-old woman with follicular, small-cleaved cell lymphoma had cicatrizing conjunctivitis, stomatitis, vulvitis, and skin lesions. A 25-year-old man with malignant thymoma had cicatrizing conjunctivitis, erosive stomatitis, and penile papules. Histopathologic studies of conjunctiva and skin biopsy specimens in the first patient and labial biopsy specimens in the second revealed lichen planus. CONCLUSION: Paraneoplastic lichen planus is a possible cause of cicatrizing conjunctivitis associated with inflammatory skin and mucous membrane disease. ( info) |
| We present a case of lichen planus affecting the oesophagus of an 80-year-old woman. Symptomatically, the lesions manifested themselves as dysphagia requiring repeated oesophageal dilatations. The patient was unable to tolerate several conventional treatments and so pulsed methylprednisolone was given with some beneficial short-term effects. Due to potential for malignant change in lichen planus of the mucous membranes it is important to remember this complication and investigate patients with oesophageal symptoms. ( info) |