E. Wilson Jones

“ACTINIC RETICULOID”: A CHRONIC DKHMATOSIS ASSOCIATED WITH SEVERE PHOTOSENSITIVITY AND THE HISTOLOGICAL RESEMBLANCE TO LYMPHOMA

SUMMARY.— Ten elderly male patients with severe persistent photosensitivity of unknown cause are described. The clinical presentation was that of an intense eczematous eruption affecting exposed sites but spreading elsewhere. Frequent episodes of erythroderma were characteristic and in some cases the marked thickening and ridging of the exposed skin simulated a lymphoma. In contrast to most other photodermatoses the photosensitivity extended through the u.v. to the visible spectrum. A wide range of histological changes were noted, in several instances closely resembling lymphoma because of the intensity and pleomorphism of the infiltrate.

Histiocytoma cutis: a tumour of dermal dendrocytes (dermal dendrocytoma)

The histogenesis of histiocytoma (dermatofibroma) was investigated using new antibodies that demonstrate factor XIIIa (FXIIIa) positive cells and the monocyte macrophage cell series (MAC 387), in formalin fixed tissue. The distribution of S100 protein, vimentin and Ulex europaeus agglutinin 1 (UEA‐1) were also studied. The antibody against FXIIIa labelled the normal dermal population of fixed connective tissue cells (dermal dendrocytes) emphasizing their dendritic processes; cells that are widely distributed, but are most numerous in the papillary dermis. In contrast, the antibody, MAC 387 against monocyte derived macrophages, did not label this cell population.

Dermatofibrosarcoma protuberans: a clinicopathological and immunohistochemical study with a review of the literature

Forty‐one cases of dermatofibrosarcoma protuberans are presented. The clinical features and histopathological appearances are described. Immunohistochemical staining of thirteen cases with antisera to lysozyme, α1‐antichymotrypsin and S‐100 protein has provided no evidence to support either a histiocytic or neuroectodermal origin for these tumours. In reviewing the literature, the histogenetic origin, differential diagnosis and malignant potential of dermatofibrosarcoma protuberans are discussed.

Kaposi's sarcoma and immunosuppressive therapy: an appraisal.

Kaposis sarcoma can probably be added to the list of tumours (lymphomas and squamous cell epitheliomas) that are especially prone to arise as a result of immunosuppressive therapy. Sixteen examples of this complication have been described in the literature and we report three further instances. These three patients had been on treatment with prolonged steroid therapy, but many of the previosly reported patients had been on aggressive immunosuppressive regimes to prevent rejection of kidney transplants. Kaposis sarcoma associated with steroid and immunosuppressive therapy may show enhanced incidence in women (7 females as against 12 males) remembering that the spontaneous disease occurs ten times more frequently in men than women. We discuss the implications of these findings against the background of the high incidence of Kaposis sarcoma in parts of Africa where immunological derangement consequent on malaria may be significant.

Epithelioid cell histiocytoma—a new entity

This is a report of a previously undescribed type of histiocytoma distinguished clinically by its occurrence as a solitary elevated nodule often with a vascular appearance and cytologically by the presence of large angulated epithelioid cells. Nineteen lesions from 11 women and eight men have been studied. The lower limb was the commonest site for the nodules. The mean age at presentation was 42 years (range 23–63). Only one lesion recurred after attempted removal. This lesion in the past may, have been mistaken for a Spitz naevus.

Secondary syphilis: a clinico-pathological review

The histological appearances found in biopsies from fifty‐seven patients with secondary syphilis have been correlated with the clinical morphology of the eruptions. Considerable variation of histological pattern was encountered, and the frequency with which some of the classically described changes were found to be absent or inconspicuous is stressed. Of particular interest were the findings that, in nearly one‐quarter of the biopsies, plasma cell infiltration was either absent or very sparse, and that vascular damage was seen in less than half. Where present, the vessel changes were almost entirely confined to swelling of the endothelial cells. Proliferation of the endothelial cells was most uncommon.

PITYRIASIS LICHENOIDES: A rEAPPRAISAL

Summary.— One hundred and twenty‐eight cases of pityriasis lichenoides presenting at St. Johns Hospital for Diseases of the Skin in the past decade have been reviewed. Sixty of these were seen personally, and 44 of these had lesiuns at the time of interview. Eighteen other patients completed proformata, and adequate hospital records were available for the rest. One hundred and two biopsies from 82 patients with pityriasis lichenoides were also examined in the course of this study. The various clinical manifestations seen in this group of patients has been analysed in order to define the clinical range of the Disease. None of the patients studied developed a cutaneous reticulosis and thus the risk for such an occurrence must be small. Patients with pityriasis lichenoides did not appear prone to any systemic complaint, and the disease seemed to be limited to the skin. Some of the patients who had had the disease for several years developed haemorrhagic and necrotic lesions, suggesting that pityriasis lichenoides varioliformis et acuta and pityriasis lichenoides chronica are variants of a single disease process. Altliough small vessel involvement in the inflammatory process was usual, no histological evidence was obtained to suggest that a vasculitis is of central importance in the pathogenesis of the disease.

Juvenile elastoma and osteopoikilosis (the Buschke--Ollendorff syndrome).

Sixteen patients from seven different families with the Buschke‐OUendorff syndrome have been studied. Osteopoikilosis was found in two‐thirds of the patients radiologically examined and all but two had skin involvement. The predominant clinical pattern consisted of grouped skin coloured papules and discs that were distributed asymmetrically and which usually had presented at an early age. The skin lesions showed the characteristic histological changes of juvenile elastoma which, it is suggested, is the specific dermatological abnormality of the Buschke‐Ollendorff syndrome.

Lichen aureus: A localized persistent form of pigmented purpuric dermatitis

Localized persistent pigmented macules and plaques due to a chronic form of pigmented purpuric dermatitis are described in forty‐two patients. The designation ‘lichen aureus’ for this eruption can be justified because of the striking yellowish or bronze‐like colour assumed by many of the lesions. The lower legs were the commonest sites but other body regions can be affected also. Histologically lichen aureus differs from other pigmented purpuric dermatoses in the density of the lichenoid tissue reaction and the marked accumulation of pigment‐containing macrophages. Recently developed endothelial cell markers have been studied in selected cases.

Multinucleate cell angiohistiocytoma: an acquired vascular anomaly to be distinguished from Kaposi's sarcoma

Multinucleate cell atigiohistiocytoma is a newly described benign vascular condition that usually arises on the extremities of women over the age of 40 as discrete grouped violaceous erythematous papules, often mimicking Kaposis sarcoma. Of 10 patients, nine were women aged between 37–66 (average 51.5) years at the onset of their condition. The legs, and in particular the calves and thighs, were the commonest sites to be involved. However, in three patients the papules were confined to the back of the hands, and the male patient had lesions across the front of the chest. Bilateral lesions occurred in four patients. Histologically, the salient features were proliferation of capillaries and small venules at the level of the subpapillary plexus and the mid dermis, in association with prominent connective tissue cells and larger angulated multinucleate cells. Detailed histochemical and immunocytochemical studies have not elucidated the histogenesis of the multinucleate cells that seem to be a characteristic feature of this condition.