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Cutaneous Waldenström's macroglobulinaemia.

We report a case of Waldenström's macroglobulinaemia that presented with infiltrated skin nodules and plaques but without systemic symptoms. Cutaneous manifestations such as purpura, oedema, urticaria and ulceration may be seen in Waldenström's macroglobulinaemia and are a consequence of hyperviscosity, cryoglobulinaemia and/or tissue deposition of immunoglobulins. Direct cutaneous infiltration by neoplastic lymphoid cells is less common, and very rare as the initial presentation of Waldenström's macroglobulinaemia.

Aged↗

Identification of a recurrent mutation in the CYLD gene in Brooke-Spiegler syndrome.

Brooke-Spiegler syndrome is an autosomal dominantly inherited disease with predisposition to neoplasms of the skin appendages. The disease has been mapped to 16q, and mutations in the CYLD gene have been identified in families with this disorder. We describe an individual with BSS exhibiting clinical heterogeneity in which a heterozygous frameshift mutation in CYLD, 2172delA, has been identified. These findings extend the body of evidence that mutations in CYLD are involved in Brooke-Spiegler syndrome and provide additional information for phenotype-genotype correlation.

Aged↗

Carney complex and the familial lentiginosis syndromes: link to inherited neoplasias and developmental disorders, and genetic loci.

Lentigines, synonymous but not identical to freckles or ephelides, are common skin lesions. In a small number of patients, however, these lesions constitute part of genetic syndromes that are associated with inherited forms of neoplasias or other pathologic processes of the cardiovascular, endocrine, and gastrointestinal systems. The familial lentiginoses, as these syndromes are collectively known, include Carney complex, the LEOPARD and Peutz-Jeghers syndromes, and the newly described 'syndrome of arterial dissections with lentiginosis'; isolated, familial lentiginosis has also been described. In the majority of the reported kindreds with these syndromes, the lesions were inherited in an autosomal dominant manner. The specific genes that are responsible for these disorders have remained elusive, but the genetic loci of Carney complex and Peutz-Jeghers syndromes were recently identified on chromosomes 2p 16 and distal 19p, respectively. Cytogenetic studies of tumours from patients with Carney complex suggest that the gene responsible for most patients with this syndrome may not have a tumour suppression function. We suggest that the genes responsible for the lentiginosis syndromes are important regulators of melanocyte function, they participate in the pleiotropy of human pigmentation, and are involved in the function, growth and proliferation of neural crest and mesenchymal cells.

Diagnosis, Differential↗

Granuloma faciale: successful treatment of nine cases with a combination of cryotherapy and intralesional corticosteroid injection.

BACKGROUND: Granuloma faciale is a rare disorder characterized by asymptomatic papules, nodules, and plaques on the face. Although the exact pathogenesis of this disease in unclear, it is considered a variant of leukocytoclastic vasculitis confined to the skin. Several medical and surgical methods have been used to treat it with variable results. CASE REPORTS: We report nine cases of granuloma faciale treated with a combination of cryotherapy with liquid nitrogen and intralesional injection of corticosteroids. RESULTS: The lesions cleared completely in all of the patients without any side-effects. No recurrences have been observed. CONCLUSIONS: Cryotherapy with liquid nitrogen, followed by intralesional injection of corticosteroids, is a safe and effective method to treat granuloma faciale.

Adrenal Cortex Hormones↗

The progression of nonsegmental vitiligo: clinical analysis of 318 patients.

BACKGROUND: Vitiligo is an acquired disorder with destruction of melanocytes and is characterized by cutaneous depigmentation with a progressive clinical course. We attempted to evaluate whether there was a relationship between the initial site and ultimate progression of nonsegmental vitiligo. METHODS: Three hundred and eighteen nonsegmental vitiligo patients were examined. Questionnaires regarding age, sex, duration of disease, family history, distribution of initial lesions, progression, and clinical type were recorded from personal interviews and case notes. RESULTS: Two hundred and eighty nine (90.9%) of the 318 patients showed progression of vitiligo at the time of examination. When the initial sites were the posterior trunk, hands, or feet, there was more progression of vitiligo to other body areas. The progression pattern was usually contiguous to the initial site. When the hands were the initial site, however, vitiligo most commonly progressed to the face. CONCLUSIONS: There may be a possible relationship between the initial site and ultimate progression of nonsegmental vitiligo. The progression pattern is usually contiguous to the initial site.

Back↗

The significance of Demodex folliculorum density in rosacea.

BACKGROUND: Demodex folliculorum has been reported in rosacea in a number of clinical studies. As the Demodex mite is also present in many healthy individuals, it has been suggested that the mite may have a pathogenic role only when it is present in high densities. Moreover, some authors have proposed that a mite density above 5/cm2 may be a criterion for the diagnosis of inflammatory rosacea. In this study, the possible role of D. folliculorum and the importance of mite density in rosacea were investigated using a skin surface biopsy technique. METHODS: Thirty-eight patients with rosacea and 38 age-and-sex-matched healthy subjects entered the study. With the skin surface biopsy technique, we obtained samples from three facial sites. We then determined the mite positivities, the mean mite counts in both study groups, the mean mite densities at each facial site and in the rosacea subgroups, and the mite densities above 5/cm2. RESULTS: The mean mite count in the rosacea group (6,684) was significantly higher than that in controls (2,868; p < 0.05). The cheek was the most frequently and heavily infested facial region. Ten rosacea patients and five normal subjects had mite densities over 5/cm2; the difference was not statistically significant (p > 0.05). CONCLUSIONS: Rosacea is a disease of multifactorial origin, and individual properties may modify the severity of the inflammatory response to Demodex. We suggest that a certain mite density is not an appropriate criterion in the diagnosis of the disease; nevertheless, large numbers of D. folliculorum may have an important role in the pathogenesis of rosacea, together with other triggering factors.

Adult↗

A questionnaire study of road pavers' and roofers' work-related skin symptoms and bitumen exposure.

BACKGROUND: Skin contact with the components in asphalt and bitumen can lead to irritant and allergic contact dermatitis, but few data are available in the dermatologic literature on the skin symptoms caused by work with bitumen. In addition, working methods have changed markedly during recent decades. METHODS: A questionnaire was delivered to 50 roofers and 101 road pavers. The questionnaire dealt with skin symptoms, symptoms caused by solvent products, the use of personal protection, smoking, eating habits, work conditions, changing and cleaning of overalls, etc. RESULTS: Forty-eight per cent of the road pavers and 58% of the roofers responded to the questionnaire. Relatively high percentages of work-induced skin irritation were reported by both the roofers (44%) and the road pavers (31%); 22% of the road pavers had dermatitis often or sometimes compared with 15% of the roofers. The hands, arms, face, and lower extremities were the most common sites affected. In addition to bitumen products, the road pavers considered amine adhesion-improving agents for paving and naphtha and solvents used in machine repairs, to be the main causes of their skin symptoms. The skin problems of the roofers were caused by man-made mineral fibers, cutback bitumen, and hot bitumen burns. CONCLUSIONS: Roofers endure greater exposure to chemicals than road pavers. Certain components, e.g. polycyclic aromatic hydrocarbons in bitumen, can be absorbed through the skin. Skin exposure should be lowered by keeping the tools, working clothes, shoes, and gloves clean. Overalls and gloves are recommended to be changed at least once a week. Water laundering is not sufficient in dissolving bitumen from overalls and underwear. Cleaning the skin with solvents or naphtha is not recommended, as they are skin irritants.

Alkanes↗

Relevance of complement fixing antinuclear antibodies.

BACKGROUND: Connective tissue diseases (CTDs) are a heterogeneous group of disorders defined by the association of a variety of clinical manifestations with immunologic and other laboratory findings. Overlap of syndromes and aberrant findings appear rather frequently. METHODS: Sera of eight antinuclear antibody (ANA) negative, cases of subacute cutaneous lupus erythematosus (SCLE) with antibodies to Ro (SS-A) and a ninth case with clinical and laboratory signs of Sjögren's syndrome and systemic lupus erythematosus (SLE) were tested for complement (C') fixing antinuclear antibodies (C-ANAs). The ninth case was examined in depth by direct immunofluorescence (DIF) and a two-step "C + DIF" test of biopsies for C' fixation to in vivo bound ANAs, as well as serum tests for C-ANA, ANA, and SCLE markers. RESULTS: Sera of five of the eight ANA negative, Ro(SS-A) positive SCLE cases had C-ANAs. The ninth case, a 50-year-old woman with clinical and laboratory signs of Sjögren's syndrome and SLE, gave a strong positive C + DIF reaction in the skin biopsy for in vivo bound ANAs that fix C', but negative ANAs and C-ANAs in routine serum tests; they revealed antimitochondrial antibodies. Serum tests on normal skin, however, revealed weak ANA and strong C-ANA reactions with in vitro fixed C'. CONCLUSIONS: ANA negative cases of SCLE or Sjögren's syndrome may have C-ANAs. A case with Sjögren's syndrome and signs of SLE had both in vivo and in vitro C' fixing ANAs. C-ANA tests can aid in the identification of such cases.

Antibodies, Antinuclear↗

Acneiform eruptions after facial beauty treatment.

BACKGROUND: Female patients in the post-adolescent age group presented with predominantly deep-seated nodules and a few comedones situated mainly on the cheeks. As most of these subjects related the onset of their symptoms to antecedent facial beauty treatment, we decided to study the clinical and histologic profile of these patients. METHODS: Thirty seven subjects (36 women and one man) were questioned in detail about their acneiform eruptions. The patients were examined and a biopsy of typical lesions was taken in eight patients. RESULTS: On direct questioning, all patients related the onset of their lesions to facial beauty treatment taken 3-8 weeks previously. The predominant types of lesion were deep-seated nodules, although a few closed comedones were present in some cases. Most lesions took a long time to heal and, on healing, left behind hyperpigmentation. The cheeks were universally involved in all patients, and the chin and forehead were involved in 14. The histopathologic study revealed a predominantly peri-appendageal dermal infiltrate consisting of lymphocytes and histiocytes admixed with polymorphs. A granulomatous infiltrate was seen in one-third of the biopsies. CONCLUSIONS: This eruption is unlike the earlier eruption described as acne cosmetica in being inflammatory, indolent, and often occurring after the first cream massage itself.

Acneiform Eruptions↗

Allergic contact dermatitis in the United Arab Emirates.

BACKGROUND: No data exist on allergic contact dermatitis (ACD) in the United Arab Emirates (UAE). AIM: Our aim was threefold: (i) to determine the incidence of ACD; (ii) to identify responsible allergens using the European Standard Series, TRUE TEST, and other substances; (iii) to tentatively explore population-specific reactions in Al Ain, UAE. DESIGN: This is a prospective, descriptive, hospital-based, study. SETTING: Tawam Hospital, Al Ain Medical District, UAE. PATIENTS: During the years 1989-1996, 373 patients (male: female = 2 : 3), presenting with cutaneous manifestations possibly related to contact allergy, were patch tested. RESULTS: 93.8% of patients presented with dermatitis affecting mostly the hands (45.1%), feet (21.4%), face (12.6%), and legs (4.6%). Two hundred and twenty-four patients (60%) (male : female = 1 : 2) tested positive for at least one substance. Nickel was the commonest sensitizer (15%) in both genders. "Fragrance mix" (8%), p-tertiary butylphenolformaldehyde (p-TBPF) resin (7.5%), thiomersal (7.5%), chromium (7.2%), cobalt (6.4%), ethylenediamine (6.4%), neomycin (5.1%), and parabens (5.1%) were prominent allergens. Glutardialdehyde, an additional substance tested together with the European Standard Series, scored significantly (4.8%). Variations from the results reported from other countries may be explained by the specific lifestyles and customs of the UAE population. CONCLUSIONS: Substances contained in the TRUE TEST, plus glutardialdehyde, may be considered as the standard series for patch testing in the UAE population.

Adolescent↗