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Hermansky-Pudlak syndrome is a rare, inherited, autosomal recessive disease. Diagnosis is based on a triad of signs: oculocutaneous albinism, a hemorrhagic tendency due to a platelet disorder, and an accumulation of lipopigments in different organs, particularly the medullary macrophages. We describe a child with the characteristic findings of this syndrome, which often goes unrecognized because of the discrete nature of the cutaneous and hemorrhagic manifestations. This diagnosis is important because of the risk not only of hemorrhage but also of granulomatous colitis and long-term pulmonary fibrosis.

Microascus cirrosus is very rarely the aetiological agent of onychomycosis. We report two additional cases of toenail infections caused by this fungus.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin disorders) is a rare multisystemic disease associated with plasma cell dyscrasia. A 68-year-old woman with chronic renal insufficiency and arterial hypertension included in her medical history was admitted to the hospital with confusion, somnolence and asthenia. She presented ascites, hepatosplenomegaly, leg oedema, distal dysesthesias, leuconychia and multiple nodular purple red angiomas on the trunk, upper limbs and fingers. Hypothyroidism was revealed in the laboratory investigations and monoclonal IgG peak in immunoelectrophoresis. Electromyography showed both demyelinisating and axonal degenerative neuropathy. The diagnosis of POEMS syndrome was based on the dermatopathological examination of a cutaneous angioma; histology revealed features of glomeruloid angioma, a specific marker of this syndrome.

Background: Cosmetics have been used since the oldest known civilisations, and nowadays almost everybody resorts to beauty products. Objective: Considering the increasing incidence of contact dermatitis, the aim of the study is to determine the impact of allergy to cosmetics. Methods: From January 1998 to December 1999, 819 patients were subjected to epicutaneous tests for suspicion of allergic contact dermatitis (ACD). The results were analysed retrospectively in the total population and in that of children under 16: the interest has been focused on cosmetics. Results: 297 patients (36.3%) with ACD to 1 or more cosmetic ingredients have been detected. 34 (48.6%) out of 70 children patch tested showed an ACD: cosmetics represented the first cause. Conclusion: For two decades, the incidence of ACD has been rising; two main reasons can be put forward: a rising product consumption and a more exhaustive allergen research in patch testing.

Background. Experimental data suggest that there is an imbalance between Th1 and Th2 cells in atopic dermatitis (AD) skin compared to allergic contact dermatitis (ACD). This imbalance (Th2 and Th1 predominance, respectively) implies the production of different cytokines in these two conditions leading to different expression of adhesion molecules on skin endothelial cells. Objective. The expression of VCAM-1 (IL-4/Th2-dependent) and ICAM-1 (INF-γ/IL-1) on dermal vessels was compared in six patients with AD and six patients with ACD. The effect of cetirizine, a highly selective H1-receptor antagonist on the expressions was studied. Methods. Six patients with AD were challenged with Dermatophagoides pteronyssimus (DPT patch tests applied to clinically normal skin) and six patients with ACD challenged in the same way with allergens of the European standard series. Skin biopsies at challenged sites were performed before and 6, 24 and 48 h after challenge. The experiment was carried out under double-blind cross-over conditions during a 4-day treatment with a placebo and cetirizine. Results. In AD patients, the scores for both VCAM-1 and ICAM-1 were high before and after challenge. In ACD patients, the ICAM-1 score was high at each experimental time, but the VCAM-1 score, which was significantly lower before challenge, increased at 6, 24 and 48 h after challenge. The administration of cetirizine significantly reduced the VCAM-1 expression in AD patients at each experimental time. Conclusion. It is concluded that the increased VCAM-1 expression in AD patients compared to ACD may reflect greater IL-4 and/or IL-13 production in situ. The study also confirms the existence of a modulating effect of cetirizine in vivo on adhesion molecule expression.

Hereditary progressive mucinous histiocytosis is a rare autosomal dominant non-Langerhans cell histiocytosis. We describe a sporadic case of this syndrome in a 64-year-old woman who had multiple dark-red dome-shaped papulonodules located mainly on the back of her hands, forearms and thighs. Light microscopy revealed a circumscribed upper dermal aggregate of ovoid or spindle-shaped histiocytes with abundant mucin deposition. Iron deposits and numerous mast cells were scattered throughout the tumour but giant cells were rare. Electron microscopy revealed a high number of zebra bodies and myeloid bodies in the cytoplasm of the histiocytes. Immunohistochemistry showed positive labelling with alpha-1 antitrypsin, Factor XIIIa and CD68, while CD1a, CD34 and S100 protein were negative. The differential diagnosis of histiocytic syndromes is discussed.

Standardized skin surface biopsy (SSSB) is an appropriate method for measuring the density of Demodex folliculorum (Dd). Nevertheless, the observation of a clinical case demonstrates that, contrary to what was presumed before, a single SSSB can fail to collect the complete biotope of the mite. This could give rise to false negative results regarding the measurement of Dd.

Background: Alopecia areata (AA) is a polygenic immune-mediated disorder affecting the hair follicle for which an association with human leukocyte antigen HLA-DRB1*11 has been described. Objective: Two parameters including age of onset and extent of the disease (patchy AA and AT/AU forms) were correlated with the presence or absence of HLA-DRB1*11 and its alleles in 88 severe AA patients. Methods: Patients and healthy controls were typed for HLA-DR and -DQ by molecular method. Results: Among AA patients, 37.5% (a proportion rising to 72% when taking patients who began their first patch before the age of 20 years) were positive for HLA-DRB1*11 compared to 21.2% healthy controls (p = 0.004, RR = 2.1). DRB1*11-positive status was associated with earlier development of the first AA patch, at the mean age of 16 years compared to 27 years (p = 0.003) in DRB1*11-negative patients. Among the DRB1*11 alleles, the presence of DRB1*1104 was associated with the earliest occurrence of AA. Conclusion: Our data indicate that the HLA system largely through DRB1*1104 allele influences AA onset rather than extension considering patchy AA and AT/AU.

We report the case of a second patient with the extraordinary ultrastructural findings of vacuolated structures intermingled with membranes in the perinuclear part of the upper epidermal cells. Clinical, light microscopic, and electron microscopic features of this particular presentation of ichthyosis congenita type III have already been presented by K. M. Niemi and L. Kanerva in 1989. Although our patient has more or less the same light and electron microscopic findings, the clinical picture is more severe. The patient was born as a collodion baby. Later, he showed signs of generalized severe involvement with large scales, erythrodermia, and itching. Successful therapy with retinoids resulted in complete removal of the hyperkeratosis but left the striking reticulate skin pattern. Noting the heterogeneous clinical presentation, the specific electron microscopic findings are diagnostic. No biochemical data on this disease are known.

Sarcoidosis is a systemic disease of unknown etiology. The diagnosis is based on consistent clinical, biological and radiological findings supported by histologic evidence of noncaseating epithelioid granulomas. Skin lesions occur in about a quarter of patients with sarcoidosis. The purpose of this article is to analyse through the clinical presentation of dermatological lesions, a potential link with systemic disease.

Three children will be described who present recurrent episodes of pruritic papulopustular follicular lesions on the face, the extremities and the trunk. The episodes lasted for 1-3 months with intermittent remission. Each flare was accompanied by hypereosinophilia and an increased total IgE titer. RAST and prick tests were positive for Dermatophagoides pteronyssinus (DPT). Laboratory tests disclosed no infectious or parasitic etiology. Histological examination showed eosinophilic pustular folliculitis (EPF) in each of the 3 cases. The lesions responded well to topical corticosteroids. The aim of this article is to underline the importance of hypersensitivity reactions (in these particular cases to DPT) in the pathogenesis of EPF.

A case of early congenital syphilis is reported in order to emphasize that syphilis is still present nowadays and that detection of syphilis in pregnant women is primordial for the prevention of this disease.

Basal cell carcinoma (BCC) is the most common malignant skin tumour. In the last few years, the incidence of multiple BCC has also increased in young patients. We describe the clinical case of a young 29-year-old woman who developed 7 BCC on her abdomen during her first pregnancy and 4 other similar tumours 2 years later during her second pregnancy, all located on the abdomen. Polymerase chain reaction revealed the presence of numerous human papillomavirus DNA sequences. To our knowledge, such a clinical presentation has not been previously reported. Different physiopathological considerations are discussed.

We report a case of a cutaneous Mycobacterium chelonae infection on the dorsum of the forearm of a patient undergoing chronic hemodialysis. The infection showed a linear and distal extension. This unusual distal dissemination was apparently secondary to a venous reflux, a circulatory repercussion of the patient’s arteriovenous fistula.

A 49-year-old man presented with a tumoral lesion of the tip of the nose which had been manifest for 2 months. Within a few weeks, the tumour increased in size and became infiltrated. The biopsy showed a squamous cell carcinoma. Treatment consisted of a radical surgical excision.

Acitretin (Ro 10-1670 or Neotigason), a free acid and the main metabolite of etretinate (Ro 10-9359 or Tigason), is the most recent of the retinoids used orally in the treatment of psoriasis and numerous other dermatoses exhibiting disorders of keratinization. In the majority of cases, its side-effects are similar to those of hypervitaminosis A.

A male infant was born with generalized erythroderma and scaling; the newborn demonstrated poor neonatal development and developed several complications such as hypernatremic dehydration, septicemia, gastroenteritis and seizures. In the neonatal period, the erythema faded, but exfoliation persisted. The parents are healthy but related. One older brother, who died at the age of 3 months, had shown the same clinical picture in the neonatal period and was diagnosed with congenital psoriasis. All clinical investigations, including serum immunoglobulins, complement levels and lymphocyte counts, were normal. Only raised total IgE and multiple positive specific IgE reactions were noted. Skin biopsy revealed an image of ichthyosis. Polarization microscopy of scalp hair showed trichorrhexis nodosa and discrete focal twisting of the hair shaft. This clinical picture and all histological findings are compatible with the indications of Netherton's syndrome. The purpose of this report is to call attention to this severe presentation of congenital ichthyosis in the neonatal period and to the difficulty of a correct diagnosis when confronted with congenital erythroderma.

We report a case of cholesterol crystal embolism showing typical cutaneous features as livedo reticularis and purple toes and a hitherto undocumented manifestation in the form of punctiform subungual haemorrhages.

We report a case of recurrent vulvar ulcerations developed on vegetations associated with Crohn’s disease. Lesions responded to low-dose thalidomide treatment. A biopsy revealed a neutrophilic infiltrate with rare giant cells. Vulvar manifestations were consistent with metastatic Crohn’s disease or with a reactive neutrophilic dermatitis. Thalidomide reduces the activity of tumor necrosis factor (TNF) α by accelerating the degradation of its mRNA. Nowadays, it has become a promising drug in a broad variety of disorders in which TNF-α seems to play a pivotal role, including inflammatory bowel diseases. This is the first case report presenting the efficacy of thalidomide in the treatment of vulvar involvement in Crohn’s disease.

Background: We found pre-established directives inadequate to cope with the current increase in anthropophilic tinea capitis in Brussels. Objectives: To study new epidemiological profiles and to define new strategies for management and prevention. Patients and Methods: A total of 122 children affected by tinea capitis were followed in our department from October 1, 2001, until September 30, 2002. The results were assessed retrospectively. Results: Anthropophilic tinea capitis represented 89.34% of the cases. The implicated anthropophilic dermatophytes were by decreasing frequency: Microsporum langeronii (39.34%), Trichophyton soudanense (28.69%), Trichophyton violaceum (18.03%) and Trichophyton tonsurans (3.28%). Conclusion: The responsible pathogens reflect immigration flows originating mostly from Black and North Africa. Precise recommendations for each visit are detailed.

We report a case of a cutaneous Mycobacterium chelonae infection on the dorsum of the forearm of a patient undergoing chronic hemodialysis. The infection showed a linear and distal extension. This unusual distal dissemination was apparently secondary to a venous reflux, a circulatory repercussion of the patient's arteriovenous fistula.

Eccrine syringofibroadenoma (ESFA) is a rare eccrine tumor. We report the case of a 70-year-old woman who had presented a pincer nail deformity on the toes for 55 years and a left painless subungual tumor for 2 years. Histologic examination revealed branching anastomosing strands of epithelial cells with ductal structures in a fibrovascular stroma. The luminal cells showed positive staining for carcinoembryonic antigen, as in acrosyringeal cells and cells of dermal ducts. To our knowledge, our case is the first with a subungual location. In the context of chronic trauma due to a pincer nail, it may be classified in the reactive subtype recently described.

A 2-month-old boy experienced cutaneous lesions that revealed an underlying severe, combined immunodeficiency (SCID). It is important to recognize cutaneous manifestations of primary immunodeficiency disorders, as they may provide the earliest clue to a defect in immune function.

We report a case of acne neonatorum developed in a boy since birth. His mother and his aunt had different signs of hyperandrogenism including acne and hirsutism. This case illustrates the correlation between acne neonatorum and familial hyperandrogenism. It shows the importance of the family history and the eventual clinical and serological presence of hyperandrogenism in the mother when confronted with acne neonatorum.

Tufted angioma is a rare acquired cutaneous angiomatous proliferation characterized by slowly spreading erythematous macules and plaques, sometimes surmounted by nodular formations. Synonyms are progressive capillary hemangioma and Nakagawa's angioblastoma. Tufted angioma appears to be benign since follow-up studies did not report malignant transformation. This lesion must be distinguished from other vascular tumors, especially from Kaposi's sarcoma. If this lesion appears in an older patient, angiosarcoma should be excluded.

Docetaxel (RP 56976 or Taxotere), a semisynthetic drug belonging to the family of taxoids, is a new chemotherapeutic agent used in phase II trials for breast, ovarian and lung cancer. We report 2 cases of subungual hemorrhages, which, to our knowledge, have yet to be cited in association with the use of docetaxel. Although not incapacitating, the patient should be made aware of the potential risk of this drug reaction.

Seven children with severe, rapidly evolving alopecia areata (AA) were treated with pulse steroid therapy (PST). AA had been present for 3-44 weeks and involved more than 30% of the scalp. One patient had alopecia totalis. Intravenous methylprednisolone (5 mg/kg twice a day) was administered for 3 days. No serious side effects were noted. At the 12-month follow-up, complete regrowth had occurred in 5 patients (71%). The patient with alopecia totalis had no regrowth. PST appears to be a promising and safe treatment for extensive, recent-onset AA in children. No response is to be expected in alopecia totalis or long-standing AA.

We report the cases of 2 women presenting allergy to paraphenylenediamine (PPD). Both patients had a history of eczema that worsened following the ingestion of the antihistamine Polaronil® (dexamethasone/dexchlorpheniramine). This clinical presentation could be explained by cross-sensitivity to sulfanilic acid (4-aminobenzene sulfonic acid), a metabolite of sunset yellow (FD&C No. 6). Sunset yellow is an azo dye present in this tablet. Indeed, PPD-allergic subjects may suffer from cross-sensitivity to related compounds, especially to those that can be ingested such as azo dyes. Such compounds are used in some instances by the food and pharmaceutical industries, but their presence is often undisclosed.

Epithelioid sarcoma (ES) is a rare soft tissue neoplasm that usually occurs on the upper extremity of adolescents and young adults. It rarely occurs during childhood. ES is a slowly growing tumor with a high propensity for recurrences and metastases. This neoplasm is likely to be confused with a variety of benign and malignant conditions. Recognition of ES is important. The treatment consists of wide excision. Misdiagnosis can lead to inappropriate treatment and local recurrences or metastases. We report the case of a 5-year-old girl with an ES on the right forefinger.

Various skin disorders are claimed to be commonly associated with human immunodeficiency virus (HIV) infection. Actually several of these skin diseases, including dermatomycoses are not more prevalent in HIV infected individuals as compared to the general population (Fisher and Warner, 1987; Valle, 1987; Goodman et al., 1987; Sindrup et al., 1987). The difference, as will be shown by other participants at this symposium, lies in the unusual presentation and the severity of the clinical manifestations.

A case of basal cell nevus syndrome or Gorlin's syndrome is reported in a newborn. The skin condition is associated with congenital hydrocephaly and skeletal malformations. To our knowledge, this is the first case of basal cell nevus syndrome with skin tumors present at birth and localized on the fingers.

Aspergillus, which has a saprophytic life, is an opportunistic fungus and manifests his pathogenicity when a weakened state of the host facilitates its development. The conditions are particularly favorable in severely burned patients like the 54-year-old woman presented here. The criteria of pathogenicity of this opportunistic fungus, the incidence of epidemiology and prognosis of this type of infection and the clinical signs that allow an early diagnosis are discussed here.

The frequency of positive patch tests to rubber additives in Belgium is recorded. Allergy due to rubber protective gloves represents 41% of the overall sensitivity to rubber.

Seborrhoeic dermatitis, psoriasis and atopic dermatitis in infants were studied morphologically and autoradiographically on skin biopsies. A similar picture with an increased germinative cell population and accelerated cell turnover has been found in psoriasis and seborrhoeic dermatitis. These features may suggest a relationship between both diseases.

The authors report 2 cases of bullous dermatoses in the child. Nowadays Duhring-Brocq dermatitis is divided into three closely related affections: bullous pemphigoid, dermatitis herpetiformis, and the benign chronic bullous dermatosis of the child. The clinical, histological, ultrastructural, and immunofluorescence features and also the prognosis of the therapeutic response of these three affections are recapitulated.

Dominant dystrophic epidermolysis bullosa of Cockayne-Touraine occurred in a father and his son. Clinically, the child presented evident blistering, while the father, despite of his work, where minimal skin traumata frequently occur, was rarely affected. Absent or rudimentary anchoring fibrils were seen in the normal-appearing skin of the father; this suggests that other factors may play a role in the dermolytic separation.