[Atypical histiocytoma in a child]. / Histiocytofibrome atypique chez une enfant.

BACKGROUND: Histiocytoma (HC) is a very common benign tumour generally seen in the lower limbs of adults, particularly women. There are, however, atypical forms of HC that behave like locally aggressive tumours, occasionally with relapse or even metastasis. Herein we report a case of locally aggressive HC in a child, which, on account of its clinical extension, required seven surgical procedures to achieve complete excision. PATIENTS AND METHODS: A 13-year-old child consulted for a hard purplish papule measuring 8 mm in diameter located in the right lumbar region. Punch biopsy revealed a poorly delineated dermal-hypodermic tumour comprising randomly distributed moderately pleomorphic fusiform cells, arranged in bands or with storiform architecture, certain of which were multi-nucleated. The mitotic index was high (11 mitoses in 10 fields at high magnification). There was no expression by the tumour of melanocytic markers (PS100, Melan-A), histiocytic markers (CD68) or CD34. FISH analysis showed the absence of COL1A1-PDGFB fusion gene. Based on these immunohistochemical and molecular findings, a diagnosis was made of atypical HC with high cellular density. Since the lower margins of the section showed tumoural foci, surgical excision was performed with 5-mm margins. Because the lateral and vertical limits were reached in all cases, a series of five further procedures (the last of was preceded by multiple peripheral biopsies) was necessary to achieve complete excision. These multiple excision procedures resulted in total excision of 25 cm across the longest side. No clinical relapse was seen after 25 months. DISCUSSION: Cellular or atypical forms of HC carry a high likelihood of post-surgical relapse. They are characterised by marked pleomorphism and high cellular density. In our patient, the extent of the lesion had been greatly underestimated initially, resulting in the need for several surgical procedures in order to achieve complete excision. It is thus important to highlight the predictive factors for this type of tumour in order to enable sufficiently extensive excision, or excision guided by previous biopsies, to be contemplated from the outset. These predictive factors are: young patient age, unusual location (trunk, face, neck), high cellularity, marked mitotic activity and deep extension.

Effect of a preceding laser dermabrasion on the outcome of combined therapy with narrowband ultraviolet B and potent topical steroids for treating nonsegmental vitiligo in resistant localizations.

BACKGROUND: The treatment of vitiligo remains unsatisfactory. OBJECTIVES: To assess in patients with vitiligo the effect of a laser dermabrasion in addition to the association of topical steroids and ultraviolet (UV) B in difficult-to-treat areas. METHODS: This was a single-centre prospective randomized trial including patients with nonsegmental vitiligo who had at least two symmetrical lesions located on bony prominences and/or extremities. An erbium laser-assisted dermabrasion was first performed on one side (randomly assigned). After 48 h, hydrocortisone 17-butyrate cream applied daily for three periods of 3weeks followed by a 1-week steroid-free interval and narrowband UVB treatment was performed on both sides twice weekly for 12weeks. The evaluation was performed on standardized pictures by two physicians blinded to the type of treatment received. The criterion of success was a repigmentation of at least 50%, 1month after the end of the treatment. RESULTS: Eighteen patients were included (24 paired lesions treated). Two patients dropped out for personal reasons. Almost 50% of lesions achieved at least 50% repigmentation in the dermabrasion side while only 4·2% did so with topical steroids and UVB alone (P<10(-4) ). Side-effects were delayed healing, pain and two hypertrophic scars. The tolerance and patient satisfaction were 4·2 and 4/10 for the laser-treated side and 8·4 and 3/10 in the UVB+ steroids alone group, respectively. CONCLUSIONS: Laser dermabrasion significantly improves the repigmentation rate in vitiligo lesions. Despite a high rate of repigmentation in such difficult-to-treat areas the high rate of side-effects and the poor tolerance strongly limit its use in current practice.

308-nm excimer lamp vs. 308-nm excimer laser for treating vitiligo: a randomized study.

BACKGROUND: The 308-nm excimer laser and 308-nm excimer lamp have both been shown to be effective in treating vitiligo but a direct comparison has never been performed. OBJECTIVES: To test the equivalence of these two devices for treating nonsegmental vitiligo. PATIENTS AND METHODS: A randomized monocentric study was undertaken. One lesion was treated with the 308-nm excimer laser and its counterpart with the 308-nm excimer lamp. Lesions were treated twice weekly with the same dose on both sides for a total of 24 sessions. The evaluation was done by two independent physicians blinded to the treatment on direct light and ultraviolet light photos. RESULTS: Twenty patients were included: 17 completed the study and 104 lesions were treated. The two treatments showed similar results in terms of efficacy for a repigmentation of at least 50% (P = 0.006). The lamp induced more erythema than the laser. CONCLUSIONS: The 308-nm excimer lamp and laser showed a similar efficacy in treating vitiligo. For the same fluence, the lamp induced more erythema suggesting photobiological differences between the two devices.

[Efficacy of intravenous immunoglobulin in the treatment of scleromyxoedema]. / Efficacité des immunoglobulines intraveineuses dans le traitement d'une mucinose papuleuse généralisée.

BACKGROUND: Scleromyxoedema is a rare disorder of unknown pathogenesis that is very difficult to treat. We report a case resistant to corticosteroid treatment but controlled by intravenous gammaglobulin (IVIG). CASE REPORT: A 60-year-old woman presented progressive generalized papular eruption with infiltrated and itchy lesions of between 2 and 5mm in diameter. Otherwise, the clinical examination was normal. Monoclonal gammopathy of the IgG lambda type was found. Histology confirmed the diagnosis of scleromyxoedema. The disease continued to progress despite oral corticosteroids (0.5mg/kg per day). Thalidomide was introduced but was discontinued after 2 months due to side effects. Treatment comprising six monthly infusions of IVIG (2g/kg on 5 days) resulted in a marked reduction (>50%) in lesions. Two months after discontinuation of IVIG, recurrence was observed and maintenance infusions of IVIG every 6 weeks were needed to control the disease. DISCUSSION: The course of scleromyxoedema is unpredictable and treatment is extremely difficult. Successful therapy with IGIV has been reported but this approach seems to afford only temporary relief and maintenance infusions are required, as confirmed by the initial efficacy of treatment in our patient and the rapid recurrence of lesions following withdrawal.