A study of nineteen immunocompromised patients with extensive skin lesions caused by Pseudomonas aeruginosa with and without bacteremia.

Nineteen immunocompromised patients with extensive skin lesions caused by Pseudomonas aeruginosa with or without P. aeruginosa bacteremia were analysed. Patients whose lesions originated in the skin were in the majority (14 patients). Skin lesions were located at the site of entry of bacteria (apocrine areas in 12 patients). Cutaneous lesions were pleomorphic but the typical picture of ecthyma gangrenosum was common in this group of patients. Only 2 of them developed P. aeruginosa septicemia and the prognosis was relatively good (7.5% mortality rate). These observations confirm that ecthyma gangrenosum may be a primary cutaneous disorder not systematically associated with bacteremia.

A variety of tumours induced by the middle T antigen of polyoma virus in a transgenic mouse family.

A transgenic mouse family expressing the middle T antigen of polyoma virus under control of the immunoglobulin heavy chain (IgE) enhancer showed the frequent occurrence of carcinomas in various organs, predominantly in females. Most frequently affected were the salivary and thyroid glands, but mammary tumours, liver haemangiomas and adenocarcinomas of unknown origin were also observed. In all tumours, the middle T antigen was found to be complexed with cellular tyrosine kinases. These results extend the range of tumour types associated with in vivo expression of middle T and with the subsequent deregulation of pp60c-src and related tyrosine kinases.

Dermatomyositis-like syndrome in X-linked hypogammaglobulinemia. Case-report and review of the literature.

A case of dermatomyositis-like syndrome is described in a 19-year-old man with a history of Bruton's hypogammaglobulinemia. Although the patient had central-nervous-system manifestations (seizures), no echovirus was isolated in the cerebrospinal fluid, in contrast to previously reported cases. Data for our case and the 15 cases previously reported in the literature are reviewed. HLA typing of our patient revealed the presence of HLA B8 and DR3, which seems to play a major role in juvenile dermatomyositis.

Hyperpigmented acral papular mucinosis, systemic lupus erythematosus and universal alopecia.

A 42-year-old man presented with systemic lupus erythematosus, universal alopecia and non-pruritic hyperpigmented papular mucinosis. The latter was most evident on acral areas. In hyperpigmented areas of the face the immunofluorescence showed deposits as in LE and with alcian blue and colloidal iron an abundance of mucin was demonstrated in the dermis. A lesion on the back showed only papular mucinosis. Fifteen cases of LE and papular mucinosis reported in the literature are reviewed. Our patient differs with respect to the marked pigmentation of his lesions, their localization and the association with universal alopecia.

High affinity binding of the large T protein of polyoma virus to a genomic mouse DNA sequence.

We purified a fragment of mouse DNA to which the large T protein of polyoma virus was bound in chromatin prepared from transformed mouse cells. This sequence, which is not repeated to a measurable extent within the mouse genome, does not show any significant homology to the viral ori region, except in a short region, which comprises a sequence related to the consensus for recognition by large T proteins ((A,T)GPuGGC). This region of pCG4 was confirmed by in vitro binding assays to be essential for T antigen binding.

Disseminated pagetoid reticulosis associated with mycosis fungoides: immunomorphologic study.

We report a 77-year-old woman with erythrosquamous plaques on the legs for 10 years. Biopsies taken 4 years ago revealed a pagetoid reticulosis with a massive epidermal cell infiltrate. She has now also developed a typical tumor of mycosis fungoides. The epidermal infiltrate of the two types of lesions bore the surface membrane marker for T lymphocytes rich in both T helper and T cytotoxic-suppressor subsets (ratio, 1.8). Both lesions also showed dendritic OKT6-positive Langerhans cells, and staining of the intercellular material with the DR locus of human lymphocyte antigen was positive. A dense dermal infiltrate was evident only in the mycosis fungoides lesions, and it was similar to that in the epidermis. Electron microscopy of the two lesions showed the atypical cells described in mycosis fungoides and the Sézary syndrome. These observations suggest that disseminated pagetoid reticulosis probably is a variant of mycosis fungoides.

Nevoid hyperkeratosis of the nipple and areola mammae: ineffectiveness of etretinate therapy.

A woman with nevoid hyperkeratosis of the nipple and areola appearing during puberty is described. It remained unchanged during two pregnancies and caused no problem when breast feeding her two children. Etretinate (1 mg/kg/day) was ineffective in the treatment of this dermatosis.

Epidermolysis bullosa acquisita with negative direct immunofluorescence.

Epidermolysis bullosa (EB) appeared in a patient at the age of 54 years. Other bullous disorders could be excluded by electron microscopy, and there was no family history of EB. The patient would therefore best be classified as having EB acquisita. Repeated direct immunofluorescence studies were, however, negative for all tested serum samples, suggesting that there might be a subgroup lacking immunoglobulin deposits in the skin. Collagen IV, laminin, and fibronectin were expressed normally at the dermoepidermal junction.

Pseudomonas aeruginosa O-11 folliculitis. Development into ecthyma gangrenosum in immunosuppressed patients.

Six immunocompromised patients were shown to exhibit Pseudomonas aeruginosa folliculitis in apocrine regions similar to "swimming pool" folliculitis. The lesions evolved within 24 hours as severe ecthyma gangrenosum. The source of the P aeruginosa, serotype O-11, was found in the water system of the hospital. Clinical identification of the lesions and early treatment is important to prevent severe manifestations.

Familial rolled and spiral hairs with palmoplantar keratoderma.

A 59-year-old man with palmoplantar keratoderma and rolled spiral hairs on the abdomen and extremities is reported. His father had the same skin manifestations but his brother and sister only keratoderma palmoplantare. Scanning electron microscopy of the rolled hairs showed that they were coiled in a spiral around their own axis. These spiral hairs had lower cysteine than the normal appearing hairs on the body. The scalp hair appeared normal but was low in cysteine which was compensated by an increase in threonine. Urine analysis showed a decrease of cysteine.

Response of epidermal polyamines to orally administered aromatic retinoid RO 10-9359.

Polyamine levels were measured in skin (pure epidermis) and 24-h urine before and 15 days after the start of continuous oral treatment with Etretinate (1 mg/kg/day) in 20 patients with various dermatoses. In uninvolved epidermis, treatment modified levels of spermidine (45% increase, P less than 0.05) and spermine (30% increase, P less than 0.05). In urine, the putrescine concentration was significantly altered, increasing from 1.96 to 2.60 micrograms/mg creat (P less than 0.05). During the time interval considered, variations in polyamine levels did not reflect the inhibiting mechanism of retinoids on ornithine decarboxylase, the key enzyme in the regulation of polyamine synthesis.