Gabriella Pravatà

Proliferating Tricholemmal Cyst Should Always Be Considered as a Low-Grade Carcinoma

Proliferating tricholemmal cyst Malignancy Carcinoma Prof. M. Aricò, Department of Dermatology, University of Palermo, Policlinico ‘P. Giaccone’, via del Vespro 131, I-90143 Palermo (Italy), Fax +39 91 6554021, E-Mail istderm@mbox.unipa.it Proliferating tricholemmal cyst (PTC) is an uncommon append-ageal skin neoplasm undergoing outer root sheath differentiation, showing a compact, abrupt tricholemmal keratinization without granular layer interposition. PTC shares this kind of keratinization with the tricholemmal cyst, with the outer root sheath at the level of the isthmus where the inner root sheath disappears, and with an infundibular sac in catagen and telogen hairs [1]. PTC may also present matrical structures with a pattern similar to that detectable in squamous carcinomas with pilar differentiation [2, 3]. Clinically, PTC usually appears as a

Multiple nevoid spiradenomas.

This report delineates a new clinical presentation of multiple benign spiradenoma (BS), a diffuse linear nevoid pattern. A 16-year-old girl, in otherwise good health, had many papulonodular lesions, varying in color from blue-gray to bright red, disposed linearly on the right half of her body, involving the periorbital region, cheek, lateral side of the neck, trunk, linea alba, right thigh, and leg. Only a few nodules were slightly painful. Lesions were reported to be present at birth; no family members had similar lesions, either in solitary or multiple form. Excisional biopsies of three lesions from the neck, abdomen, and leg, respectively, were performed; histologically, all three samples showed a picture of BS. In some areas, the tumor was directly connected to a pilosebaceous unit. Furthermore, a focal area with pilar differentiation at the periphery of the lobules was observed, which might suggest that BS can differentiate toward various cutaneous adnexal structures. Our patient probably is the first one reported with diffuse linear nevoid lesions of BS, present at birth and involving the right half of the body.

Localized crusted scabies in the acquired immunodeficiency syndrome

Crusted scabies (CrS) is an uncommon occurrence among patients with AIDS. Indeed to date only five cases have been described, all with widespread lesions. A case of localized CrS appearing as a yellowish and crusted plaque on the second right toe is reported in a woman with AIDS. Scraping off the verrucous surface, as well as punch‐biopsy revealed many mites within the horny layer. The infestation is related to the cutaneous immune response and thus CrS should be considered an opportunistic infestation in AIDS. The importance of the early diagnosis of CrS in order to prevent disseminated lesions and involvement of other health‐care workers is underlined.

Transfusion-associated graft-versus-host disease : report of two further cases with an immunohistochemical analysis

Transfusion‐associated graft‐vs.‐host disease (tGVHD) is a severe disease usually affecting immunocompromised hosts with haematological neoplasia. Two patients with acute leukaemia are reported, who developed fatal tGVHD after blood transfusions. Intercellular adhesion molecule 1 (ICAM‐1), vascular cell adhesion molecule 1 (VCAM‐1) and endothelial leucocyte adhesion molecule 1 (ELAM‐1) expression and the CD4/CD8 ratio were assessed in lesional skin. ICAM‐1 was strongly expressed on epidermal keratinocytes and endothelial cells (EC) and correlated with HLA‐DR staining. VCAM‐1 was strongly expressed on EC in the superficial dermal vessels. ELAM‐1 stained weakly on EC in some of the superficial vessels. CD8+ lymphocytes showed prominent epidermotropism; the CD4/CD8 ratio was 0.8 in case 1 and 1.2 in case 2. Infiltrating cells were positive for CD3, CD11a, and CD18. Langcrhans1 cells were almost completely absent. The dermatologist must be aware of the importance of such a rare, unexpected and almost always fatal complication of blood transfusion, in order to make an early diagnosis. Irradiation of blood products is the only effective way to prevent tGVHD in all subjects at risk.

Reticulate postinflammatory hyperpigmentation with band-like mucin deposition.

Background Mucinoses of the skin are a group of disorders sharing accumulation of mucin in the skin or hair follicles. Postinflammatory hyperpigmentation, with pigmentary incontinence, is due to loss of melanin from epidermal basal cells and its accumulation in dermal macrophages.

Increased SS bonds in chronic solar elastosis: a study with N-(7-dimethylamino-4-methyl-3-coumarinyl) maleimide (DACM) stain

We have studied the distribution of SH groups and SS linkages in solar elastosis, in comparison with that in aged and juvenile sun-unexposed skin, using N-(7-dimethylamino-4-methyl-3-coumarinyl) maleimide (DACM) stain. In heavy solar elastosis we found increased fluorescence for SS bonds in the upper and middle reticular dermis in the elastotic masses; these were separated from the epidermis by a network of actinically-damaged, SS-positive elastic fibers, showing irregularly and variously interwoven oxytalan and elaunic fibers. These latter, unlike the elastotic masses, revealed also SH groups. In moderate and weak elastosis, where the thick irregular masses were absent, the fluorescence for SH and SS linkages was present in equal proportions in the altered elastic fibers; collagen bundles revealed only fluorescence for SS bonds. Aged and juvenile sun-unexposed skin showed the same proportions of SH and SS linkages in the changed elastic fibers. Collagen bundles of aged and juvenile skin showed fluorescence for SS bonds; SH groups were present on a few collagen fibers only in aged skin. Our results are consistent with opinion supporting an elastic origin of elastotic degeneration, which could be the consequence of a defect, due to chronic solar exposure, in one of the steps--degradation or synthesis--of metabolic turnover of elastic fibers, resulting in abnormal and excessive polypeptide chains extremely rich in disulfide bonds.

Lack of Antiandrogenic Effects of Topical Bifonazole on Sebaceous Glands and Hairs in the Hamster Flank Organ

The activity of topically applied bifonazole was evaluated in vivo in the three androgen-dependent structures of the hamster flank organ, i.e. the pigmented spot, sebaceous glands and hairs. Topical bifonazole in our experience did not demonstrate any morphological effect on sebaceous gland and hair even when applied in the dosage of 3 mg/cm2/day. On the basis of our morphometric results we can conclude that topically applied bifonazole does not interfere with cutaneous androgen metabolic transformations in the pilosebaceous unit of the flank organ.

Morphea-like cutaneous mucinosis

Abstract A whitish, indurated, oval plaque, lasting for 1.5 year, was observed in the right paraumbilical skin of a middle-aged man in otherwise good health. No erythema was present or referred. Histological examination revealed massive deposits of a globular, slightly basophilic, amorphous substance in the dermis, accompanied by a slight perivascular mononuclear cell infiltrate and scattered mast cells. Special staining with Alcian blue at pH 2.5 confirmed the intense deposition of mucin involving the entire dermis, sparing epidermis, adnexa and perivascular areas. No abnormal laboratory results indicating lupus erythematosus or thyroid dysfunction were present. Morphea was excluded on clinical and histological grounds. A plaque-like form of cutaneous mucinosis was diagnosed. Plaque-like mucinosis and reticular erythematous mucinosis (REM) syndrome are currently considered to be the same disease, although some authors noted a different response to treatment. Clinical presentation of our case was unusual: no erythema preceded or accompanied the plaque growth, which had a whitish, indurated, morphea-like appearance from the beginning but with preservation of hair follicles, quite different from archetypical REM syndrome based on clinical grounds.

Combined treatment of epidermodysplasia verruciformis with etretinate and α-interferon*

Epidermodysplasia verruciformis (EV) is an uncommon cutaneous disease in which a focal and genetically determined immunological impairment is associated with chronic human papilloma virus (HPV) infection. In sun‐exposed areas, when an oncogenic HPV type is the agent, skin cancer may occur. The treatment of EV is difficult and often unsatisfactory; etretinate has been reported in some instances as effective in improving lesions. We report a typical case of EV with pityriasis versicolor‐like lesions on the trunk and many flat, erythematous wart‐like lesions on the face, dorsal areas of the hands and legs. We performed a treatment with etretinate (1 mg/kg/day for 6 weeks) and subsequently with etretinate and α2r‐interferon (3 MU, 3 times per week for 2 weeks, then 6 MU, as above, for 4 weeks). After 4 weeks of therapy with etretinate we observed moderate improvement; we did not observe any further clinical improvement in the final 2 weeks. The subsequent combined treatment with etretinate and a‐interferon achieved further improvement. We conclude that the association of etretinate and a‐interferon may represent an efficacious treatment of EV.