Biomedical subjects
Publications and source records attributed to A M Peluso.
The term circumscribed storiform collagenoma was coined by Maize et al in 1989 to describe four dermal fibromas with pathologic features resembling the sclerotic fibromas associated with Cowden's disease. We report a subungual nodule with the pathologic features of circumscribed storiform collagenoma in a patient without Cowden's disease.
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BACKGROUND: Nail involvement in lichen striatus (LS) is uncommon and has always been reported in association with typical skin lesions. OBJECTIVE: We attempted to characterize the clinical and pathologic features and the long-term prognosis of nail LS. METHODS: Five cases of LS of the nail including three cases with exclusive nail involvement were evaluated and the literature reviewed. RESULTS: Biopsy specimens showed a moderately dense bandlike lymphohistiocytic infiltrate affecting the proximal nailfold, the nail bed, and the nail matrix dermis. Exocytosis with slight spongiosis, focal hypergranulosis, and dyskeratotic cells were detectable in the nail matrix epithelium. Spontaneous regression of the onychodystrophy occurred after 4 to 12 months from the time of diagnosis (mean, 8.4 months). CONCLUSION: Nail LS is not necessarily associated with skin lesions but can also be an isolated finding. The diagnosis of nail LS should be strongly suspected when a child or a young patient presents with lichen planus-like nail abnormalities localized to the lateral or medial portion of a single nail.
Contact stomatitis is rather uncommon because of the relative resistance of the oral mucosa to irritant agents and allergens. The clinical manifestations of contact stomatitis are extremely variable and include erythema, erosions, ulcerations, leukoplakia-like lesions, and lichenoid reactions. Clinical signs are frequently less pronounced than subjective symptoms, and patients commonly experience severe functional problems despite only mild mucosal alterations. Allergic stomatitis is rare and almost always attributable to metallic mercury and gold salts. A careful history and an accurate examination of the oral cavity, teeth, and dental restorations are essential for a correct diagnosis. Patch testing is indicated in all lesions that are not clearly related to trauma or physical injuries. Patch testing is not useful in the burning mouth syndrome. Evaluation of clinical relevance of patch test results is always very difficult and requires an interdisciplinary approach to the patient. Successful treatment requires the identification and elimination of the causative factor, when possible. It is important to bear in mind that replacement of dental restorations and prostheses may be very expensive and stressful for the patient and thus should not be recommended when their causative role is doubtful.
Five women affected by androgenetic alopecia developed severe hypertrichosis of the face and limbs after 2-3 months of treatment with 5% topical minoxidil. Minoxidil was discontinued and in all patients the hypertrichosis disappeared from the face and arms after 1-3 months, and from legs after 4-5 months. Systemic absorption of minoxidil, and a high sensitivity to minoxidil of the follicular apparatus in these areas, is hypothesized.
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.
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Hailey-Hailey disease is a cutaneous abnormality transmitted as an autosomal dominant trait in which impaired interkeratinocyte adhesion produces recurrent blisters in characteristic skin sites. We report here a confirmation of the initial mapping of the mutant gene to chromosome 3q in an additional seven kindreds, narrowing of the candidate region to the sequences flanked by D3S1589 and D3S1541, and the finding in one family of a genomic DNA deletion whose centromeric end is located between these two flanking markers.
Hailey-Hailey disease (familial benign chronic pemphigus) is an autosomal dominant skin disease characterized by impaired keratinocyte cohesion and consequent blister formation. In the present study we have used linkage analysis to map the gene for this disease to a region of chromosome 3q between D3S1589 and D3S1316. The maximum combined two point lod score in four families studied was 14.60 at theta = 0 at the D3S1290 microsatellite repeat. These findings suggest the presence of a gene not previously known to be involved in keratinocyte cohesion at this site.
Hailey-Hailey (Familial Benign Chronic Pemphigus) Disease is a rare autosomal dominant disorder characterized by blisters caused by suprabasal epidermal acantholysis. Another autosomal dominant skin disease, Darier's disease, has clinical and histologic features which overlap those of Hailey-Hailey disease and recently has been mapped to chromosome 12q23-q24.1. We have used linkage analysis to test whether or not a mutation in this region might also underlie Hailey-Hailey disease. This analysis, using polymorphic loci tightly linked to Darier's disease, excluded this region as the site for the disease-causing mutation in two kindreds affected with Hailey-Hailey disease.
We assessed the prevalence of nail abnormalities in 272 children with alopecia areata who were seen in our department during an eight-year period. Of these, 126 (46%; 50 girls, 76 boys) had nail abnormalities that were related to alopecia areata. Nail pitting was detected in 92 patients, including 37 with alopecia totalis or alopecia universalis. Three patients experienced an onychomadesis of all 20 nails during the acute onset of alopecia areata universalis. Thirty-two (11.7%) had nail thinning and severe nail plate surface abnormalities that were consistent with a diagnosis of trachyonychia.
Only 2 cases of histiocytoid hemangioma localized in the nail apparatus have been reported in the literature. We describe here a patient affected by multiple histiocytoid hemangiomas involving the 3rd right digit and fingernail. The pathological features were typical for a diagnosis of histiocytoid hemangioma showing vascular proliferation characterized by enlarged endothelial cells associated with inflammatory cells. Phenotypic characterization of the proliferating cells confirmed their vascular origin.
We describe a 30-year-old woman with syringomyelia, who developed recurrent painless whitlows of the left hand that led to amputation of the terminal phalanx of the 4th finger and considerable shortening of the terminal phalanges of the 1st, 2nd, 3rd and 5th fingers. A roentgenogram of the left hand showed a complete resorption of the terminal phalanges of the 3rd, 4th and 5th fingers and partial resorption of the terminal phalanx of the 2nd finger. Although the defective sensory function undoubtedly contributes to the development of bone resorption by facilitating mechanical repetitive injuries and infections, impaired vasomotor function possibly plays an equally important role.
A large number of drugs may interfere with the hair cycle and produce hair loss. Drugs may affect anagen follicles through 2 main different modalities: (i) by inducing an abrupt cessation of mitotic activity in rapidly dividing hair matrix cells (anagen effluvium) or (ii) by precipitating the follicles into premature rest (telogen effluvium). In anagen effluvium, hair loss usually occurs within days to weeks of drug administration, whereas in telogen effluvium, hair loss becomes evident 2 to 4 months after starting treatment. Anagen effluvium is a prominent adverse effect of antineoplastic agents, which cause acute damage of rapidly dividing hair matrix cells. Telogen effluvium may be a consequence of a large number of drugs including anticoagulants, retinol (vitamin A) and its derivatives, interferons and antihyperlipidaemic drugs. Drug-induced hair loss is usually reversible after interruption of treatment. The prevalence and severity of alopecia depend on the drug as well as on individual predisposition. Some drugs produce hair loss in most patients receiving appropriate dosages while other drugs are only occasionally responsible for hair abnormalities. Both hirsutism and hypertrichosis may be associated with drug administration. Drugs most commonly responsible for the development of hirsutism include testosterone, danazol, corticotrophin (ACTH), metyrapone, anabolic steroids and glucocorticoids. Hypertrichosis is a common adverse effect of cyclosporin, minoxidil and diazoxide.
The authors report a 39-year-old man who developed a phalangeal osteomyelitis of the right thumb in consequence of nail biting. Cultures grew Staphylococcus aureus. Treatment with intravenous teicoplamine 400 mg/day for 3 weeks resulted in complete cure of infection.
The aim of this study was to evaluate the long-term effects of intermittent Cyclosporin A treatment of severe plaque psoriasis. For this purpose we considered the clinical records of 26 patients who had been intermittently treated with Cyclosporin A for 2 to 4 years. All 26 patients had severe plaque-type psoriasis (PASI score > 18) that was unresponsive to conventional treatment. The initial Cyclosporin A dosage was 5 mg/kg/day in 8 cases and 3 mg/kg/day in 18 cases. In all patients, Cyclosporin A treatment was prolonged until complete or nearly complete remission of psoriasis (mean 2 months; range 1-4 months). All patients subsequently underwent a 2-4 months maintenance treatment with Cyclosporin A dosages that were gradually reduced until tapering off. In order to maintain clinical improvement after Cyclosporin A withdrawal, patients were treated with topical steroids, topical tar, emollients and UVA exposure and/or eliotherapy. Cyclosporin A treatment (2.5-3 mg/kg/day) was reintroduced only when clinical relapses reached a PASI score of 12 or more. Duration and dosages of Cyclosporin A cycles were always adapted for the purpose of obtaining an improvement acceptable to the patient (PASI < 8) rather than total clearance of psoriasis. So far, the 26 patients have undergone 3-5 cycles of therapy with low doses of Cyclosporin A. None of these 26 patients interrupted Cyclosporin A treatment because of side effects. In conclusion, in our experience cyclic CyA treatment is effective for the long-term treatment of psoriatic patients.
BACKGROUND: We studied a large series of patients with lichen planus (LP) limited to the nails. OBJECTIVE: Our purpose was to review the clinical and histopathologic features of 24 patients with LP limited to the nails and to discuss treatment and long-term prognosis. METHODS: The records of 24 patients with biopsy-confirmed nail LP were analyzed. Clinical and follow-up data were obtained. RESULTS: Nail LP usually appears during the fifth or sixth decade of life. Neither gender-associated susceptibility nor seasonal influences were detected. In most cases, nail LP is self-limiting or promptly regresses with treatment. Recurrences of nail lesions as well as development of LP in other regions of the body are possible. The development of severe and early destruction of the nail matrix characterizes a small subset of patients with nail LP. CONCLUSION: Approximately 25% of patients with nail LP have LP in other sites before or after the onset of nail lesions. Long-term observation indicates that permanent damage to the nail is rare even in patients with diffuse involvement of the matrix.