F. Wojnarowska

Mucosal involvement in systemic and chronic cutaneous lupus erythematosus

The prevalence and pattern of mucosal involvement in 121 patients with lupus erythematosus (LE) was investigated. Fifty‐three patients had systemic LE (SLE) and 68 patients had chronic cutaneous LE (CCLE). Twenty‐one per cent (11/53) of patients with SLE and 24% (16/68) of patients with CCLE had signs of mucosal involvement, but the pattern of involvement differed in the two groups. Nasal mucosal lesions were a feature in 2% (1/53) of patients with SLE and 9% (6/68) of patients with CCLE. Hyperkeratotic lichen planus‐like plaques on buccal mucosa and the palate occurred in 9% (6/68) of patients with CCLE and 4% (2/53) of patients with SLE. Episcleritis occurred in 9% (5/53) of patients with SLE and was not seen in CCLE. Erythematous plaques on the lower eyelids were present in 6% (4/68) of patients with CCLE and these were associated with conjunctival scarring in two patients. Vulval lesions were present in 5% (2/42) of female patients with CCLE. Oral plaques may occur when the disease is relatively quiescent elsewhere. The prevalence of mucosal involvement in lupus is underestimated as the lesions may be asymptomatic.

Autoimmune progesterone dermatitis responding to Tamoxifen

A case of autoimmune progesterone dermatitis is described. Exacerbation occurred premenstrually and after intramuscular and oral challenge with synthetic progesterone. The condition failed to respond to oestrogen, but there has been a marked improvement with the antioestrogen drug Tamoxifen.

Reactive perforating collagenosis: light, ultrastructural and immunohistological studies.

Reactive perforating collagenosis is an uncommon disorder and few accounts refer to ultrastructural features. This report includes a study by light and transmission electron microscopy of serially sectioned biopsies from early lesions in two patients. Immunohistological investigations utilizing antibodies to basement membrane, laminin, collagen and cytokeratin were also done. Collagen and elastin were demonstrated within the centre of the lesions and there was a defect in the basal lamina at the base of the lesion. The collagen, cytokeratin and the basal lamina in the lesions were antigenically similar to those in the surrounding normal skin. These results are compared with previous findings and discussed in the light of the current views on the pathogenesis of this disorder.

The lupus band test in oral mucosa, conjunctiva and skin

The prevalence and clinical significance of subepithelial immunoglobulin and complement deposition (the lupus band) were examined in the uninvolved sun‐protected skin of the forearm, the uninvolved sun‐protected lip mucosa and sun‐protected bulbar conjunctival mucosa in systemic lupus erythematosus (SLE) and chronic cutaneous lupus erythematosus (CCLE). In SLE, linear deposition of an immunoreactant at the BMZ was detected in 32% (6/19) of skin biopsies; 21% (4/19) of lip mucosal biopsies and 42% (5/12) of conjunctival biopsies. There was no significant difference in the sensitivity of the test at different sites in SLE and no correlation between a positive test in skin, lip or conjunctiva and clinical mucosal involvement. In CCLE, linear deposition of an immunoreactant at the BMZ was found in 3% (1/32) of skin biopsies; 3% (1/29) of lip mucosal biopsies and 50% (10/20) of conjunctival biopsies. There was no correlation between a positive test in skin, lip or conjunctiva and clinical mucosal involvement. In the conjunctiva, IgG was present in all but one of the biopsies and was the only immunoreactant in 90% (9/10) of positive CCLE biopsies and 60% (3/5) of positive SLE biopsies. In lupus erythematosus immunoreactants may be deposited in the basement membrane zone beneath non‐keratinizing mucosal surfaces of the lip and the eye as well as the skin. In CCLE, the test may be positive in conjunctiva when skin and lip are negative.

42) Benign mucous membrane pemphigoid and penicillamine

when he stopped his treatment. Signs of extra-pyramidal dysfunction developed without any evidence of intellectual impairment. At the age of 15 penicillamine was restarted at an increased dose of 15 g/day. At the age of 20, after an approximate total cumulative dose of 4 5 kg of penicillamine, haemorrhagic plaques developed over pressure areas. Examination. Haemorrhagic plaques studded with milia were present over pressure areas on his elbows (Fig. i), knees, knuckles and shoulders. Extensive atrophic striae could be seen on his back and flanks. Histology. The upper dermal collagen showed degeneration and was infiltrated by a conspicuous granulomatous mixed inflammatory cell infiltrate, including multinucleate giant cells as well as lymphocytes eosinophils and plasma cells. Keratin-inclusion cysts were present in the epidermis. Van Gieson-stained elastin sections showed a diminution of collagen particularly, but also of elastin in the upper dermis. Electron microscopy confirmed the diminished amount of elastin in the epidermis, but the upper dermal collagen present appeared normal and did not show the extreme variations of thickness of individual fibres that has been described in this condition previously. Comment. The haemorrhagic plaques are typical examples of penicillamine dermopathy (Sternlieb & Scheinberg, 1964). They occur after long-term high dose penicillamine treatment, such as is given for Wilsons disease or cysteinuria. Excessive skin wrinkling and striae are also documented after penicillamine therapy and may result from inhibition of a copper-dependent lysyl oxidase causing deficient collagen and elastin cross-linkage (Katz, 1967).

Haemorrhagic pompholyx as a sign of bullous pemphigoid and as an indication for low‐dose prednisolone therapy

Four patients are described who presented with haemorrhagic pompholyx as a sign of bullous pemphigoid. Three of these patients initially had lesions localized to the hands and feet. The diagnosis of bullous pemphigoid was made by histopathological and immunofluorescent examination. All four patients have responded well to low‐dose prednisolone (20 mg) therapy.

Reactive perforating collagenosis

This uncommon condition was first described by Mehregan, Scwartz and Livingood (1967). It is one of the true perforating disorders where collagen is extruded through the epidermis and it is believed that superficial trauma, in genetically predisposed individuals, leads to this skin response.