Bibliothek

Notizen: A 2-year-old girl presented with a history of an erythematous rash which occurred immediately after exposure to sunlight and had been a problem since birth. Extensive laboratory investigations to exclude genophotodermatoses. photosensitivity secondary to metabolic disorders and photoaggravated dermatoses were negative. Monochromator irradiation phototesting demonstrated immediate erythematous flares to all ultraviolet B (UVB). UVA and visible wavelengths up to 500 nm. A diagnosis of solar urticaria was made and she responded to loratidine 10 mg daily. We believe this is the first report of solar urticaria. confirmed by phototesting with a monochromator so early in life.

Notizen: One hundred and thirty-seven patients with Darier's disease and 66 patients with Hailey-Hailey disease completed the Dermatology Life Quality Index (DLQ1). The average DLQI score for Darier's disease was 5.89 (19.6% of the maximum score), median = 4, quartiles: 1.9. The average DLQl score for Hailey-Hailey disease was 6.06 (20.2% ofthe maximum score), median = 5, quartiles: 2.9. The DLQf score did not correlate with the physician's assessment of clinical severity in both diseases, thus emphasizing the importance of remembering the potential for handicap even in patients with disease that seems to be mild. The domains of the questionnaire with the highest mean scores in both diseases were for questions relating to symptoms and feelings. Domains relating to personal relationships and work obtained low mean scores even in severe disease. It is encouraging that most patients with Darier's disease and Hailey-Hailey disease maintain good relationships at work and home and seem to be able to adapt to their skin disease.

Notizen: We have assessed the cutaneous signs in 73 patients with systemic lupus erythematosus (SLE), seen during a 5-year period in an English hospital. Most previous information about the cutaneous manifestations of SLE has been obtained from studies performed in the U.S.A. We classified lesions as specific cutaneous and mucosal LE (acute, subacute and chronic) or non-specific LE-related, e.g. photosensitivity, urticaria, erythema, Raynaud's phenomenon or vasculitis. Acute cutaneous LE lesions included a butterfly rash with erythematous macules, telangiectasia or papulosquamous lesions, seen in 37 patients (51%) and facial oedema seen in four patients (5%). Five patients (7%) had psoriasiform subacute cutaneous LE, Chronic cutaneous LE was common: 18 patients (25%) had chronic discoid lesions (DLE) and, in 12 (15%), these had preceded systemic disease. One patient had facial lupus profundus. Ten patients (14%) had scarring alopecia secondary to DLE. Fifteen patients (20.5%) had chronic chilblain lupus. Twenty-three patients (31.5%) had a history of mouth ulceration. Of these, 11 (15%) gave a history of ulcers at the onset of their disease. Three (4%) had erythema and superficial ulceration of the palate, not typical of aphthous ulcers, and three (4%) had chronic buccal plaques. Cheilitis due to DLE was seen in three (4%), episcleritis in three (4%), five (7%) had nasal disease, six (8%) bullous skin eruptions, one ‘the bullous eruption of SLE’, four bullae associated with cutaneous vasculitis, and one bullae associated with ultraviolet radiation. Forty-six (63%) observed photosensitivity. A non-scarring alopecia occurred in 29 (40%). Vascular phenomena were common: three patients (4%) had chronic palmar erythema. Raynaud's phenomenon occurred in 44 patients (60%), chronic urticaria, worsened by sun exposure, was noted by 32(44%) (in whom the lesions often lasted more than 36h), eight (11%) had cutaneous vasculitis and three (4%) livedo reticularis.Skin changes play a prominent part in SLE and may provide helpful diagnostic information. In this British population, chilblains and urticaria were particularly common. Lesions of subacute cutaneous LE were relatively unusual in this group of patients with SLE.

Notizen: A patient with cicatricial pemphigold and a widespread nail dystrophy is described. This association has not previously been reported.

Notizen: Summary A 48-year-old man presented with a history of recurrent erylhematous lesions on the trunk and limbs. Examination suggested a fixed drug eruption and this was confirmed by oral challenge with paracetamol. The histology was unusual as it showed a teucocytoclastic vasculitis. Patients may have difficulty avoiding the offending drug because many drugs bought over the counter have similar names but different constituents. In addition, it may be difficult lo persuade the patient that a specific drug is responsible for their skin problem.