Beatrice Passarini

Syringoma : A review of twenty-nine cases

The purpose of our study was to identify the clinical characteristics, epidemiologic data and histologic features in 29 cases of syringoma with a duration of lesions prior to the observation between 1 and 25 years. Only one patient complained of moderate itching. In two cases the lesion was solitary, in another the papules formed a lichenified plaque. In six patients only the eyelids were involved and in two patients a symmetrical localization on the forearms was observed. The other 18 patients showed generalized syringoma, 16 with an eruptive onset, 6 of which were familial. One of our cases showed lesions mimicking urticaria pigmentosa and two patients were affected by Downs syndrome. In two cases, histopathology showed association between syringoma and a melanocytic naevus and in one patient with a solitary lesion a clear cell syringoma was observed.

Systemic and discoid lupus erythematosus in HIV-infected patients treated with highly active antiretroviral therapy

Although HIV infection is often associated with several rheumatic diseases, the coexistence of this retroviral infection and systemic lupus erythematosus (SLE) is extremely uncommon. Generally, HIV-related immunosuppression improves SLE symptoms, and antiretroviral therapy may lead to an autoimmune disease flare subsequent to the increase of circulating CD4+ cell number. Two HIV-infected female patients with SLE and discoid lupus erythematosus (DLE) diagnosed a few months after the highly active antiretroviral therapy initiation, are described. To our knowledge, this is the second case of DLE and the twenty-seventh case of SLE reported to date in association with HIV infection.

Cutaneous manifestations in inflammatory bowel diseases: eight cases of psoriasis induced by anti-tumor-necrosis-factor antibody therapy.

Background: Ulcerous rectocolitis and Crohn’s disease are the best known forms of inflammatory bowel disease (IBD). Skin manifestations are not uncommon in IBD and may be divided into specific cutaneous signs, aspecific cutaneous signs, and cutaneous signs caused by drugs used for IBD therapy. The specific signs (fistulas, rhagades and ulcers) are the result of the diffusion of the intestinal inflammatory process into the skin. Aspecific cutaneous signs (stomatic aphthosis, erythema nodosum, pyoderma gangrenosum, Sweet’s syndrome, vasculitis, bullous diseases) are quite frequently found in those suffering from IBD, but also in apparently healthy subjects, and may sometimes be the first sign of the intestinal disease. Cutaneous manifestations due to drugs vary in clinical aspect and are the direct consequence of the therapies adopted, which in IBD patients can be quite numerous: steroids, immunosuppressants, 5-aminosalicylic acid, biological agents, antibiotics. Objective and Methods:Due to the frequent finding of cutaneous manifestations in patients affected by IBD, a collaboration was set up between the Dermatological Clinic of the University of Bologna and the Center for the Study of IBD of the same university hospital. The aim was to diagnose the cutaneous signs appearing during IBD and to establish their etiopathogenesis in order to assess whether they were the result of epiphenomena of the IBD or side effects of the therapies adopted. Results: The cutaneous manifestations we observed can be divided into three distinct groups: signs that were specific to the basic disease, aspecific signs and finally signs attributable to the drugs used for therapy. Particular attention was given to the aspecific signs and those consequential to therapy. The aspecific cutaneous signs seen in our clinic generally reflect those reported in the literature. The cutaneous manifestations due to drugs were further divided into three groups: rosacea, acneiform dermatitis and psoriasis-like dermatitis. The most notable aspect of our series is the high number of patients presenting psoriasiform-type dermatitides with a generally widespread diffusion. Conclusion: We would like to draw attention to the fact that all patients with psoriasis had been undergoing treatment with drugs inhibiting tumor necrosis factor α (TNF-α) as part of IBD therapy. In all cases, the cutaneous reaction started after the third or fourth infusion of the biological drug. Anti-TNF-α agents have also been successfully used to treat psoriasis in the last few years. The reason for this apparently paradoxical effect of the therapy is still unclear.

Chloracne: still cause for concern.

Chloracne, first described by Herxheimer in 1899, is a dermatosis consisting of more or less diffuse acneiform lesions distributed prevalently on the face and on body areas not usually affected by acne and caused by chronic or acute exposure to halogenated chemical compounds. Dioxin is the common name for dibenzo-p-dioxins and dibenzofurans, contaminants nearly ubiquitous in the environment and highly resistant to chemical and biological degradation. These compounds can survive for decades in the environment and accumulate in the human and animal food chains. Chloracne is characterized by the onset of numerous comedo-like lesions and yellowish cysts on the face, particularly on the cheeks, that can spread to the trunk and other body regions not usually affected by acne vulgaris, with diffuse grayish skin pigmentation and sometimes associated with hypertrichosis and areas of folliculitis. The lesions may occasionally be accompanied by skin or systemic manifestations. We report 9 cases of chloracne, 8 of them with rapid onset in patients residing in the same building, and 1 in a patient occupationally exposed to halogenated compounds. In our series, the doses of dioxin and polychlorinated biphenyls in the soil, water and plant material, and the serum titer of dioxin were within the normal range. This consideration raises the issue of the need to revise the serum threshold for dioxin poisoning and the environmental threshold. We wish also to underline the value of dermatopathology in the differential diagnosis of chloracne.

Neutrophilic Figurate Erythema of Infancy

Abstract:  We report here a new case of neutrophilic figurate erythema of infancy in a 1‐year and 9‐month‐old white boy. Neutrophilic figurate erythema of infancy is a rare inflammatory dermatosis which is part of the figurate inflammatory dermatoses of infancy and is considered a variant of annular erythema of infancy. The disease is clinically characterized by annular erythematous lesions, sometimes with a polycyclic configuration, and histologically by a dermal neutrophilic infiltration with leukocytoclasia. Differential diagnosis mainly includes clear‐cut severe diseases with a well‐known etiology, such as neonatal lupus erythematosus and its variant erythema gyratum atrophicans transiens neonatale, erythema chronicum migrans and erythema marginatum rheumaticum, and diseases of unknown origin and with less clear limits, such as erythema annulare centrifugum and its variant familial annular erythema. Anamnesis, laboratory findings, clinical features, and histology allow the correct diagnosis and therefore, having excluded severe diseases, parents should be reassured as neutrophilic figurate erythema of infancy, as the annular erythema of infancy, is a benign disease, in most cases unassociated with other conditions and usually self‐limiting over a few months, even though a chronic course may rarely occur. Our case was characterized by a chronic persistent course and by a complete resolution of the lesions only during febrile episodes.

Persistent Milia, Steatocystoma multiplex and Eruptive Vellus Hair Cysts: Variable Expression of Multiple Pilosebaceous Cysts within an Affected Family

Background: Steatocystoma multiplex (SM) and eruptive vellus hair cysts (EVHC) are conditions characterized by numerous cutaneous cysts with a clinical resemblance, such as age of onset, location, appearance of the lesions and mode of inheritance, but with distinctive histologic features. Recently, some authors have proposed that the two conditions are variants of one entity originating in the pilosebaceous duct, while others suggest that SM and EVHC are two distinct entities, on the basis of the different expression of keratins. Milia are small round cysts, commonly involving the face. In 1994, Menni and Piccinno reported the first association, in a family, between persistent infantile milia in a 9-month-old girl and SM in the father. The authors hypothesized a relationship between these two conditions. Objective: We describe a family in which the mother showed cystic lesions and milia and her 4-year-old son and her 18-month-old daughter presented persistant infantile milia. Methods: In addition to the clinical and genetic documentation, a biopsy was performed in the son and two biopsies in the mother. Results: The histologic examination of one of the son’s milium-like papules showed in the superficial dermis a small cyst typical of a milium. In serial sections it was possible to see a connection with a vellus hair follicle by an epithelial pedicle. The histologic examination of one of the mother’s papules on the forehead showed a large round cystic structure with histologic features of EVHC. Close to the cyst wall, serial sections showed flattened sebaceous gland lobules. The histologic findings of the second biopsy taken from the chest showed the typical features of a steatocystoma. Conclusions: The relationship between milia, SM and EVHC is discussed; the three diseases should be considered as subtypes of multiple pilosebaceous cysts that may all present overlapping histologic features. The different level of the pilosebaceous duct where the cyst originates should explain the different clinical manifestations.

Mid-dermal elastolysis: a pathological and ultrastructural study of five cases

The aim of this study was to evaluate the presence of inflammatory phenomena and elastic fiber phagocytosis in mid‐dermal elastolysis. The pathological and ultrastructural features of 5 Caucasian female patients (ranging from 26 to 40 years) with acquired diffuse asymptomatic areas of skin wrinkling have been reviewed. The clinical features of all cases were characteristic of this condition and only in one patient were erythematous urticaria‐like, non pruriginous patches also observed. In 4 cases a history of prolonged sun bathing was present and in 3 cases there was a short history of oral contraception. The pathological study confirmed the typical absence of elastic fibers in the mid‐reticular dermis. In two cases elastic fibers were still detectable in the periadnexal dermis. Hematoxylin and eosin sections showed a mild perivascular infiltrate in two cases, while in three patients histiocytes were scattered among collagen bundles. Multinucleated giant cells containing fragmented elastic fibers were detectable in one patient. Ultrastructural analysis revealed large mononuclear cells with phagocytic aspects toward elastic fibers in all cases.

Keratosis lichenoides chronica: A Pediatric Case

Keratosis lichenoides chronica (KLC) is a rare chronic disorder of keratinization characterized by lichenoid hyperkeratotic papules arranged in a linear pattern, erythematosquamous plaques and seborrhea-like dermatitis on the face. Adults between 20 and 50 years of age are usually affected, but the disease is very uncommon in childhood. Our purpose was to study the clinical and histopathologic findings and course of KLC in one pediatric case. Detailed clinical data were studied. Two punch biopsies were performed and histopathologic features were compared with those of other reported cases of KLC. In our patient, a 4-year-old boy, the clinical features of the lesions did not deviate notably from those of other cases of KLC. The histologic pattern of the papules was typical of KLC, while that of the erythematosquamous plaques showed some dyskeratotic keratinocytes. The histologic pattern of the erythematosquamous lesions is peculiar in our case, whereas only a nonspecific pattern is reported in the literature. The papular and erythematosquamous lesions showed similar histopathologic features suggesting that they could be different degrees of evolution of the same lesion.

Porokeratosis palmaris et plantaris disseminata: An unusual clinical presentation

A 54-year-old man and his son had porokeratoses of the trunk and extremities, predominantly on the palms and soles. Numerous small, slightly depressed, opalescent rings with hyperemic borders were present on the oral mucosa. The spectrum of porokeratosis palmaris et plantaris disseminata and the differential diagnosis of other types of porokeratosis are discussed.

Porokeratosis in the elderly: a new subtype of disseminated superficial actinic porokeratosis.

In a review of all cases of porokeratosis histologically diagnosed in our Department during the period 1991-98 we found that 12 patients (22%) were in their seventh to ninth decade. In all 12 (2 males and 10 females) the age of onset of the disease varied between 58 and 89 years (mean age 68.6 years). The clinical picture was similar in all the patients, with the number of lesions varying from a few to 20-50 annular plaques 10-15 mm in diameter, localized mainly on the lower limbs. We suggest that our patients had a very mild form of disseminated superficial actinic porokeratosis confined to the extremities with an unusually late onset. This peculiar variety of late-onset disseminated superficial actinic porokeratosis may represent a type of immunosuppression-induced porokeratosis where the pathologic clone for porokeratosis is present but remains latent until the amount of sun exposure, together with the physiological age-related lowering of immunocompetence, bring about its proliferation.