Monika Sonntag

Phototesting in lupus erythematosus tumidus--review of 60 patients.

Photosensitivity is an important characteristic feature of several forms of lupus erythematosus (LE), and induction of skin lesions by UV‐A and UV‐B irradiation has been proved to be an optimal model for evaluating light sensitivity in patients with this disease. Because lupus erythematosus tumidus (LET) has rarely been documented in the literature and is often difficult to differentiate from other photodermatoses such as polymorphous light eruption, we performed photoprovocation tests in 60 patients with LET according to a standardized protocol. Areas of uninvolved skin on the upper back were irradiated with single doses of UV‐A (100 J/cm2) and/or UV‐B (1.5 minimal erythema dose) daily for three consecutive days. Interestingly, patients with LET are more photosensitive than those with subacute cutaneous lupus erythematosus, and in our study experimental phototesting revealed characteristic skin lesions in 43 patients (72%). Because of the latency period in developing positive phototest reactions, it might be difficult for these patients to link sun exposure with their skin lesions. Furthermore, our data revealed a positive correlation of antinuclear antibodies and positive provocative phototest reactions in these patients as seen for other forms of LE. In conclusion, the high incidence of positive phototest reactions in correlation with the clinical findings, history of photosensitivity and antinuclear antibodies enable the classification of LET as the most photosensitive type of LE.

Upregulation of epidermal surface molecule expression in primary and ultraviolet-induced lesions of lupus erythematosus tumidus.

Summary Background Lupus erythematosus tumidus (LET), a photosensitive skin disorder with characteristic clinical and histological features, has not been generally accepted as a subset of cutaneous lupus erythematosus (CLE).

Characterization of the inflammatory infiltrate and expression of endothelial cell adhesion molecules in lupus erythematosus tumidus

Abstract. Lupus erythematosus tumidus (LET) is a disease with characteristic clinical and histopathologic features that has not always been considered a subset of cutaneous lupus erythematosus (CLE). Although LET was first mentioned in the literature in 1930, it has rarely been documented, and immunohistochemical studies have never been performed. The aim of the present study was to characterize the inflammatory infiltrate and to analyze the expression of endothelial cell adhesion molecules in skin specimens from patients with LET and to compare the results with those from patients with other variants of CLE, such as discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE). Cryostat sections of lesional skin specimens from ten patients with LET demonstrated an infiltrate composed of more than 75% CD4+, CD8+, and HLA-DR+ cells. Interestingly, CD45RO+ cells, in contrast to CD45RA+ cells, were the prevailing inflammatory cell population. Compared with skin specimens from patients with DLE and SCLE, the mean expression of CD4+ and CD8+ cells was higher (but not significantly so) in LET, and no differences were observed with the other three antibodies. Furthermore, in contrast to controls, intercellular adhesion molecule-1, vascular adhesion molecule-1, E-selectin, and P-selectin showed the same expression pattern in skin specimens from patients with DLE, SCLE, and LET. In conclusion, the inflammatory infiltrate of LET primarily consists of CD4+/CD8+ lymphocytes. Furthermore, expression of endothelial cell adhesion molecules was equally upregulated in LET compared with the expression in DLE and SCLE, suggesting a similar immunopathomechanism of these subtypes of CLE.

Lupus erythematosus tumidus in childhood. Report of 3 patients.

Lupus erythematosus (LE) is a rare disorder in childhood. Most patients reported in the literature present with systemic manifestations, and, to date, fewer than 20 children with chronic cutaneous LE have been documented. In this article, we describe 3 patients with childhood LE tumidus, an uncommon but distinct subtype of chronic cutaneous LE. The lesions are characterized by erythematous, urticaria-like, nonscarring plaques in sun-exposed areas and, unlike in other variants of chronic cutaneous LE, there is no epidermal involvement. The clinical, photobiological, and histologic features as well as the differential diagnoses of childhood LE tumidus are discussed and compared with the adult form of this disease.

Papulonodular Mucinosis Associated with Subacute Cutaneous Lupus erythematosus

Mucin deposition is a common histopathologic finding in lupus erythematosus (LE) but is rarely present in sufficient quantity to produce clinically apparent skin lesions. Until now, fewer than 40 cases of papulonodular mucinosis associated with LE have been reported in the literature, and it was associated with either systemic LE or discoid LE. For the first time, to our knowledge, we describe 2 patients with subacute cutaneous LE who presented with papulonodular mucinosis as a major clinical manifestation of their disease.

Botulinumtoxinbehandlung eines ekkrinen Schweißdrüsennävus

1. Gorlin RJ, Goltz RW (1960) Multiple nevoid basalcell epithelioma, jaw cysts and bifid rib. A syndrome. N Engl J Med 262:908–911 2. Horn M, Wolf P, Wulf HC et al. (2003) Topical methyl aminolaevulinate photodynamic therapy in patients with basal cell carcinoma prone to complications and poor cosmetic outcome with conventional treatment. Br J Dermatol 149:1242–1249 3. Kimonis VE, Goldstein AM, Pastakia B et al. (1997) Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 69:299–308 4. Reifenberger J (2004) Hereditary tumor syndromes. Cutaneous manifestations and molecular pathogenesis of Gorlin and Cowden syndromes. Hautarzt 55:942–945 noide aufgrund ihrer proliferationshemmenden und differenzierungsfördernden Eigenschaften eingesetzt. Aufgrund des bestehenden Kinderwunsches mussten wir bei unserer Patientin jedoch hiervon absehen. Ferner ist den Patienten ein konsequenter UV-Schutz anzuraten [4].

Treating excessive sweating with poison

A 23-year-old woman presented with hyperhidrosis localised to her right forearm and the back of her right hand. She had experienced hyperhidrotic attacks since childhood. They had become more frequent and troublesome over the years, and now occurred on average fi ve times a day, lasting about 30 min. The excessive sweating was embarrassing for our patient; her hand literally dripped with sweat, aff ecting her work. Attacks occurred spontaneously or were precipitated by emotional stress, exercise, alcohol, or coff ee. The skin was of otherwise unremarkable appearance. The patient had no other symptoms and was otherwise healthy. Treatment with topical aluminium chloride and tap-water iontophoresis over the previous 2 years had not provided relief. A skin biopsy specimen showed increased numbers of eccrine sweat glands without any other abnormality, consistent with an eccrine naevus (fi gure). The problem resolved following intradermal injections of botulinum toxin, as confi rmed by repeated Minor’s iodine-starch tests over a period of 6 months. The treatment was well tolerated and the patient’s quality of life appreciably improved. In recent years, botulinum toxin has been used as a therapeutic option for hyperhidrosis. These photographs illustrate the profound eff ects of botulinum toxin injections in focal hyperhidrosis caused by an eccrine naevus. Lancet 2007; 369: 1372