S.M. Burge

Cutaneous manifestations of systemic lupus erythematosus

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, Raynauds 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. Raynauds 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.

Bullous systemic lupus erythematosus: revised criteria for diagnosis

Summary Blistering in systemic lupus erythematosus has been divided into three groups.1 A specific subgroup of ‘bullous systemic lupus erythematosus’ has been defined by Gammon et al. on the basis of a number of criteria.2 From our experience of seven patients with bullous systemic lupus erythematosus, and after reviewing the literature, we suggest that the current classification is too narrow. Our patients displayed clinical and immunohistolegical (based on direct and indirect immunofluorescence and Western immunoblotting) heterogeneity. Sera from two patients bound to epidermal epitopes in sodium chloride‐split skin, but immunoblotting was negative. In neither of these patients could the target antigen be type VII collagen, the only antigen identified as pathogenic in this disease. Patients with epidermal binding should not be excluded from a diagnosis of bullous systemic lupus erythematosus. SLE is a disease in which there is a genetic predisposition to form antibodies to type VTI collagen, along with other autoantibodies, many of which may be implicated in blistering. We suggest that the criteria for the diagnosis of BSLE should be revised. We define this disease as an acquired subepidermal blistering disease in a patient with SLE, in which immune reactants are present at the basement membrane zone on either direct or indirect immunofluorescence.

Handicap in Darier's disease and Hailey-Hailey disease

One hundred and thirty‐seven patients with Dariers disease and 66 patients with Hailey‐Hailey disease completed the Dermatology Life Quality Index (DLQ1). The average DLQI score for Dariers 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 physicians 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 Dariers disease and Hailey‐Hailey disease maintain good relationships at work and home and seem to be able to adapt to their skin disease.

Topical isotretinoin in Darier's disease.

Topical 0·05% isotretinoin (Isotrex ® gel) was used to treat a test patch of skin in 11 patients with Dariers disease. Hyperkeratosis and papules improved in six patients after treatment for 3 months. Erythema, burning and irritation were common adverse effects, and these were severe in three patients, one of whom stopped treatment. Patients with mild Dariers disease may find topical isotretinoin helpful, but it is likely that most patients with widespread disease will require treatment with systemic retinoids.

Comedonal Darier's disease

Dariers disease is an inherited disorder with well‐recognized patterns of presentation. Lesions commonly affect the trunk and flexures. The diagnosis is based on the typical clinical appearance and histology showing acantholytic dyskeratosis. We report two unusual cases with prominent nodular, comedonal lesions on the face and scalp.

Solar urticaria in an infant

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.

Breast abscess due to Corynebacterium striatum

Corynebacterium striatum, a normal constituent of the skin flora, is rarely pathogenic. Previous reports of infection are few, and are mainly confined to immunosuppressed patients or those with indwelling prosthetic devices. We report a case in which the organism caused a recurrent breast abscess in a woman with normal immune function. The only previous reports of Corynebacterium striatum mastitis have been in cows.

Cantharidin-induced acantholysis in Darier's disease : does acantholysis initiate dyskeratosis ?

We have examined the action of cantharidin on the skin of patients with Dariers disease, and used immunohistological techniques to determine the distribution of desmosomal components, keratin intermediate filaments, and proteases in cantharidin‐induced blisters. Cantharidin induced acantho‐ lysis, but the presence of acantholysis did not trigger the development of the characteristic warty, dyskeratotic papules in patients with Dariers disease.

Cutaneous tuberculous abscess: a management problem

Summary A 66‐year‐old woman presented with a 6‐week history of an indolent ulcerating lesion on the anterior chest wall. She had a past medical history of pulmonary tuberculosis which had been treated successfully in 1947. Biopsy of the ulcer showed granulomas and acid‐fast bacilli. Cultures grew Mycobacterium tuberculosis, sensitive to all antituberculous drugs. After 7 months of treatment with isoniazid and rifampicin, there was little sign of healing. A sinogram showed a fistula leading into the plombage mass in the left upper lobe. Surgery to remove the plombage and excise the fistula was planned but was rejected by the patient as the risks of this operation are considerable. With continued antituberculous medication the discharge has reduced although the ulcer has not healed.

Subcutaneous morphoea with contracture of the the flexor muscles of the finger.

lymph node in the left side of his neck. No primary tumour could be detected on repeated examination. His plasma IL-6 level was 41-6 pg/ml (normal value: < lOpg/ mil before excision of the neck tumour. After surgical operation, his facial erythema laded and the plasma IL-b level changed from 41-f)pg/ml to < lOpg/ml (normal). The plasma levels of IL-la. IL-2. IL-3. IL-4 and IT,-S were normal. Expression of IL-6 protein and mRNA in ihe tumour cells was examined. Immunohistochemical staining was performed hy using antihuman ll,-6 antibody (C_vtokine Research Producers. Cambridge. U.S.A.) and Histotine SAB-PO kit INichirei Corp.. Tokyo. Japan), and in silu hybridization by using IL-6 probe (Genemed Biotechnologies. Inc., South San Francisco. U.S.A.). as described.^ Both IL-6 protein and IL-6 mRNA were positive in tumour cells, while neither IL-6 protein nor mRNA were demonstrated in SCC cells obtained from cutaneotis SCC as the control.