Stage IV cutaneous acute graft-versus-host disease. Clinical and histological study of 15 cases.

BACKGROUND: The most severe form of cutaneous acute graft-versus-host disease (aGVHD), stage IV, is characterized by the appearance of vesicles and blisters. OBJECTIVE: To describe the clinicopathological characteristics and evolution of stage IV cutaneous aGVHD presented in our hospital. METHOD: Retrospective study. The following criteria for inclusion were applied: (i) patients subjected to allogeneic stem cell transplantation between 1st January 1984 and 31st of December 2006; (ii) development of vesicles and/or blisters; (iii) extracutaneous coincidental aGVHD manifestations; and (iv) presence of histopathological features consistent with aGVHD. RESULTS: Fifteen cases (10 females and 5 males) were studied. The mean age was 38.1 years. The lesions appeared after a median interval of 19 days, always following a milder stage of GVHD. Two patterns of clinical evolution were found. Mucosal involvement was observed in nine patients. Nikolsky's sign was positive in eight patients. Nine of the patients had biopsies of the vesiculobullous stage which showed a subepidermal blister with epidermal necrosis and basal vacuolar degeneration. Only two patients survived. CONCLUSION: Stage IV cutaneous aGVHD is a severe and unusual complication after haematopoietic stem cell transplantation. Prognosis is poor with a very high mortality rate, although the cause of death is varied and not strictly linked to the cutaneous disease.

Cutaneous lichenoid graft-versus-host disease mimicking lupus erythematosus.

Graft-versus-host disease (GVHD) is the main complication of allogeneic bone marrow transplantation and the skin is the most commonly involved organ. The clinical presentation is varied and may resemble autoimmune diseases, such as scleroderma, lichen planus or lichen sclerosus. Chronic GVHD presenting with a butterfly malar rash mimicking lupus erythematosus is uncommon. We report a series of five patients with cutaneous lichenoid GVHD that presented with a butterfly malar rash. Two of our patients had positive antinuclear antibody titres. The evolution was poor with development of sclerodermatous GVHD lesions in three patients and relapse of their haematological disease in two.

Flare and change of psoriasis morphology during the course of treatment with tumour necrosis factor blockers.

Tumour necrosis factor (TNF) blockers represent an exciting advance in the management of psoriasis. However, the safety profile of these drugs is not completely established. We present a review of the literature, and report on eight patients: two with the unexpected appearance of psoriasis, and the remaining six with exacerbation and change in morphology of their existing psoriasis, all of which occurred during treatment with the TNF blockers adalimumab, etanercept and infliximab. The two new cases, neither of whom had any personal or family history of psoriasis, developed pustular psoriasis on the palms and/or soles. The other six patients, previously diagnosed with severe chronic plaque psoriasis (four patients), generalized pustular psoriasis (one) and erythrodermic psoriasis (one), developed eruptive guttate psoriasis between 15 days and 18 months after the beginning of therapy. These patients had never before presented guttate-type psoriatic lesions, and the lesions appeared in areas of the body that were free of psoriatic plaques at baseline.