Pustular generalized perforating granuloma annulare.

We report a 84-year-old man with a 13-year history of recurrent generalized asymptomatic pustular lesions. Histology revealed areas of necrobiosis surrounded by palisading granulomas and transepidermal and follicular elimination of the necrobiotic material. A dense infiltrate of neutrophils was also found. Although 26% of patients with generalized perforating granuloma annulare have some yellow pustule-like papules, these correspond histologically to the yellow viscous necrobiotic material extruding through the epidermis and not to a real neutrophilic infiltrate. This is the first case report of perforating granuloma annulare with recurrent generalized pustular lesions with a dense infiltrate of neutrophils.

[Acromelanosis]. / Acromelanosis.

Acromelanosis is an independent disease entity, characterized by increased skin pigmentation, usually located on the acral areas of the fingers and toes. It is mostly seen in newborns or during the first years of life. Only a few cases of this entity have previously been described in the medical literature. In some of these cases, the cutaneous lesions spread to affect large parts of the skin surface. A possible association with other benign and malignant diseases has been proposed. Differential diagnosis must be made with a wide variety of systemic and dermatologic conditions, especially dermatoses with acral distribution of macular hyperpigmentation, including acropigmentation. In this article, we report a new case of acromelanosis in a 5-week-old girl showing two peculiar clinical features: associated melanosis of the genital mucosa and stabilization of the lesions after an initial phase of progression and proximal spread. In addition, the most important features of this rare cutaneous disease are discussed.

[Hypohidrotic ectodermal dysplasia: A cause of fever of unknown origin]. / Displasia ectodérmica hipohidrótica: una causa de fiebre de origen desconocido.

The term ectodermal dysplasias includes many disorders that share some clinical features such as involvement of one or several ectodermal structures and congenital origin. Currently, 154 different types divided into 11 clinical subgroups (Freire Maia classification 1994) have been described. The most frequent entity is hypo- or anhidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome). This is a rare hereditary disease whose main characteristic is the absence, or more often the reduction, of sweat glands, leading to an increase in body temperature together with anomalies of the epidermis and its appendages (hair and nails). We present a case of hypohidrotic ectodermal dysplasia in a premature 18-month-old boy who was referred to our department because of markedly dry skin since birth and recurrent eczematous and lichenification lesions that had been successfully treated with topical corticosteroids. Physical examination revealed mild alopecia with sparse and fine blonde hair and the absence of dental alveoli. The boy's mother had noticed slight sweating and episodes of fever without clinical symptoms, which were more frequent in summer. Hypohidrotic ectodermal dysplasia should be included in the differential diagnosis of fever of unknown origin.

Photosensitivity induced by oral itraconazole.

A case of photosensitivity induced by itraconazole is reported. A 70-year-old woman had erythema, oedema and vesicles on sun-exposed areas after 5 days of itraconazole treatment for oral candidiasis. Oral photochallenge using itraconazole and sun irradiation was positive, but photopatch test was negative. Photosensitivity from azoles is an uncommon adverse effect. Only three other cases have been described, two induced by ketoconazole and one by itraconazole.

Lichen myxoedematosus associated with human immunodeficiency virus infection--report of two cases and review of the literature.

Lichen myxoedematosus (LM), or papular mucinosis, is an uncommon papular eruption caused by the dermal deposition of mucin. Three clinical forms can be distinguished, namely localised, disseminated (involving more than one site), and generalised LM, the last is called scleromyxoedema, and demonstrates erythema and skin sclerosis. Paraproteinaemia, often consisting of an abnormal IgG with lambda light chains, is usually present in patients with LM. Visceral involvement has also been documented. An association between LM and human immunodeficiency virus (HIV) infection has been reported recently. We now describe two further HIV-positive patients with LM and present a review of the literature regarding this association.

[Perniosis and lupus anticoagulant]. / Perniosis y anticoagulante lúpico.

Five patients with acral chilblains and lupus anticoagulant activity revealed by coagulation analysis are reported. Three patients suffered a systemic lupus erythematosus, and two of them a completely developed antiphospholipid syndrome as well. Another case had chronic cutaneous lupus with only facial discoid lesions, and in the last one the lupus anticoagulant was likely related to a chronic liver infection of hepatitis B virus because she had not erythematous lupus. There are few dermatologic references about chilblain associated to lupus anticoagulant. Certain evidences suggest a pathogenic relation of this findings.

[Lichen planus and hepatitis C virus infection. Study of 6 cases]. / Liquen plano e infección por el virus de la hepatitis C. Estudio de seis casos.

Lichen planus is an inflammatory dermatosis that has been associated to different chronic hepatopaties with a variable frequency. In recent years several case reports have been published dealing with lichen planus and its association to hepatitis C virus infection. We report six patients with lichen planus (four males, two females; aged 30-75) who also suffered a chronic hepatitis C virus infection. Lichen planus was confirmed by biopsy and viral infection was based on ELISA and RIBA-4 tests in all patients and also on PCR in five of them. A patient had also been diagnosed of porphyria cutanea tarda. Two patients were treated with alfa-Interferon. It did not modify the evolution of their lichen planus. We recommend to search for hepatitis C virus infection in lichen planus patients and also look for lichen planus patients suffering of chronic hepatopathies to improve our knowledge of the possible relation between both diseases.

Cryoglobulinemic vasculitis associated with hepatitis C virus infection. A report of eight cases.

Cryoglobulinemia has an unknown etiology in many cases. In the last years, hepatitis C virus has been known to be involved as a causative agent of so called essential mixed cryoglobulinemia. We report 8 patients suffering from cutaneous vasculitis and chronic hepatitis C virus infection. Their sera showed cryoglobulinemia (6 cases mixed, 2 cases not typified), rheumatoid factor and complement consume. Their hepatitis C virus infection was confirmed by PCR. Hepatitis C virus RNA was found by PCR in 6/6 cryoprecipitates and 5/6 supernatants. The patients were followed for 0.5-12 years. Three of them developed renal affection (2 membranoproliferative glomerulonephritis, 1 not biopsied). Two patients were treated with alpha interferon. One patient noted a marked improvement in her cutaneous vasculitis with interferon treatment, lasting for 18 months after the end of treatment. We recommend the search of hepatitis C virus infection in every case of mixed cryoglobulinemia. Interferon therapy may be useful in some of these cases.

Cutaneous plasmacytosis: report of a case in a white man.

We describe a 40-year-old white man with a peculiar skin eruption in association with polyclonal hypergammaglobulinemia. No underlying disease was detected. A skin biopsy specimen showed a proliferation of mature plasma cells intermingled with some lymphocytes and histiocytes, an appearance consistent with cutaneous plasmacytosis. This disease had been previously described only in Japanese patients. In our patient the disease progressed slowly. Lymph node infiltration by mature plasma cells was later noted.

Primary cutaneous extramedullary plasmacytoma secreting lambda IgG.

An adult male was described, who developed a tumour on his left arm, which was diagnosed as a primary cutaneous extramedullary plasmacytoma after histopathological, electron microscopic and immunocytochemical studies. Further studies ruled out the presence of multiple myeloma, extramedullary plasmacytoma of another site, or paraproteinaemia/paraproteinuria. During a relapse of the tumour 2 1/2 years after initial treatment, a new immunocytochemical study demonstrated that the tumour cells expressed monoclonal lambda IgG at the intracytoplasmic level, an unusual finding.