Manifestaciones cutáneas en lactante con síndrome de hiper-IgE / Cutaneous manifestations in an infant with hyper-IgE síndrome

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Lesiones hiperqueratósicas y prurito en paciente inmunodeprimida / Hyperkeratotic lesions and pruritus in an immunosuppressed patient

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Systemic amyloidosis with an exceptional cutaneous presentation.

Alopecia and nail distrophy are rare signs of systemic amyloidosis. We present a case with both manifestations and give a brief review of the cutaneous signs of this disease. A biopsy of affected or unaffected skin may provide the diagnosis.

Variants of milia successfully treated with CO(2) laser vaporization.

BACKGROUND: Milia are a usual consultation in dermatologic practice and optimal treatment modalities are not established. OBJECTIVE: To evaluate the efficacy and the safety of CO(2) laser vaporization in the treatment of milia. METHODS: We report four patients, aged from 12 to 50 years old, with different variants of milia: milia en plaque, milia post-photodermatitis, multiple eruptive milia and milia post-trauma. They were treated with the CO(2) laser in superpulsed and focused mode at 2 W/cm(2), with two passes in each session. The follow-up was 12-36 months. RESULTS: All patients showed marked improvement after a few sessions of CO(2) laser. No-one had recurrence or noticeable side effects. CONCLUSION: The CO(2) laser is an option in adult and child patients with multiple milia lesions or milia en plaque, obtaining good cosmetic results with minimal side effects.

Papular elastorrhexis, a distinctive entity?

Papular elastorrhexis is a rare entity of elastic tissue characterized by multiple white papules usually located on the trunk. We report a case of papular elastorrhexis in a 22-year-old man with sparse lesions and discuss the main controversial facts in this entity: its consideration as an independent disease or as an incomplete form of Buschke-Ollendorff syndrome and the confusion with and its possible inclusion as a variant of elastic tissue naevi.

Bowen's disease treated by carbon dioxide laser. A series of 44 patients.

BACKGROUND: Bowen's disease is a common form of intraepidermal (in situ) squamous cell carcinoma of the skin and mucous membrane. Treatment with destruction of the epidermis by any method is necessary to prevent invasive squamous cell carcinoma. OBJECTIVE: To report our experience with CO2 laser vaporization in the treatment of Bowen's disease and try to identify which factors could have any influence on results. METHODS: A total of 44 patients were treated with the CO2 laser in superpulsed mode, focalized at 2 W/cm2. RESULTS: In 86.3% of patients, a unique treatment session was required. 'Clearance after one treatment' was achieved in 86.3% of the total series of patients and only 7.9% of these patients developed recurrence of the lesion. In 11.3% of the cases, there was 'clearance after more than one treatment' without clinical recurrences in any of these patients during the follow-up. 'No response' was achieved in 2.2% of the total series of patients. CONCLUSION: We conclude that the CO2 laser in superpulsed mode is an effective, efficient, safe, functional and good cosmetics treatment for Bowen's disease and it provides the same recurrence rate as other more aggressive or more expensive modalities of treatment.

A case of generalized eruptive histiocytosis.

Histiocytoses are a heterogeneous group of diseases, characterized by the accumulation of reactive or neoplastic histiocytes in various tissues. Generalized eruptive histiocytosis belongs to cutaneous non-Langerhans' cell histiocytoses and is a rare, generalized, self-healing disorder that usually follows a benign clinical course. Herein, we report a case of generalized eruptive histiocytosis in a 41-year-old woman with peculiar clinical and histological features. Clinically, the papules showed a marked distribution into the seborrhoeic areas of the trunk, with a great tendency to coalesce. Furthermore, immunohistochemical labelling demonstrated that the histiocytes were positive for CD68, but negative for CD34, S100, CD1a and XIIIa factor. This is the second report of generalized eruptive histiocytosis with a negative XIIIa factor. We discuss the differential diagnoses of the clinical picture and emphasize that this benign cutaneous disorder should be subjected to close follow-up, owing to the possibility of evolution to a more severe type of histiocytosis or the association with underlying diseases. Spontaneous regression was observed in this actual case.

Epidermal nevi treated by carbon dioxide laser vaporization: a series of 25 patients.

BACKGROUND: Epidermal nevi are benign proliferations of epidermis. Numerous treatment modalities have been tried, but no ideal treatment is yet available. OBJECTIVE: To report our experience with CO(2) laser vaporization in the treatment of verrucous epidermal nevi, and to identify which factors could have influence in long-term results. METHODS: A total of 25 patients were treated with the CO(2) laser in the superpulsed mode, focalized at 2 W/cm(2). The patients were between 3 and 41 years old (mean: 17 years). A total of 44% of lesions were <20 cm(2), 40% measured between 20 and 100 cm(2) and 16% were >100 cm(2). The follow-up was 4-79 months (mean: 28 months). In 76% of patients, multiple treatment sessions were required. RESULTS: Good results were achieved in 92% of patients with soft, flattened nevi and in only 33% patients with keratotic nevi. In addition, 87% of the latter had moderate results and 12.5% had poor results. CONCLUSION: We conclude that the CO(2) laser in superpulsed mode is an effective and safe treatment for verrucous epidermal nevi and provides fewer recurrences than other laser therapies. We also believe that the most determining factor for the cosmetic result is thickness of the nevus.

Treatment of cutaneous and mucosal telangiectases in hereditary hemorrhagic telangiectasia: Report of three cases.

INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is an autosomal dominant disorder characterized by epistaxis, mucocutaneous telangiectases and visceral arteriovenous malformations. Cutaneous telangiectases are the most obvious sign of this disorder and are not a merely cosmetic problem owing to their risk for bleeding, which may constitute an authentic matter of concern to these patients. METHODS: Three patients with type-II HHT disease who presented cutaneous and labial lesions were treated with an intense pulsed light (IPL) system associated with a long-pulsed Nd:YAG laser (Photoderm-Vasculight, Lumenis). Labial lesions were treated with the IPL system and cutaneous lesions were treated with the IPL system and the long-pulsed Nd:YAG laser. All treatments were accomplished on an outpatient basis, without anesthesia. RESULTS: All three patients experienced a reduction in the frequency and severity of bleeding episodes and a great cosmetic improvement, with total lesion clearance. Side effects, such as purpura, hypopigmentation, hyperpigmentation or textural changes, were not observed. Recurrences were not detected after a maximum period of 24 months of follow-up. CONCLUSION: The combination of an IPL system and a long-pulsed Nd:YAG laser is efficient and safe for the treatment of cutaneous and labial telangiectases in patients with HHT disease and constitutes an important tool in improving their quality of life.

Neutrophilic dermatosis of the hands: presentation of eight cases and review of the literature.

Pustular dermatosis of the dorsal hands is a peculiar variant of Sweet's syndrome described by Strutton et al. Nevertheless, not all authors accept this entity. We present a retrospective study of eight cases of neutrophilic vasculitis of the hands. Three patients presented with lesions only on the dorsal hands, three with lesions on the palmar surface and two with lesions on the dorsal and palmar surfaces of the hands. The patients with lesions on the dorsal surface presented a pustular aspect, and the histopathological examination revealed a high degree of vascular damage with presence of vasculitis and, in one case, with fibrinoid necrosis. Therefore, the term pustular vasculitis of the dorsal hands introduced by Strutton et al. should probably be conserved. Our cases may have demonstrated a peculiar clinicopathological disease with its own entity.