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.

Congenital erythropoietic porphyria: dilemmas in present day management.

Congenital erythropoietic porphyria is a rare autosomal recessive disorder of haem biosynthesis caused by a deficiency of uroporphyrinogen III synthetase. There is resultant accumulation and hyperexcretion of porphyrinogens of the isomer I variety. These are converted by spontaneous oxidation into their corresponding photoactive porphyrins leading to photodamage. Accumulation of porphyrins results in haemolysis and extensive photosensitivity. The consequences of chronic haemolysis are splenomegaly, reactive erythroid hyperplasia, erythrodontia, bone fragility, extreme photosensitivity and photomutilation. We present a 35-year-old man who has the severe infantile form and illustrates the haematological and photodestructive complications despite attempts at treatment with hypertransfusion, oral charcoal therapy and beta-carotene. Allogenic bone marrow transplantation has been considered but because of the high associated mortality this procedure has been discounted at present in the management of our patient.

Phenytoin-induced toxic epidermal necrolysis: a case report.

We present a case of phenytoin-induced toxic epidermal necrolysis resulting in 60-70% skin involvement. Systemic corticosteroids and prophylactic antibiotics used initially were discontinued, and subsequent management concentrated on intensive supportive treatment. The patient survived, but is left with disabling ocular complications.

Multiple endocrine neoplasia type 1 presenting as rosacea.

We present a case of malignant carcinoid initially diagnosed as rosacea. This patient was later found to have an additional functioning parathyroid tumour. Although a pituitary tumour was not identified, the association represents a probable case of multiple endocrine neoplasia type 1 (MEN 1). This autosomal dominant syndrome is characterized by tumours of the pancreas, parathyroid and pituitary. Inoperable carcinoid tumour is best treated with a long-acting somatostatin analogue, octreotide. A diagnosis of MEN 1 has important connotations for the proband's first-degree relatives, who should be entered into an appropriate screening programme.

Mitozantrone-induced onycholysis. A series of five cases.

The anthracenedione cytotoxic agent, mitozantrone, has a well-established place in the treatment of acute leukaemia, lymphoma, breast carcinoma and other malignant tumours. In general it is well tolerated apart from myelosuppression and dose-related cardiotoxicity. Alopecia is a common side-effect with mitozantrone but otherwise the skin and its appendages are rarely affected. We present five cases of mitozantrone-induced onycholysis.

Serum sickness-like illness following streptokinase therapy. A case report.

We report a patient who developed a serum sickness-like illness characterized by fever, arthritis, haematuria and a purpuric eruption 7 days following streptokinase therapy for myocardial infarction. Blood tests demonstrated increased circulating immune complexes. The skin involvement resulted in extensive, painful cutaneous infarction with secondary bacterial infection. Serum sickness is an uncommon but recognized complication of streptokinase therapy.

Vascular proliferation and angiogenic factors in psoriasis.

Psoriasis is a common, chronic skin disorder characterized by hyperproliferation of the epidermis, inflammatory cell accumulation and increased tortuosity and dilatation of dermal papillary blood vessels. Research into the pathogenesis of psoriasis has concentrated mainly on the interplay between inflammatory cells and epidermal proliferation. Central to the proposed pathogenetic pathway are cytokines produced by activated keratinocytes, which are thought to induce both keratinocyte proliferation and lymphocyte migration. Cytokines also mediate upregulation of adhesion molecules on vascular endothelium which in turn permits lymphocyte recruitment. The close spatial relationship between altered microvasculature and epidermis is clearly important in psoriasis. Consequently, understanding the mechanisms underlying vascular changes is fundamental to an elucidation of pathogenetic mechanisms in psoriasis.