Anna Maria Carrozzo

Unusual spindle cell squamous carcinoma in a renal transplant patient.

The histopathological diagnosis of spindle cell tumours is often difficult. We present a patient who developed a painful tumour on his head 6 years after renal transplantation. Histologically, the tumour was first interpreted as scar tissue with an unusual fibrohistiocytic component. Cytokeratins, desmin, CD31, CD34 and S100 could not be detected using conventional immunohistochemistry. After two recurrences and intensive immunohistochemical examinations, cytokeratin-positive tumour areas in direct connection with the epidermis were detected. The final diagnosis was therefore spindle cell squamous carcinoma. The patient finally died from brain metastases after experiencing further painful recurrences that were removed surgically. Our case underscores the importance of refined immunohistochemical methods in establishing the diagnosis of spindle cell squamous carcinomas, methods that should be taken into account especially if painful spindle cell tumours arise on sun-exposed skin in immunosuppressed patients.

Diagnosis of pigmented skin lesions by dermoscopy: web-based training improves diagnostic performance of non-experts

Summary Background Dermoscopy has been shown to enhance the diagnosis of melanoma. However, use of dermoscopy requires training and expertise to be effective.

Pachydermoperiostosis: study of epidermal growth factor and steroid receptors

Pachydermoperiostosis is a rare osteo‐cutaneous disease characterized by hypertrophy of bones and surrounding soft tissues. The cutaneous manifestations include coarsening of facial features, cutisverticis gyrata, digital clubbing, hyperhidrosis and scborrhoea.

TREATMENT OF INFLAMMATORY LINEAR VERRUCOUS EPIDERMAL NAEVUS WITH CALCIPOTRIOL

white, milk, wheat, soybean, moulds {PeniciUium. Cladosporium. Candida. AUernaria and AspergiUus). mites, ceder, house dust, and Pityrosporum ovale. The condition of her hands improved after she stopped handling and eating rice for 3 weeks. She began to wear rubber gloves whenever she washed rice, and thereafter never suffered from contact urticaria on the hands again. The severity of her atopic dermatitis remained unchanged, even after she started eating rice again. The principal food of the Japanese is hoiled rice. Before cooking it is vigorously hand-washed, and the water is changed several times. The results of the investigations performed in our patient suggest that water-soluble allergens were responsible for producing the contact urticaria. They were proteins which were heat-stable, but degradabic by trypsin digestion. The molecular weight was thought to be between 10 and 30 kDa, but it is possible that there were some antigens larger than 30 kDa.

The use of high-dose immunoglobulin in the treatment of pyoderma gangrenosum.

BACKGROUND: Immunosuppressive medications such as corticosteroids and cyclosporin are the most commonly employed therapies in pyoderma gangrenosum. We describe a patient with multiple ulcers of pyoderma gangrenosum on the lower extremities in whom immunosuppressive therapy caused serious side effects and had to be discontinued but who was subsequently treated successfully with high dose intravenous immunoglobulin (IVIG). METHODS: IVIG was given intravenously at a dose of 400 mg/kg per day for 5 consecutive days. After 1 week there was an arrest in the progression of the ulcers and a marked reduction in pain. Two weeks later clinical improvement of the ulcers was observed. Subsequently, IVIG was given at a dose of 1 g/kg per day for 2 consecutive days. RESULTS: The treatment induced a dramatic clinical improvement of one ulcer and healing of the others. Side effects were minimal and well tolerated, and consisted of chills and a slight fever, which resolved with the administration of acetaminophen. CONCLUSION: We feel that IVIG can be used in patients with pyoderma gangrenosum in whom conventional therapies are ineffective or produce serious side effects. ( J Dermatol Treat (2000) 12: 19-22)

Calcipotriol treatment of confluent and reticulated papillomatosis (Gougerot-Carteaud syndrome).

Gougerot–Carteaud syndrome or confluent and reticulated papillomatosis (CRP), was first described by Gougerot and Carteaud as dermatosis. 1,2 It is generally considered a rare condition. The eruption consists confluent, flat, brown papules localized primarily to the intermammary and interscapular regions with subsequent spread to the breast and abdomen; at the periphery, the papules spread out forming a pigmented reticulated pattern. At present, the aetiology of CRP remains unknown. The two prominent theories are an abnormal host response to fungi 3 and a keratinization defect. 4,5 Other hypothesis include photosensitivity, 6 genetic factor, 7 amylodosis cutis 8 and endocrinopathy. 9

Pyoderma vegetans and ulcerative colitis

Pyoderma vegetans (PV) is a chronic, vegetating pustular disorder characterized clinically by erythematous vesiculopustular vegetating cutaneous plaques. Marked epidermal hyperplasia, intraepidermal and subepidermal neutrophilic microabscesses and a dermal inflammatory infiltrate are the prominent histopathological findings. We describe a patient with PV associated with ulcerative colitis and mammary Paget’s disease. Pustular eruptions associated with ulcerative colitis are reviewed.

Efficacy evaluation of an oil-in-water emulsion (Dermoflan) in atopic dermatitis

Sir, Atopic dermatitis (AD) is a chronic pruritic disorder clinically characterized by erythematous, often scaling, macules, patches, papules and plaques that become licheni® ed, excoriated and crusted. The prevalence of AD has substantially increased during past decades, and has resulted in a signi® cant economic burden on the Health Service and on families of affected children (1). Diagnosis of AD is based on physical examination as well as family and patient history of allergic asthma, rhinitis and conjunctivitis (2). Although clinical manifestations vary with age, they may be present in any combination depending on a complex relationship among genetic, immunologic, pharmacologic and emotional factors. A variety of topical therapies are currently available for treatment of AD (3, 4), although corticosteroids are likely one of the most used therapeutic approaches. A short-term treatment and the use of low potency steroids, particularly in children, are usually recommended because of their well-known side effects. Emollients can be employed as ® rst-line treatment in mild AD either alone or as steroid-sparing agents, or as adjuncts to systemic therapy in severe AD (5 ± 7). The main function of emollients is to improve the skin barrier function, thus preventing irritant or allergic dermatitis. The objective of our study was to examine the ef® cacy of an oil-in-water (O/W) emollient (Dermo an1) in patients with AD, as assessed by evaluation of SCORAD index, hydration and pH of the skin.

Griscelli syndrome type 2: long-term follow-up after unrelated donor bone marrow transplantation.

Griscelli syndrome (GS) is a rare autosomal recessive disease characterized by silvery hair (‘partial albinism’). Three forms exist; GS type 2 (GS2), the most common one, is characterized by severe primary immunodeficiency with acute episodes of hemophagocytic lymphohistiocytosis (HLH) which may be fatal in the absence of hematopoietic stem cell transplantation. A 5-year-old boy with HLH was referred to us because of silvery-gray hair present since birth. Abnormal pigment clumps were observed in the medulla of hair shafts on light microscopy. Electron microscopy of a skin biopsy revealed melanosomes in melanocytes, but not in keratinocytes. Leukocytes were devoid of intracytoplasmic granules on blood smear. Neurological signs were absent. Genotyping revealed a homozygous haplotype for polymorphic markers linked to the RAB27A locus, but no RAB27A mutation. A diagnosis of GS2 was established. The patient received bone marrow transplantation (BMT) from an unrelated donor, and after 72 months he did not show relapse of HLH. The long, uneventful follow-up supports the use of BMT from an unrelated donor if transplantation from a relative is not possible.