Lorenzo Marchesi

Randomized controlled trial comparing the effectiveness of 308‐nm excimer laser alone or in combination with topical hydrocortisone 17‐butyrate cream in the treatment of vitiligo of the face and neck

Background  Vitiligo is a pigmentary disorder which may have disfiguring consequences. Its treatment remains a challenge.

Cutaneous manifestations associated with antiphospholipid antibodies in patients with suspected primary antiphospholipid syndrome: a case-control study.

OBJECTIVE: To study the association of a variety of dermatological manifestations related to vascular abnormalities with antiphospholipid antibodies in patients with suspected primary antiphospholipid syndrome. METHOD: Case-control study. Consecutive patients referred to the coagulation and haemostasis service of a general hospital for the first determination of antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibodies) and newly diagnosed disorders (for example, thrombocytopenia, thrombotic disorders, and unexplained repeated abortions) were selected. Patients were examined by two dermatologists according to predefined criteria, and information about general characteristics and relevant dermatological and medical histories were collected using an ad hoc questionnaire. The study was limited to patients without evidence of systemic lupus erythematosus. A total of 35 patients was examined; 13 subjects were positive for lupus anticoagulant or anticardiolipin antibodies, or both (cases), and 22 were negative (controls). RESULTS: Moderate to severe livedo reticularis and acrocyanosis were significantly associated with antiphospholipid antibodies, with relative risks of 13.1 (95% confidence interval 1.1 to 149.0) and 8.6 (95% confidence interval 1.1 to 65.1). Capillaritis was also associated with the antibodies. Histories of Raynauds phenomenon and superficial thrombophlebitis were more common in cases than controls. CONCLUSIONS: This study provides quantitative evidence of the association of antiphospholipid antibodies with several cutaneous diseases in which vascular abnormalities seem to play a major part. The study suggests that these manifestations might appear early in the development of the antiphospholipid syndrome.

A Case of Hypopigmented Mycosis Fungoides in a Young Caucasian Boy

Abstract: Hypopigmented mycosis fungoides is a variant of mycosis fungoides characterized by the presence of hypopigmented patches as the sole manifestation of the disease. It has been described aimost always in young black or dark‐skinned patients. The only white patient described was a 64‐year‐oid woman who not oniy had hypopigmented lesions, but also nodular lesions with lymphadenopathy. We describe hypopigmented lesions arising in a white boy 12 years of age, born in northern Italy, without any foreign ancestors. The microscopic alterations, with epidermotropism, the immunoiogic markers, the negativity of T‐cell receptor gene rearrangement, and the good response to PUVA therapy correspond to the main findings in black patients with this disease. Long‐term follow‐up of these patients is important to obtain better knowledge of the natural history of the disorder, Hypopigmented mycosis fungoides must now be included in the differential diagnosis of hypopigmented macular lesions not only in black or dark‐skinned patients but also in white patients.

Agminate blue nevus combined with lentigo : a variant of Speckled lentiginous nevus ?

We report a case of agminate blue nevus combined with a lentigo patch in a 38-year-old woman. The combination was interpreted as a unique and peculiar variant of speckled lentiginous nevus.

Allergic reaction with persistent hypopigmentation due to temporary tattoing with henna in a baby

Recently, temporary paint-on tattoos have become increasingly popular as a safe alternative to permanent tattoos not only in Asia and Northern Africa but also in our countries. The most common dye for such temporary tattoos is henna, a vegetable dye obtained from the dried leaves of a shrub (Lawsonia inermis), used for thousands of years to colour the hair and the skin for the ritualistic staining. Allergic reactions to this compound have frequently been reported in adults, rarely in childhood. The most likely causative agent for the eczematous reactions is p-phenylendiamine (PPD) present in the commercial black henna, added to obtain a dark, blackish colour. We describe the case of a little baby who had an allergic contact dermatitis due to temporary paint-on tattoos with a persistent hypopigmentation after the resolution of the eczematous lesions.

Cutaneous Malignant Melanoma Appearing during Photochemotherapy of Mycosis fungoides

We report a case of malignant melanoma that appeared in a 56-year-old man with mycosis fungoides (stage Ia) during treatment with PUVA. The cumulative UVA dose was 1,177 J/cm2. The pigmented lesion was removed and PUVA therapy discontinued. Histological examination revealed a superficial spreading malignant melanoma (1.77 mm thick, Clark level IV). The delayed-type cutaneous hypersensitivity was studied. The presence of a second malignancy after mycosis fungoides and PUVA therapy may have been coincidental. Nevertheless, this case suggests that the immunosuppression induced by mycosis fungoides and by PUVA therapy might be a pathogenetic factor in the development of malignant melanoma.

Antiphospholipid Antibodies and Necrotizing Purpura

A 30-year-old woman with recurrent necrotizing purpura on the legs which did not fit, to our knowledge, within any distinctive pathological entity, is described. Laboratory investigations disclosed a mild thrombocytopenia and antiphospholipid antibodies, i.e. lupus anticoagulant and anticardiolipin. Cutaneous lesions may be an early marker of the antiphospholipid antibody syndrome.

Combined Clark's nevus.

We report five cases of combined Clarks nevi. Like all melanocytic nevi, Clarks nevus can combine with other nevi to produce lesions that are hard to interpret clinically and histologically. The morphology of combined Clarks nevus can resemble that of a melanoma associated with a nevus.

Unusual manifestations of primary cutaneous amyloidosis in association with Raynaud's phenomenon and livedo reticularis

A patient with unusual manifestations of primary cutaneous amyloidosis, including macules, papules, oedematous plaques and urticarial lesions is described. Raynauds phenomenon and livedo reticularis were an associated finding. During the acute phase, single doses of systemic corticosteroids resulted in an impressive, long‐lasting improvement in cutaneous manifestations.

Warty linear streaks of the palm and sole: possible late manifestations of incontinentia pigmenti.

Sir, Molluscum contagiosum (MC) is a poxvirus skin infection that often complicates the course of patients with acquired or iatrogenic immunosuppression. Both the clinical and histological features of disease in these cases may be atypical. We report the unique clinical and histological features of a case of fulminant MC infection with concomitant leukaemia cutis in a patient with relapse of chronic myeloid leukaemia (CML) after allogeneic bone marrow transplantation (BMT). A 49-year-old Chinese woman underwent BMT (conditioning: busulphan, cyclophosphamide, total body irradiation) from an HLA identical sister for Ph positive CML in accelerated phase. She engrafted uneventfully with no chronic graft-vs.-host disease (GVHD). Serial bone marrow reassessments, up to 18 months post-BMT, were negative for residual disease by polymerase chain reaction and cytogenetics. At 36 months, she was found to have haematological relapse of CML, with cytogenetic subclonal evolution. She was treated with hydroxyurea and donor lymphocyte infusions (DLI) (6 ́1 10 kg cells infused in three doses) to enhance the graft-vs.-leukaemia (GVL) effect. There was good control of the peripheral cell counts and no evidence of GVHD. A repeat marrow biopsy at 40 months showed suppression of the abnormal clone to 3% of analysed metaphases. Unfortunately, at 46 months the disease progressed again with increased neutrophil counts (52 10 L), and leukaemia cutis documented by skin biopsy (Figs 1a, 1b). This was treated with combination chemotherapy (cytosine arabinoside 150 mg 5, thioguanine 160 mg 5) resulting in normalization of cell count and resolution of all skin lesions. A second course of DLI (4 ́2 10 kg cells) was administered at 49 months post-BMT. Three weeks after DLI, however, the patient presented with a dense eruption of erythematous papular lesions over the entire face, upper limbs and upper trunk. Photography of the lesions was refused. A biopsy of one lesion showed lobules of abnormal epidermal cells with cytoplasm packed with eosinophilic viral inclusion bodies (Fig. 2a). Electron microscopy showed numerous intracytoplasmic poxvirus particles (240 95 nm in size) within the abnormal epidermal cells, consistent with MC (Fig. 2b). In addition, an infiltrate of promyelocytes and immature myeloid blast cells was seen, consistent with recurrent leukaemia cutis. She died 1 week later of an intracranial haemorrhage, probably related to intracerebral disease. The use of DLI is the treatment of choice for relapse of CML after BMT. The main side-effects are profound marrow and immune suppression, with or without acute and chronic GVHD. Reactivation of dormant DNA viruses like cytomegalovirus, varicella zoster and hepatitis B viruses are potential complications of immunosuppression caused by DLI. This is the first report of severe MC complicating DLI. In immunosuppressed hosts, due to the fulminant replication of the poxvirus, the clinical and pathological features of MC can be highly variable, and aggressive treatment is often needed. Viral particles have even been found in the histologically normal skin epidermis adjacent to MC lesions in patients infected with HIV. There have been two previous reports of skin changes in MC mimicking haematological malignancy involving the skin. In our case, the clinical setting and pathological features are highly supportive of a genuine double pathology. It is recognized that post-BMT patients have an increased incidence of leukaemic involvement of extramedullary sites, including the skin. The incidence may be even higher in patients salvaged with DLI, due to a weaker GVL effect outside the marrow. The precise colocalization of MC replication and leukaemic infiltration in the skin lesions may have been due to a high concentration of chemotactic