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Biomedical subjects

A Patrizi

Publications and source records attributed to A Patrizi.

At least 55 records · Page 3Linked to original sources

Loose anagen hair.

OBJECTIVE: To review clinical and pathologic features and the long-term follow-up of patients with loose anagen hair (LAH). DESIGN: Clinical evaluation and long-term follow-up. SETTING: A university medical center. PATIENTS: Beginning in January 1990, 14 children and 5 adults (age range, 8 months to 47 years) were diagnosed as having LAH. Associated diseases included alopecia areata in a 3-year-old boy and Noonan syndrome in a 5-year-old boy. Two adult patients were parents of 2 affected children; the other 3 adults were the only members of their families with LAH. These 3 patients presented with a diffuse hair shedding that had suddenly developed 1 to 3 years before our observation. In all cases, findings of a trichogram showed a predominance of anagen hairs (80% to 100%) devoid of sheaths. INTERVENTION: None. RESULTS: In 4 children and 1 adult the condition remained stable; in 2 children and 1 adult, a considerable improvement in hair density was noticed. The pathologic study of hair from 5 patients did not reveal morphological abnormalities of the hair follicles except for a high incidence of fragmentations of the inner root sheath. CONCLUSIONS: Analysis of our patients with LAH reveals that the condition does not develop exclusively during childhood but can occasionally manifest itself later in life. The development of LAH may be sporadic, occur in association with developmental or acquired conditions, or, less commonly, be a familial disorder. While adult-onset LAH may not be exceptional, it can be easily misdiagnosed as telogen hair loss. The pathologic findings of LAH do not demonstrate any specific features and are of little value in the diagnosis of this condition.

Adult↗

[Perforating milia-like idiopathic calcinosis of the extremities in a patient with Down syndrome].

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.

Calcinosis↗

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.

Adult↗

Pigmented and hyperkeratotic napkin dermatitis: a liquid detergent irritant dermatitis.

BACKGROUND: Napkin or diaper dermatitis (DD) is an inflammatory cutaneous eruption limited to the diaper area and common in the first 2 years of life. A number of clinical variants of DD have been identified. OBJECTIVE: We report a new variant of DD characterized by papyraceous skin, brownish discoloration and predilection for the depth of folds. METHODS: 15 infants and toddlers affected by this peculiar type of DD were evaluated regarding duration, localization, morphology and evolution of their dermatosis. RESULTS: This variant of DD was mainly confined to the depth of inguinal and gluteal folds and invariably associated with severe xerosis with papyraceous and glazed skin. The patients were healthy and asymptomatic and all laboratory investigations performed were normal. All patients were frequently changed and thoroughly washed with synthetic detergents with acid pH. DD improved rapidly with reduced frequency of washing and discontinuation of liquid detergents. CONCLUSIONS: We conclude that this condition is a type of irritant contact dermatitis from excessive use of lipid acid detergents.

Dermatitis, Irritant↗

Sacral medial telangiectatic vascular nevus: a study of 43 children.

BACKGROUND: Medial telangiectatic vascular nevi are capillary vascular malformations frequently observed at birth occurring mostly on the face or on the nape as a single lesion or as multiple macules affecting more than one site simultaneously. In 1990, Metzker and Shamir reported a medial telangiectatic vascular nevus (MTVN) in the sacral region along the midline and called this particular variety of MTVN 'butterfly-shaped mark'. OBJECTIVE: Our study was performed to investigate the morphology and localization of an MTVN in the sacral region (sMTVN) in a group of Caucasian children. METHODS: We observed 43 children with sMTVN, ranging in age from 1 month to 12 years (mean 8 years), for a period of 6 years. RESULTS: sMTVN was found as a red-violet macular lesion of rhomboid or triangular shape in 16 patients and as a group of little red-violet macules in 10 patients. In 16 patients moreover the whole back was involved with many small red-violet spots on and around the spinal column, and in 1 patient with classic sMTVN satellite macules were also present over both buttocks. Four patients suffered from epilepsy with mental deficiency. In 6 patients, the family history showed sMTVN in one or more members of the family. No case presented an association with spina bifida. CONCLUSION: In our study, sMTVN shows a morphological polymorphism while Metzker and Shamir reported the same clinical aspect in all 25 of their patients. In accordance with these authors, we noted that sMTVN persist into childhood and adult life in the same way as occipital MTVN.

Buttocks↗

Perianal streptococcal dermatitis in adults.

Perianal streptococcal dermatitis is an uncommon superficial cutaneous infection of the perianal area almost exclusively described in children. We report here four adult cases. Beta-haemolytic streptococcus group A was detected in the perianal areas of all the patients. Systemic erythromycin gave complete resolution. The incidence of perianal streptococcal dermatitis in adults is probably underestimated, and culture from the affected area should always be performed in patients with persistent perianal erythema.

Adult↗

Vulvitis plasmacellularis: two new cases.

Vulvitis chronica plasmacellularis or Zoon's vulvitis is a rare benign circumscribed inflammation of the vulvar mucosa. It is found in women ranging in age from 26 to 70 years. Shiny, macular erythematous lesions, which are irregular in shape and sharply marginated are usually observed. The histologic findings show chronic subepithelial dense inflammation composed largely of plasma cells. We here report two cases of vulvitis plasmacellularis with typical clinical manifestations, courses and histopathologic findings.

Adrenal Cortex Hormones↗

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.

Adult↗

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.

Child, Preschool↗

Epidermolysis bullosa simplex associated with muscular dystrophy: a new case.

We report an infant with a rare form of epidermolysis bullosa simplex characterized by an autosomal recessive pattern of inheritance, severe cutaneous involvement, oral and nail lesions, associated with muscular dystrophy, and a poor prognosis, due to extracutaneous disease. In addition to the usual presentation of this disease, our patient had severe anemia, with immature circulating white cells, and bone marrow histology suggestive of a pre-leukemic state, a finding which has not before been reported in the literature.

Anemia↗

Perianal streptococcal dermatitis associated with guttate psoriasis and/or balanoposthitis: a study of five cases.

Perianal streptococcal dermatitis (PSD) is a recently described cutaneous entity caused by group A beta-hemolytic streptococci. It is characterized by perianal erythema, sometimes associated with functional disturbances. We describe four children (2 boys, 2 girls) who had acute guttate psoriasis and also PSD. One of these patients also had balanoposthitis. A fifth patient experienced an association of PSD and balanoposthitis without psoriasis. To our knowledge, the association between guttate psoriasis and PSD has only been reported in five children, and the one with balanitis has not been previously reported.

Anal Canal↗

Trichomicosis pubis: black variety.

A case of a 25 year old man with the black variety of trichomicosis pubis is presented on account of its extreme rareity. Scanning electron microscopy confirms that trichomicosis pubis is caused by bacterial colonisation of the pubic hair and shows that bacteria are able to penetrate cuticular horny cells directly through their free plasma membrane.

Adult↗

Pseudoxanthoma-elasticum-like papillary dermal elastolysis: another case.

Pseudoxanthoma-elasticum-like papillary dermal elastolysis is a rare acquired idiopathic non-inflammatory elastolytic disorder. This disease, described in aged females, is characterized by asymptomatic skin lesions of the neck and supraclavicular regions clinically resembling pseudoxanthoma elasticum. Histological and ultrastructural examinations show a total loss of the elastic fibres in the papillary dermis. We report another case of this entity in a 69-year-old Italian woman.

Aged↗

Dyschromatosis symmetrica hereditaria associated with idiopathic torsion dystonia. A case report.

The authors report a case of a family in which a diagnosis of dyschromatosis symmetrica hereditaria was established by a clinical pattern of cutaneous lesions and by assessment of cellular DNA repair synthesis. The skin lesions were characterized by a mixture of hyperpigmented and hypopigmented macules and were localized on the back of the feet of three patients (two male brothers and one sister). All the patients also had small freckle-like pigmented macules on their face. The father presented large symmetrical hypopigmented vitiligo-like macules. In this patient, mottled pigmentation and depigmentation of the extremities had been present since childhood. In the four patients no cellular abnormalities in DNA repair ability were found. These data exclude a mild form of xeroderma pigmentosum. The daughter, a 9-year-old girl, had since the age of 7 also shown a neurological disorder diagnosed as idiopathic torsion dystonia. The authors emphasize the association of dyschromatosis symmetrica hereditaria, a rare cutaneous disease, with idiopathic torsion dystonia, a rare idiopathic neurological disease, in this young girl.

Adolescent↗