Treatment of psoriasis with cyclosporin. Experience at Johannesburg Hospital.

Ten patients with moderate to severe plaque psoriasis were treated with cyclosporin A (CyA) for 2-19 months (mean 12 months). Initial dosages were 2.5 mg/kg/d in 6 patients and 5.0 mg/kg/d in 4. At 3 months the dosage was increased from 2.5 to 5.0 mg/kg/d in 4 patients in accordance with the study protocol. Subsequent dosages were adjusted according to clinical response and side-effects. Treatment was stopped owing to raised serum creatinine levels in 4 patients on the higher dose; levels returned to normal soon after this. The only other important side-effect was hypertension, which developed in 4 patients, in 2 of whom serum creatinine levels were raised. In all patients the psoriasis improved markedly within 2 months but relapsed, either while the dose of CyA was being tapered off or after treatment with the drug was stopped. Relapse was most rapid in patients with severe initial involvement. On a dosage of 2.5 mg/kg/d there was at least 50% clearance of the psoriasis and no evidence of renal side-effects.

Genodermatoses.

This article describes some of the genodermatoses encountered in the black populations in Africa, starting with ainhum, which appears to be genetically determined. Palmoplantar keratodermas are common in Africa but are difficult to classify and their hereditary nature is not always recognized. Of the ichthyoses, the lamellar type is commonest, with the so-called bathing-suit distribution apparently unique to Africans. Albinism and xeroderma pigmentosum are important causes of cutaneous malignancy in the tropics. Neurofibromatosis and tuberous sclerosis are relatively common, whereas hypermelanosis of Ito is rare but easy to recognize in dark skin.

Self-healing juvenile cutaneous mucinosis in a patient with nephroblastoma.

Self-healing juvenile cutaneous mucinosis is characterized by an early age of onset, the presence of plaques and nodules in a characteristic distribution, and rapid onset followed by spontaneous resolution of the lesions within a period of weeks to months. Only four previous cases have been reported. The patient in this study was an 8-year-old child who developed cutaneous lesions characteristic of self-healing juvenile cutaneous mucinosis while undergoing chemotherapy for a nephroblastoma. This is the first case described of self-healing juvenile cutaneous mucinosis associated with a neoplasm.

Anaemia, iron-related measurements and erythropoietin levels in untreated patients with active leprosy.

The mechanisms responsible for anaemia in leprosy were studied prior to the institution of therapy in 56 patients with active disease. Haematological indices, iron-related measurements, inflammatory markers and erythropoietin levels were assessed, with bone-marrow studies being performed on anaemic patients. Anaemia was more common in the patients with lepromatous leprosy (85.7%) than it was in the rest of the group (19%). The lepromatous group exhibited the disordered iron transport of the anaemia of chronic disorders in that they had a significantly lower mean serum iron level (P less than 0.05), and a mildly raised serum ferritin concentration. Anaemic lepromatous patients also showed a blunted erythropoietin response compared with controls with non-inflammatory anaemia. A subgroup of five anaemic subjects displayed apparently adequate transport of iron to the erythroid marrow (normal percentage transferrin saturations and appropriate sideroblast counts) and the blunted erythropoietin response appeared to be the dominant factor in the pathogenesis of their anaemia. Analysis of inflammatory markers revealed that while the erythrocyte sedimentation rate was very high in the lepromatous subjects, there was no concomitant rise in C-reactive protein concentration. This suggests the presence of a disordered cytokine-mediated acute phase response in the condition.

Chronic subcorneal pustulosis with vasculitis: a variant of generalized pustular psoriasis in black South Africans.

A rare, but distinctive chronic eruption in six female black South Africans is reported. The original diagnosis of subcorneal pustular dermatosis of Sneddon and Wilkinson in these patients was refuted by the subsequent histological observation of both spongiform pustules and an underlying vasculitis. This may represent a previously undocumented form of generalized pustular psoriasis.

Skin ulceration as sole manifestation of Langerhans-cell histiocytosis.

A 65-year-old black woman presented with skin ulceration of 5 years' duration which affected the left axilla, left groin, intergluteal folds and ano-perineal region. The diagnosis of Langerhans-cell histiocytosis (LCH) was confirmed by light and electron-microscopic findings and a positive S-100 stain. Extensive investigations failed to reveal any evidence of systemic involvement. A review of the literature suggests that this clinical picture as the sole manifestation of the disease is rare.

Guttate psoriasis sparing Becker's melanosis--a case report.

A young man with Becker's melanosis developed typical guttate psoriasis following an attack of tonsillitis. The psoriatic lesions spared the area of melanosis, suggesting that there may be local factors within an area of Becker's melanosis which inhibit the development of psoriasis.

Mononuclear leucocyte function in patients with lichen planus and cutaneous lupus erythematosus during chemotherapy with clofazimine.

Mitogen-induced transformation and the production of reactive oxidants by mononuclear leucocytes (MNLs) from patients with chronic dermatological disorders were investigated in vitro before and during the administration of the antimycobacterial/immunosuppressive agent clofazimine (200 mg 3 times weekly). Seven patients, 4 with lichen planus and 3 with cutaneous lupus erythematosus, were included in the study. Clofazimine administration did not influence the mitogen-induced proliferative responses of patients' MNLs. However, chemotherapy with this drug stimulated the production of reactive oxidants by MNLs. Since reactive oxidants are immunosuppressive it is possible that these effects may be involved in the pharmacotherapeutic activity of clofazimine.

Normalization of skin appearance in a patient with scleromyxoedema after intensive chemotherapy for Hodgkin's disease.

A patient with scleromyxoedema was treated for 6 years with cytostatic drugs. During this time the skin lesions followed a fluctuating but progressive course. After 6 years she developed Hodgkin's lymphoma of the mixed cellularity type. Intensive cytostatic treatment given for Hodgkin's disease resulted in virtually complete disappearance of the scleromyxoedema lesions. The development of Hodgkin's disease is considered fortuitous and not due to the previous cytostatic drugs.

Malignant atrophic papulosis (Degos' disease)--diffuse involvement of brain and bowel in an African patient.

We report pathognomonic skin lesions of malignant atrophic papulosis, accompanied by lethal central nervous system and gastrointestinal disease in a 22-year-old black man. Histopathological studies of the brain and colon showed areas of infarction and an underlying vasculitis with leukocytoclasis.

Necrotizing folliculitis in AIDS-related complex.

A clinically mild recurrent rash in a young male homosexual with acquired immune deficiency syndrome (AIDS)-related complex was found on histological examination to consist of a severe necrotizing folliculitis with underlying vasculitis. Follicular eruptions are amongst the seemingly banal dermatological disorders which may accompany AIDS or its prodromes.

Serological and virological evidence of human T-lymphotropic virus in systemic lupus erythematosus.

We present evidence that systemic lupus erythematosus (SLE) patients, but not other mixed connective tissue disease patients, have been exposed to a retrovirus similar or identical to known human T-lymphotropic viruses (HTLV). Serological studies demonstrated a high incidence of seropositivity in SLE patients to HTLV-I antigens by indirect immunofluorescence and Western blot analysis, and to HTLV-III by Western blot analysis. Furthermore, peripheral blood mononuclear cells from four patients with SLE cultivated in vitro expressed HTLV-I antigens after 3 days or more in culture, and reverse transcriptase activity was detected in supernatants from short-term cultures.

Indirect immunofluorescence microscopy of lichen planus.

Indirect immunofluorescence microscopy using serum and lesional skin revealed a lichen planus specific antigen (LPSA) in 80% of patients with lichen planus. This antigen is found only in the stratum granulosum and stratum spinosum. It was demonstrated in skin lesions in Caucasians and Negroes in South Africa and the U.S.A. but was not found in the skin of normal people or patients with other dermatoses. Indirect immunofluorescence should prove useful in distinguishing atypical forms of lichen planus from other dermatoses.

Serum from lichen planus patients reacts with fetal skin tissues.

Lichen planus specific antigen (LPSA) is a lesion-specific marker that differentiates lichen planus from other dermatoses. Serum from lichen planus patients reacted in the indirect immunofluorescent test with fetal epidermis. The reaction was not observed between serum from people with no clinical history of lichen planus and fetal skin tissues. Adsorption of lichen planus serum with a fetal skin extract removed the antibody to LPSA. Adsorption with equivalent amounts of adult normal skin did not remove the serologic activity of LP serum to fetal skin. ELISA test serum from lichen planus patients bound to fetal skin antigen extract to a greater extent than did serum from normal individuals or from patients with non-lichen planus dermatoses, although the quantity of antibody bound in these three groups was not significantly different.