José Bañuls

Acute juvenile pityriasis rubra pilaris: a superantigen mediated disease?

Juvenile acute pityriasis rubra pilaris (JAPRP) is a form of pityriasis rubra pilaris (PRP) with particular clinical characteristics and course. It is usually preceded by an infectious condition. We report a boy, initially thought to have Kawasaki disease, and subsequently diagnosed as having JAPRP, who made a satisfactory recovery. In this case, prior antibiotic treatment made it impossible to show the presence of any microorganism. However, the clinical characteristics of these patients and the similarities with other disorders involving the skin that are mediated by superantigens led us to think that these antigens may be involved in the development of this disease. Also, in view of the obvious differences between JAPRP and the other PRP, we suggest that, in the future this disorder may be considered a separate entity as a reactive exanthem.

Tissue and tumor mosaicism of the myotonin protein kinase gene trinucleotide repeat in a patient with multiple basal cell carcinomas associated with myotonic dystrophy

We describe the third case (to our knowledge) of multiple basal cell carcinoma associated with myotonic dystrophy and carry out a genetic study of the tumor comparing it with healthy skin. We consider that our results show that this association might be not a purely random phenomenon and that the particular genetic characteristics of this disorder might have a role in the pathogenesis of the tumor.

MORPHEA‐LIKE REACTION FROM VITAMIN K1

A 70-year-old woman with ehronic liver disease was treated for hypoprothrombinemia with intramuscular injections of 10 mg oleous vitamin Ki (phytomenadione) administered on alternate days for several weeks. Approximately 10 days after the first dose, she developed a pruritic, erythematous eruption at the injection sites that became later asymptomatic with two infiltrating plaques persisting on the buttocks. Eight months after the initial reaction, she was examined in our outpatient clinic for evaluation of the eruption. Physical examination showed two indurated, bright, atrophic, ivory-violaceous areas, 4 x 2 cm and 3 x 2 cm maximum diameter, with no erythematous ring, on the upper and outer gluteal quadrants (Fig. 1). The results of the rest of the physical examination were unremarkable and clinical signs of systemic sclerosis were not found. Histopathologic examination of a skin biopsy showed a normal epidermis, sclerosis of the reticular dermis, and a lymphocytic perivascular and periadnexial infiltrate (Fig. 2). The diagnosis of a cutaneous morphea-like reaction to vitamin Ki was made and no treatment was administered because spontaneous cure can be expected in most cases.

NEVOID HYPERKERATOSIS OF THE AREOLA

A 15-year-old woman presented with a painless change of color and texture of her right breast areola. The lesion had started some months previously. An examination of her right breast showed a hyperpigmentation of the areola, which appeared thickened and verrucous (Fig, 1), Her right nipple and left breast showed no alteration. She gave no personal or family history of ichthyosis, epidermal nevi, ot

Progression of Meyerson's naevus to Sutton's naevus.

Melanocytic naevi can suffer two types of perilesional inflammation, a depigmentation and a dermatitis [1, 2]. The former seems to be a form of cell-mediated, auto-immune reaction directed towards the destruction of the naevus and related to vitiligo. The dermatitis reaction is thought to be immune mediated [3, 4]. Meyerson’s phenomenon has not only been described with melanocytic naevi but also with other entities such as dermatofibroma. Although both processes involve the immune system, the mechanisms are different [5, 6]. Some authors consider these two as the opposite ends of the same immunological processes [3, 4]. On the other hand, other authors support the idea that they are different processes [5, 6] because the clinical, histological and evolutional data of both entities are very different [5]. The clinical findings which suggest that they are separate processes are that the eczematous halo does not show depigmentation and is pruriginous [1, 6]. The histology favours the latter hypothesis: the eczematous halo does not show the regression changes which are characteristic of the halo naevus [5]. Finally, the evolution of these two entities is often quite different, since in previously reported cases the eczematous halo did not evolve towards depigmentation and regression of the naevus [3–5]. A 28-year-old man developed itchy areas around the melanocytic naevi on both legs, of 1 month’s duration. Three months previously, he had experienced a similar episode that resolved spontaneously. Fig. 1. One of the naevi on the leg showing an eczematous halo. Fig. 2. Biopsy of one of the Meyerson’s naevi. The dermo-epidermal melanocytic naevus shows signs of regression in the papillary dermis. There is a lymphohistiocytic infiltrate, with numerous eosinophils in the upper dermis. Haematoxylin-eosin. Original magnification !200.

NEUTROPHILIC ECCRINE HIDRADENITIS IN AN HIV‐INFECTED PATIENT

A 30-year-old man who is an intravenous drug addict (not known to be Hiv-positive) was admitted to hospital complaining of fever and general malaise. The last dose of heroin had been administered 4 days previously and he had been taking dextropropoxyphenone since then. On admission, the leukocyte count was 9,200 per mL (neutrophils 8,100, lymphocytes 250), hemoglobin 12.4 g per dL, and the platelet count was 183,000 per mL. After a radiologic diagnosis of pneumonia was made, cloxacillin (1 g every 8 h iv) and gentamicin (80 mg every 8 h iv) were started, together with fluconazole (100 mg per day orally) for oropharyngeal candidiasis. Methadone, lorazepam, and flunitrazepam were given to prevent an opiate withdrawal syndrome. Seven days after admission, 12 erythematous maculae, of 5 mm diameter, were seen on the anterior thorax, together with pancytopenia (leukocyte count 700 per mL, neutrophils 600 per mL, lymphocytes 0 per mL, hemoglobin 10.9 g per dL, platelets 16,000 per mL) and persistent fever. Two days later, the patient died of acute respiratory insufficiency. Pneumocystis carinii and acid/alcohol-resistant bacilli were later confirmed by stain and culture, in the sputum, as was the positive HIV serology. A skin biopsy specimen showed a neutrophilic inflammatory infiltrate, together with necrosis of the epithelial cells of the eccrine coils (Fig. 1). The epidermis and the eccrine ducts in the dermis were intact. Neither mucin deposits nor squamous metaplasia of the eccrine ductal epithelium were seen. Special staining for bacteria, fungi, and acid-fast bacilli was negative.

Leiomyosarcoma of the upper lip

A 90-year-old woman was seen for a tumor on the upper lip that had been present for 2 years. The lesion measured 6x4 cm and affected the entire upper lip. The tumor was dark red in color, and the surface was ulcerated and vegetating (Fig. 1). The general condition of the patient was good. There was no lymphadenopathy. Histopathologic examination revealed a proliferation of fusiform cells in interlacing bundles (Fig. 2). The cytoplasm was eosinophilic and occasionally fibrillar. The nuclei were elongated with rounded

Bronchogenic Cyst Presenting as a Nodular Lesion

Abstract: We report a subcutaneous bronchogenic cyst in a 7‐year‐old girl with unusual presentation as a nodular lesion. The cyst was excised and no connection with deeper structures was found. Histologic examination showed the typical features of a bronchogenic cyst. The characteristic clinical and histologic features of bronchogenic cysts are reviewed. The problems of differential diagnosis are also discussed.

Dermoscopy of tumours arising in naevus sebaceous: a morphological study of 58 cases

Naevus sebaceous is a congenital hamartoma commonly associated with the development of secondary neoplasms. There are sparse data relating to the dermoscopy of tumours arising in naevus sebaceous.

Síndrome de Brooke-Spiegler : una entidad heterogénea

The Brooke-Spiegler syndrome is a rare, autosomally dominant disease with a predisposition to develop different adnexal tumors. Clinically it is characterized by the presence of multiple cylindromas, trichoepitheliomas, and occasionally, spiradenomas. Although Brooke-Spiegler syndrome, familial cylindromatosis and multiple familial trichoepithelioma were initially described as separate entities, the recently identified identical mutations in the gene of cylindromatosis suggest that they represent fenotypic variations of the same entity. In this article we present the case of a woman and her daughter, both affected by this rare genodermatosis.Resumen El sindrome de Brooke-Spiegler es una genodermatosis infrecuente, de transmision autosomica dominante, que predispone a la aparicion de diferentes neoplasias anexiales. Clinicamente se caracteriza por la presencia de multiples cilindromas, tricoepiteliomas y, ocasionalmente, espiradenomas. Aunque el sindrome de Brooke-Spiegler, la cilindromatosis familiar y el tricoepitelioma multiple familiar fueron inicialmente descritos como entidades distintas, recientemente se han identificado identicas mutaciones en el gen de la cilindromatosis, por lo que se ha sugerido que representan variaciones fenotipicas de una misma entidad. En este articulo presentamos el caso de una mujer y su hija, ambas afectas por esta rara genodermatosis.