HPV5b variant in a neoplastic lesion of an Italian patient affected by epidermodysplasia verruciformis.

Epidermodysplasia verruciformis (EV) can be defined as a genetic disorder that determines a lifelong infection of the skin by human Papillomaviruses (HPV). The benign lesions contain different HPVs; whereas in the tumors HPV types 5, 8, and, much less frequently, types 14, 17, 20 and 47 can be detected. Variants of HPV5 have been recognized on the basis of the genetic heterogeneity of the E6 open reading frame. We report a typical case of EV in which the presence and expression of the HPV type 5 were clearly detectable. Direct sequence analysis demonstrated a perfect homology with the sequence of the HPV5b variant. This variant was first isolated in a Japanese patient and thereafter in a Polish one. Its presence in the Italian patient indicates that the same variant can be detected in different geographic areas and therefore that some cellular genes may exert a selection pressure so strong as to induce the emergence of this stable HPV5b infectious variant.

Papuloverrucous colloid milium: an occupational variant.

We report a case of adult colloid milium in a 47-year-old mechanic with a long history of professional contact with lubricating oils and of sun exposure. In addition to the typical translucent papules seen on the forehead, there were warty papules on the backs of both hands with unusual histological and ultrastructural features: marked hyperplasia of the epidermis with orthokeratotic hyperkeratosis and papillary deposits of colloid material that were contiguous with the basal layer of the epidermis. The hand lesions caused by occupational exposure to mineral oils and solar radiation represent an occupational variant of adult colloid milium.

Disseminated superficial porokeratosis with dermal amyloid deposits: case report and immunohistochemical study of amyloid.

The association of porokeratosis with dermal amyloid deposits is extremely rare, only three cases are reported in the literature. We describe a case of disseminated superficial porokeratosis (DSP) with clear histologic evidence of amyloid deposition in the upper dermis. The amyloid was typed with an original immunohistochemical assay based on three anticytokeratin antibodies (MNF 116, CK1, KER B). The epidermal origin of the substance (K amyloid) was demonstrated by its strong positivity for MNF 116 and KER B.

Fibroelastolytic papulosis of the neck: a report of 20 cases.

The clinical and histological features of the entities known as 'white fibrous papulosis of the neck' (WFPN) and 'acquired elastolysis of the papillary dermis simulating pseudoxanthoma elasticum' (PDE) are not clearly defined. This study was conducted to compare our experience of WFPN/PDE with those described in the literature. Twenty patients presented at our institution with papular eruptions involving the neck. The asymptomatic lesions, which ranged in colour from normal skin tones to yellowish, were isolated or coalescent. Microscopically, the papules showed elastolysis and fibrosis of the upper reticular and papillary dermis. A review of the literature shows similar characteristics in cases reported as WFPN and PDE. This study indicates that WFPN and PDE are variants of a single disorder that can be more precisely defined as 'fibroelastolytic papulosis of the neck' and which appears to be a manifestation of intrinsic skin ageing.

Granulomatous slack skin. Report of a case and review of the literature.

We describe a patient with granulomatous slack skin (GSS) who has been followed for 15 years and present clinical, histological, immunohistochemical, ultrastructural, cytogenetic, and molecular findings. The clinical and pathological aspects of the 20 cases of GSS reported in the recent literature are reviewed and compared with those of the present case.

Macroscopic and microscopic characteristics of an African Blastomyces dermatitidis strain.

This report describes the macro- and microscopic characteristics of a strain of Blastomyces dermatitidis isolated in an Italian hospital from a Libyan patient suffering from cutaneous blastomycosis. The fungus was isolated with great difficulty because of the presence of a Proteus species on the lesion. Conversion of the mycelial into the yeast-like phase achieved the best results, using Columbia ANC culture medium. After 5-6 days, this transformation was only partial and presented swollen hyphae and yeast-like cells. The biological characteristics were typical of B. dermatitidis strains of African origin. The differences between African and North American strains are confirmed, in agreement with findings already reported in the literature.

[Weary hereditary sclerosing poikiloderma]. / Poïkilodermie sclérosante héréditaire de Weary.

INTRODUCTION: Hereditary sclerosing poikiloderma is a genodermatosis with dominant autosomal transmission and variable penetration. The first case was described by Weary in 1969 in 7 members of two black families. CASE REPORT: A 10-year-old girl had localized regional poikiloderma of the fingers and club toes. These lesions were associated secondarily with linear symmetric bands of sclerotic tissue in the axiallary regions. On the X-ray examinations of the distal phalanges of the fingers and the toes showed a proximal growth foyer and absent ungueal phalanges, excepting in the fourth finger of the left hand. Capillaroscopy of the supra-ungueal fold of the fingers showed abnormal capillary circulation. Histology and ultrastructural examinations did not reveal any pathognomonic alterations. DISCUSSION: This case is the first reported in a white patient. The radiological aspect and the results of the capillaroscopy of the fingers and the toes have not been reported previously in this rare genodermatosis. Inheritance of this genodermatosis is poorly defined.

Granulomatous slack skin: cytogenetic and molecular analyses.

Granulomatous slack skin (GSS) is a rare disorder which is considered a slowly evolving T-cell lymphoma associated with granulomatous inflammation that mediates clastolysis. A combined cytogenetic, molecular, and cellular analysis was conducted on a clinically and histologically defined case of GSS. Cell cultures obtained from the skin biopsy showed trisomy of chromosome 8, and the DNA sample extracted from the skin biopsy showed a T-cell receptor beta-chain rearrangement.

Folliculosebaceous cystic hamartoma. Reported case with a neural component.

The clinical and histological features of a recently recognized entity, folliculosebaceous cystic hamartoma, are reported. The peculiarity of our case is a previously unreported neural proliferation in the stroma.

[Dowling-Degos disease and Verneuil disease]. / Maladie de Dowling-Degos et maladie de Verneuil.

In 8 out of a series of 21 cases Dowling-Degos disease was associated with Verneuil's disease (chronic hidradenitis suppurativa). This association might be more common, since both diseases are characterized by a single defect (follicular occlusion) and occur in similar cutaneous areas.

Penile lentiginosis. An ultrastructural study.

This study on five patients has revealed more extensive alterations to melanocytes than previously reported, and emphasizes the fact that depigmentation is an essential element of the condition. In hyperpigmented areas, melanocytes were increased in number along the basal layer of the epithelium, were hyperactive, and in some cases contained bizarre melanosomes. In two cases there was suggestion of a defect in melanosome transfer to keratinocytes. Lymphocytes were closely apposed to melanocytes, and, in hypopigmented areas, were clearly involved in their disintegration. In depigmented areas, there was complete absence of melanocytes and of melanosomes in keratinocytes, and lymphocytes were present in the basal layer. In general, the appearances did not resemble melanoma in situ with spontaneous regression, although a second biopsy of one patient after one year did reveal features of melanocytes suggestive of an early stage of this condition. The study has provided no clear information as to the initial cause of the condition, but the manner of destruction of melanocytes suggests an immune reaction. Neither has it been of assistance in suggesting a more precise name for it.

[Congenital smooth muscle hamartoma. Clinical-histological considerations (3 cases)]. / Amartoma muscolare liscio congenito. Considerazioni clinico-istologiche (3 casi).

The Authors report 3 cases of "Congenital Smooth Muscle Hamartoma" (CSMH). CSMH presents as congenital patches or slightly indurated plaques with prominent overlying hair, often hyperpigmented. Histopathologic examination showed increased numbers of well-defined smooth muscle bundles in the superficial and deep dermis. CSMH and Becker's nevus should be considered in the differential diagnosis of any congenital hairy hamartoma.

[Perforating pilomatrixoma in adults]. / Pilomatricoma perforante dell'adulto.

Two rare cases of perforating pilomatrixoma similar to those reported in the literature are described. Clinical examination showed in two old-women a reddish inflammatory exophytic tumor, 1 cm in diameter, with central erosive surface, with a rapid growth (3-5 months). Histologic examination showed: multiple masses of basophilic cells situated in the upper dermis making contact with the epidermis in a "follicle-like" opening; occurrence of transepithelial elimination phenomena.

Eccrine angiomatous hamartoma: a lipomatous variant.

The eccrine angiomatous hamartoma is a rare cutaneous lesion histologically characterized by the presence in the intradermal lobules of mature eccrine sweat glands and angiomatous capillary channels. We report a case of eccrine angiomatous hamartoma with unusual lipomatous involvement.