Complete response of deep neutrophilic dermatosis associated with myelodysplastic syndrome to 5-azacytidine.

Cutaneous manifestations of myelodysplastic syndromes (MDS) may predict disease progression and a poorer prognosis. We describe a patient in whom a deep neutrophilic dermatosis preceded evolution of disease from refractory anaemia to RAEB (refractory anaemia with excess blasts) and resolved completely on treating the disease with 5-azacytidine. The dermatological response was accompanied by complete bone marrow remission and trilineage haematological improvement. We suggest that 5-azacytidine should be considered in the treatment of immune mediated cutaneous manifestations of MDS.

Sclerodermatous graft-versus-host disease: clinical spectrum and therapeutic challenges.

Sclerodermatous graft-versus-host disease (GVHD) is a rare complication of bone marrow transplantation. While GVHD is often associated with the beneficial graft vs. tumour effect, it also contributes towards significant morbidity and mortality. No reliably effective treatment has yet been established. We present 10 patients with haematological malignancies who underwent an allogeneic stem cell transplant and developed sclerodermatous GVHD. Donor lymphocyte infusion administered for relapse or reducing donor T-cell chimerism was a known trigger for sclerodermatous GVHD in four of the patients. Treatment with immunosuppressants, psoralen plus ultraviolet A (PUVA) and extracorporeal photopheresis has been largely unsuccessful in their management. Intensive immunosuppression including the use of anti-CD20 monoclonal antibody may have contributed to relapse of leukaemia in one patient 10 years after her transplant. Sclerodermatous GVHD may occur without a preceding lichenoid stage. Clinical heterogeneity is common, although sclerodermatous GVHD has a predilection for the limbs. Treatment options are largely unsatisfactory if conventional immunosuppression fails. PUVA may give some symptomatic benefit and extracorporeal photopheresis seems to be less efficacious than previously published work suggests.

Novel treatment of Sézary-like syndrome due to adult T-cell leukaemia/lymphoma with daclizumab (humanized anti-interleukin-2 receptor alpha antibody).

We describe a patient with erythrodermic adult T-cell leukaemia/lymphoma resistant to multiple systemic therapies who, on the commencement of daclizumab, a humanized anti-interleukin-2 receptor antibody, developed a rapid and sustained complete response with resolution of previously debilitating erythroderma, suggesting significant activity of this agent in this disease process.

Koebnerizing sclerodermatous graft-versus-host disease caused by donor lymphocyte infusion and interferon-alpha.

Graft-versus-host disease (GvHD) is a common sequel to allogeneic bone marrow transplants, which may be accompanied by desirable graft-versus-tumour effects. Sclerodermatous GvHD is a rare subtype that is very difficult to treat. We report the first case of sclerodermatous GvHD as part of the Koebner phenomenon. We propose that donor lymphocyte infusion and interferon-alpha were involved in the pathogenesis of this case.

Cutaneous manifestations of granulocyte colony-stimulating factor.

Granulocyte colony-stimulating factor (GCSF) is a recombinant human growth factor widely used in haematology. It is known to cause cutaneous vasculitis and neutrophilic dermatoses. We present three cases of Sweet's syndrome (SS) associated with GCSF use. Raised GCSF levels have been demonstrated in patients with SS. GCSF is the best understood mechanism by which neutrophil accumulation occurs and shows a dose-dependent effect in provoking SS.

Spectrum of clinical presentation, treatment and prognosis in a series of eight patients with leukaemia cutis.

All types of leukaemia can disseminate to the skin, producing cutaneous deposits known as leukaemia cutis (LC). We undertook a retrospective study to review the clinical presentations, treatment and outcome of eight patients with LC managed in our department over a period of 12 years. The clinical phenotype varied, with erythematous papules and nodules occurring with greatest frequency. Infiltrated haemorrhagic plaques and perifollicular acneiform papules were also seen. Although patients were treated aggressively for their underlying leukaemia, and received therapy directed towards LC, they tended to be refractory to treatment and the diagnosis was generally associated with a poor prognosis. The exception was a patient with chronic lymphocytic leukaemia, who survived 3 years after developing LC.

Trends in the presentation of cutaneous malignant melanoma over three decades at King's College Hospital, London.

The aim of this study was to examine trends in the presentation of cutaneous malignant melanoma at King's College Hospital (KCH) over the last three decades (1970-2000). KCH was one of seven centres that participated in the 1987 Cancer Research Campaign (CRC) publicity campaign aimed at promoting earlier self-recognition of melanoma. Data included patient age at presentation, sex, tumour site, Breslow thickness and histological subtype. The late 1980s saw a threefold increase in the annual number of melanomas and an eightfold increase in thin melanomas compared to the 1970s. The increase occurred in both sexes and was particularly marked after the CRC campaign but numbers had already begun to increase prior to this. The increase has predominantly been thin (Breslow < 1.5 mm) tumours of the superficial spreading variety with a resultant fall in mean Breslow thickness. There has been a decline in the annual number of melanomas since the peak in 1992 which is not explained by increased proportion of in situ tumours. The CRC campaign may have contributed to the documented increase in thin tumours but this trend had begun prior to 1987 suggesting factors other than public awareness and earlier presentation are important. It is encouraging that the number of melanomas has declined over the last 5 years at KCH but it is yet to be seen whether this reflects a real decrease in the incidence of melanoma.

Haemorrhagic bullae in a case of paroxysmal nocturnal haemoglobinuria.

Paroxysmal nocturnal haemoglobinuria is a clonal stem cell disorder manifesting as haemolysis, bone marrow failure and thrombosis. We report a rare cutaneous manifestation on the trunk of a 40-year-old patient. Skin biopsy revealed the presence of fibrin thrombi in the capillaries. The eruption responded within days after commencing oral corticosteroids.

Skin disease in Gulf war veterans.

BACKGROUND: Gulf war veterans report more symptomatic ill-health than other military controls, and skin disease is one of the most frequent reasons for military personnel to seek medical care. AIM: To compare the nature and prevalence of skin disease in UK Gulf veterans with non-Gulf veterans, and to assess whether skin disease is associated with disability. DESIGN: Prospective case comparison study. METHODS: Disabled (n=111) and non-disabled (n=98) Gulf veterans and disabled non-Gulf veterans (n=133) were randomly selected from representative cohorts of those who served in the Gulf conflict 1990-1991, UN Bosnia Peacekeeping Force 1992-1997, or veterans in active service between 1990-91, but not deployed to the Gulf. Disability was defined as reduced physical functioning as measured by the Short Form 36 [score <72.2]. All subjects recruited were examined by a dermatologist, blind to the military and health status of the veteran. RESULTS: The prevalences of skin disease in disabled Gulf, non-disabled Gulf and disabled non-Gulf veterans were 47.7, 36.7, and 42.8% respectively. Seborrhoeic dermatitis was twice as common as expected in the Gulf veterans (both disabled and non-disabled). DISCUSSION: Skin disease does not appear to be contributing to ill health in Gulf war veterans, with the exception of an unexplained two-fold increase in seborrhoeic dermatitis.

Black Skin Disease in South East London

Skin disease in black patients can present a diagnostic challenge both because of variations in clinical appearance and because certain disorders appear unique to black skin. The majority of dermatology text books and atlases concentrate mainly on white skinned patients. Our hospital serves a cosmopolitan local community in whom 40 percent of the population is black. We recorded the diagnosis made in 461 consecutive black (African, Afro-Caribbean and mixed-race) patients who attended a general dermatology new patient clinic in South East London. The aim of the study was to document the spectrum of skin disease seen in black patients and to highlight these disorders. 187 children and 274 adults were studied. In the black children atopic eczema was diagnosed in 38 percent, tinea capitis 26.7 percent, pityriasis alba 3.7 percent, viral warts 3.2 percent, keloids 2.7 percent, others 25.7 percent. Diseases recorded which are not seen in the white children included Facial Afro-Caribbean Childhood Eruption (FACE) and infantile acropustulosis. Of the 274 adults assessed, acne was diagnosed in 14.6 percent, eczema 10.2 percent, psoriasis 5.1 percent, pityriasis versicolor 4.0 percent, alopecia areata 3.3 percent, dermatofibroma 2.9 percent, urticaria 1.8 percent, pityriasis rosea 1.8 percent, cutaneous sarcoid 1.5 percent, discoid lupus 1.1 percent. Diseases more commonly seen in black skin were acne nuchae keloidalis/scalp folliculitis 14.6 percent, keloids 4.4 percent, post inflammatory pigmentation 3.6 percent and traction alopecia 1.1 percent; other disorders were seen in 30 percent. Normal variants recorded were dermatosis papulosa nigra, linear nail pigmentation and palmar pits. As the UK population becomes increasingly cosmopolitan, familiarity with skin disorders in both black and white skin is increasingly relevant to our practice. (AU)