D Creamer

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.

Reactive angioendotheliomatosis in association with the antiphospholipid syndrome

A 50-year-old woman with systemic lupus erythematosus developed extensive necrotic skin lesions on her chest and abdomen after the discontinuation of warfarin. The presence of antiphospholipid antibodies suggested a diagnosis of antiphospholipid syndrome. Histopathology from a skin lesion demonstrated marked expansion of the dermal microvasculature by intravascular cellular proliferation and focal thrombosis. The intravascular cells stained positive for the endothelial cell markers CD31 and factor VIII-associated antigen confirming reactive angioendotheliomatosis (RAE). This report is the first to identify RAE occurring in the context of the antiphospholipid syndrome. We suggest that intravascular endothelial proliferation, in concert with thrombosis, contributed to the angio-occlusive pathology.

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

Summary 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.

PNP with Waldenström's macroglobulinaemia

We describe a 65‐year‐old Caucasian man with Waldenströms macroglobulinaemia who developed paraneoplastic pemphigus (PNP) 3 years after his haematological diagnosis. This is a very rare malignancy that is associated with PNP. The evolution of PNP in this patient appears to exhibit the postulated immunological phenomenon of epitope spreading.

Whipple's disease presenting with symmetrical panniculitis

Specific cutaneous involvement in Whipples disease is extremely rare. The condition usually runs a chronic course, with symptoms preceding diagnosis by years or even decades. We report a 44‐year‐old man who presented with a rapid onset of progressive, extensive, symmetrical plaques of panniculitis affecting the inner thighs and forearms. He had accompanying large joint arthritis and was profoundly anaemic. Biopsy of the subcutis revealed a florid septal panniculitis with infiltration of the septa by foamy macrophages containing intracellular granules that stained strongly with periodic acid‐Schiff reagent. A similar but more intense infiltrate was seen in the small bowel lamina propria, and a diagnosis of Whipples disease was made. Symmetrical panniculitis has not previously been reported as a manifestation of Whipples disease.

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

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‐α were involved in the pathogenesis of this case.

Dose-limiting dermatological toxicity secondary to imatinib mesylate (STI571) in chronic myeloid leukaemia

Dose-limiting dermatological toxicity secondary to imatinib mesylate (STI571) in chronic myeloid leukaemia

Subcutaneous calcification presenting in a patient with mixed connective tissue disease and cutaneous polyarteritis nodosa

Subcutaneous calcification often occurs in connective tissue diseases, most commonly scleroderma and dermatomyositis, but is rarely found in mixed connective tissue disease (MCTD). Cutaneous polyarteritis nodosa (PAN) is usually a primary skin disorder and although associated with connective tissue disease, has not been reported previously in MCTD. Calcinosis in cutaneous PAN is not a recognized feature. We describe the case of a 37‐year‐old woman who presented with tender ulcerated subcutaneous nodules on the lower legs consistent with cutaneous PAN, and she also showed features of MCTD with extensive secondary subcutaneous calcification. The use of systemic immunosuppressive treatment has improved the clinical features of PAN and MCTD but treatment of the calcification has proved challenging. No single medical or surgical treatment has been shown to be consistently effective in subcutaneous calcification, but the introduction of diltiazem in our patient has resulted in some improvement.

Leucoderma and leucotrichia in association with chronic cutaneous graft-versus-host disease

SIR, We read with interest the timely correspondence from Bassi et al. and wish to report a third case of infliximab-induced acne. Our patient, a 40-year-old woman with Crohn disease diagnosed in 2001, had had five previous laparotomies including a subtotal colectomy and ileo-anal pouch for severe colitis. No history of acne was noted and she was otherwise well. The patient had received short courses of prednisolone in 2002 and 2005. She had also been taking 6-mercaptopurine and ciprofloxacin, both at varying doses, since 2005. In 2002, the patient was commenced on infliximab 5 mg kg with rapid and effective symptom control. Infliximab has been given intermittently since then, as required, for further control. Several weeks after starting infliximab, the patient developed a papulopustular eruption on the face and back with some comedones. The eruption persisted, albeit with fluctuating severity, and dermatological opinion was obtained in 2006. Histology was consistent with our clinical diagnosis of acne and excluded other Crohn disease-associated dermatoses. The patient was anaemic (haemoglobin 10Æ6 g dL with a mean corpuscular volume of 100 fL) but routine haematology, biochemistry and inflammatory markers were otherwise normal. The patient’s acne has responded partially to minocycline 100 mg daily. Depending on the patient’s continued response and acne severity, we will consider treatment with isotretinoin or substituting another antitumour necrosis factor (TNF)-a agent. However, we are currently reluctant to cease infliximab because of its excellent control of the bowel disease. We agree with Bassi et al. that the development of antiTNF-a-induced acne suggests a paradox similar to that of antiTNF-a-induced psoriasis. This paradox may help in our further understanding of the aetiology of acne and the action of TNF antagonists on the inflammatory cascade.

Drug rash with eosinophilia and systemic symptoms and graft-versus-host disease developing sequentially in a patient

We describe a case of drug rash with eosinophilia and systemic symptoms (DRESS) and graft‐versus‐host disease (GvHD) developing sequentially in a patient displaying reactivation of CMV. We discuss the possibility that similar pathogenic mechanisms may be involved in the development of DRESS and GvHD.