Two cases of cutaneous chronic graft versus host disease in treatment with psoralen plus ultraviolet-A-bath photochemotherapy.

BACKGROUND: Up to 50% of patients undergoing allogenic stem cell transplantation or bone marrow transplantation (BMT) can develop acute or chronic graft versus host disease (GVHD) as a severe complication. Immunosuppressive therapies may prove not only ineffective but may cause serious adverse effects. GVHD remains a major clinical problem and is often associated with high mortality rates. METHODS: This article presents the cases of a 48-year-old woman and a 23-year-old man, both presenting with severe cutaneous sclerodermic chronic GVHD following allogenic stem cell transplantation. Despite several years of treatment with oral corticosteroids, mycophenolate mofetil, sirolimus and acitretine, the skin lesions had not improved. Both patients were then treated with psoralen plus ultraviolet (PUVA)-bath photochemotherapy three times weekly, following a standardized treatment protocol. RESULTS: After a total accumulated dose of about 90 J/cm2, skin lesions in both patients had improved, showing complete remission in some body areas. Systemic corticosteroid treatment could be gradually reduced in the case of the female patient and skin conditions remained stable during maintenance treatment of PUVA-bath two times weekly and during a mean follow-up period of eight months. CONCLUSION: Oral PUVA therapy has been established as a successful treatment for acute and chronic GVHD, but unfortunately may result in systemic side effects. Psoralen plus ultraviolet-A-bath photochemotherapy provides clinicians with a therapeutic alternative that offers high clinical efficacy and safety. Therefore, PUVA-bath could be included as an alternative in the treatment protocol for chronic cutaneous GVHD.

Capillary malformation-arteriovenous malformation: a clinical review of 45 patients.

BACKGROUND: Capillary malformation-arteriovenous malformation (CM-AVM) is a recently described autosomal dominant disorder that results from mutations in RASA1. It has been initially described as multiple CMs affecting several members of the same family, associated with fast-flow malformations in at least one family member. OBJECTIVE: To report and analyze clinical data on 45 patients with CM-AVM assessed at the Department of Pediatric Dermatology, Ramos Mejía Hospital (Buenos Aires, Argentina). METHOD: Retrospective clinical review of all the patients clinically diagnosed as having CM-AVM over a period of eight years. RESULTS: Forty-five patients were recorded (24 females and 21 males). The age ranged from one month to 44 years. In 36 patients, the stains were congenital; progressive acquired lesions were observed in 39. Family history was positive in 32 subjects. Well defined, round to oval, pink-purple or reddish-brown macules were found in all the patients; pinpoint red lesions with a pale halo were found in nine cases. The macules were warmer than normal skin in 15 cases and surrounded by a white halo in 26 cases. Three subjects presented associated overgrowth, lymphatic malformation was present in one case, retinal vascular lesion in one patient, and isolated port wine stain in two cases. Three patients also had infantile hemangioma. We had no cases of fast-flow vascular malformation or combined vascular syndromes. CONCLUSIONS: CM-AVM is a heterogeneous disorder with phenotypic variability, from fast-flow malformation, limb enlargement, or Parkes Weber syndrome to multiple CMs without internal involvement.