Erythema nodosum.

Erythema nodosum is an acute inflammatory dermatosis characterized by painful nodules which are generally symmetrical and non ulcerative and are mainly located to the extensor surface of the lower legs. The nodules, due to septal panniculitis, are often accompanied by fever and resolve without permanent sequelae. Arthralgia occurs in more than 50% of patients and begins during the eruptive phase or precedes the eruption by 2-4 weeks. Erythema nodosum is presumed to be a hypersensitivity reaction and may occur in association with several systemic diseases or drug therapies, or it may be idiopathic. The most common cause of erythema nodosum is streptococcal infection in children and streptococcal infection and sarcoidosis in adults. Peak incidence occurs at age 18-34 years. Age and sex distributions vary according to etiology and race; women are affected more often than men. It is possible to distinguish between an acute and a chronic form of erythema nodosum; in the acute form, an early stage and a late stage can be detected, both clinically and histologically. Laboratory and instrumental examinations to be performed in case of erythema nodosum are varied and are intended to identify any underlying trigger disease. Erythema nodosum is a self-limited disease, so the therapy is often only symptomatic. Even if the erythema nodosum quickly responds to systemic steroids, in most cases their use is not recommended, nor necessary; is usually sufficient to use NSAIDs (eg, acetyl salicylic acid, ibuprofen, naproxen, indomethacin).

Diffuse skin spread of HIV-associated visceral leishmaniasis: cumbersome diagnostic and therapeutic issues.

An infrequent and atypical case report of HIV-associated visceral leishmaniasis complicated by a diffuse, aspecific maculo-papular cutaneous involvement was characterized by a prolonged course, and a lack of response to repeated attack/maintenance cycles performed with liposomal amphotericin B, despite a satisfactory immune response maintained thanks to a concurrent, potent combination antiretroviral treatment. Only a very prolonged administration of the older i.v. pentamidine isethionate together with oral paromomycin led to a slow, but complete cure of both visceral leishmaniasis and its related skin dissemination, in absence of adverse events and long-term disease relapses.

Eosinophilic pustular eruption associated with transdermal fentanyl.

A 70-year-old man developed a widespread eruption of sterile pustules on normal skin, mainly on the trunk, face and base of the limbs, 2 days after application of a fentanyl-transdermal therapeutic system (fentanyl-TTS) patch. The eruption was not accompanied by fever. The main histopathological finding was an intraepidermal pustule filled almost exclusively with eosinophils. Suspension of the fentanyl-TTS led to rapid and definitive spontaneous regression of the pustules in about 10 days. A similar pustular reaction related temporally to fentanyl-TTS administration had appeared several weeks before the current eruption.

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.

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 Down's 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.

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 persistent 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 disease 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.

[Perforating milia-like idiopathic calcinosis of the extremities in a patient with Down syndrome]. / Calcinose miliaire idiopathique perforante des extrêmités chez une malade trisomique 21.

INTRODUCTION: In 1989 a new type of calcinosis cutis has been described in association with Down's syndrome. This is the milia-like idiopathic calcinosis cutis, which is characterized by milia-like papulae generally located on the limbs (especially hands and feet) and sometimes associated with syringomas around lesions or on the eyelids. OBSERVATION: A 6 year old trisomic girl had about ten round shaped hard white-yellowish papules with a diameter of 2-3 mm on both palms of her hands. The biological balance and immunologic tests gave normal values. The histopathologic pattern was compatible with calcinosis cutis circumscripta associated with the transepidermal elimination phenomenon. Calcified sweat ducts were not observed at the von Kossa staining. Moreover, histology did not evidence any syringomas around the lesions. DISCUSSION: Our observation does not sustain the presently more spread pathogenetic interpretation, according to which eccrine ductal structures could have an active role in the formation of calcium deposits, since histology did not show any calcified eccrine ducts. Therefore, in our opinion, milia-like calcinosis associated with Down's syndrome should be classified among the idiopathic forms.

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 midreticular 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.

Genital porokeratosis of Mibelli.

Porokeratosis of Mibelli is a disorder of epidermal proliferation in which many different clinical forms can be distinguished. Two male patients with a localized type of porokeratosis limited to the genitalia are reported. Later in life they developed an annular skin lesion with peripheral keratotic ridge. The histological examination of a biopsy specimen showed the characteristic features of porokeratosis. There was no family history of similar skin disorders and the patients were not on any drugs. Genital porokeratosis is probably underdiagnosed and we believe that these patients should be followed up on account of the precancerous potential of this disease.

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.

Cutis verticis gyrata secondary to acute monoblastic leukemia.

A 64-year-old man affected by acute monoblastic leukemia developed a cutis verticis gyrata during the terminal phase of hemopathy. The association between these two diseases is rare. The classification of cutis verticis gyrata in primary essential, primary non-essential and secondary forms is reviewed. Performing a skin biopsy is necessary in the diagnostic approach to patients with cutis verticis gyrata.

Melanocytes in clear cell acanthoma.

The presence of melanocytes in 11 clear cell acanthoma cases was investigated by silver impregnation. Melanocytes were always present, their density showing great variability from case to case and within each individual neoplasm. The possible existence of a pigmented counterpart to clear cell acanthoma is discussed.

[The Lupus Band Test and serology of lupus erythematosus. Our experience]. / Lupus Band Test e sierologia nel lupus eritematoso. Nostra esperienza.

The Authors studied, from 1986 to 1988, 84 patients (24 men and 60 women, in an age group that went from 13 to 75 years, with an average of 42 years) in whom DLE was diagnosed. In these patients, a direct immunofluorescent assay was carried out on the affected skin, on the non-affected photoexposed skin, and on the non-affected non-photoexposed skin: furthermore, the serum of these patients was tested for the presence of antinuclear antibodies (ANA), antibodies to extractable nuclear antigens (anti-ENA), and antibodies to double-stranded DNA (anti-DNA). In the study, a significant correlation between the presence of ANA and entity of cutaneous involvement at the Lupus Band Test was found. Furthermore, it was noticed that anti-ENA and/or anti-nDNA tend to be present in association to a positive Lupus Band Test in all 3 biopsied tissues.

[Tungiasis. A clinical case]. / Tungiasi. Un caso clinico.

Tungiasis is a cutaneous infestation by the female sand flea, Tunga penetrans. Because of the increase in international travel, the disease is reported in Europe, in spite of it being formerly restricted to the equatorial zones. This report describes a case of tungiasis and discusses clinical features, diagnosis and treatment.

[Lichen striatus: a clinical and histological study of 8 cases]. / Lichen striatus: studio clinico ed istologico di 8 casi.

The authors describe eight patients with Lichen Striatus: history, clinical description and pathologic findings are discussed. Particularly, the histologic picture of Lichen Striatus is not uniform and changes, as it seems, with the "oldness" of the lesion.