[Congenital adipose plantar nodules of children].
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Biomedical subjects
Publications and source records attributed to J-L Verret.
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BACKGROUND: We report a case of multiple benign nodular hidradenomas associated with malignant nodular hidradenoma in a hypogonadic patient. CASE-REPORT: A 49 year-old man presented 11 benign nodular hidradenomas in the pectoral region. A malignant nodular hidradenoma had recently appeared in this region some distance from the pre-existing benign modular hidradenomas. This patient had peripheral hypogonadism associated with congenital bilateral cryptorchidia responsible for gynecomastia due to "relative hyperestrogenism". Large numbers of estrogen receptors were demonstrated in each of the benign nodular hidradenomas. The malignant nodular hidradenoma mass was unavailable and screening for these receptors was not possible. DISCUSSION: Cases of multiple nodular hidradenoma are extremely rare: we have seen only two. To the best of our knowledge, there have been no reports to date of combined malignant nodular hidradenoma and multiple benign nodular hidradenoma. However, there is histological evidence of transition forms. The coexistence of endocrine anomalies associated with this type of tumour has never been reported. However, the presence of estrogen receptors has occasionally been demonstrated in benign nodular hidradenomas. Our patient's endocrine disease may have played a role in the presentation of multiple hidradenomas, but this cannot be demonstrated.
INTRODUCTION: Infantile myofibromatosis is a rare fibrovascular-like, isolated or multicentric tumor, occasionally of the bone or an organ and appearing before the age of 2. We report a case of infantile myofibromatosis in a child in an atypical form with a single, ulcerated plaque and having developed after the onset of clusters of papular nodules. OBSERVATION: An infant was seen in consultation because of asymptomatic papules that had developed on the back. The histological examination of a partial biopsy revealed a histiocytofibromatus aspect and led to the diagnosis of clusters of multiple histiocytofibromatous. One year later, the papular nodules had converged, forming a large plaque with ulcerated center. The progressive extension and the absence of healing prompted surgical exeresis and the final diagnosis of myofibromatosis. DISCUSSION: Diagnosis of infantile myofibromatosis is difficult histologically and clinically and relies on a clear anatomoclinical confrontation. The clinical aspects are varied. To our knowledge, myofibromatosis with a single ulcerated plaque has never been reported in the literature before.
INTRODUCTION: A case of a rare variety of cutaneous pseudo-lymphoma is reported. CASE REPORT: A 13 Year-old boy presented with a nodule on the nose groove which corresponded histologically to a dense, peri follicular lymphocyte infiltration penetrating the hair. Following partial biopsy, the lesion disappeared within a fortnight. COMMENTS: Our case report corresponds to the entity described in 1999 in the Japanese. Our case occurred in a Caucasian French adolescent. The papulonodule, usually unique, is composed histologically of a dense, peripilary infiltrate that penetrates and disrupt the hair. The majority of cases have been removed surgically. Those that have not usually disappear following partial biopsy. The individualization of this entity is debatable: the involvement of the hair follicles by the infiltration of pseudolymphomas on the face is frequent and non-specific.
INTRODUCTION: The so-called "Lever scheme" therapeutic regimen has been proposed in the borderline forms of pemphigus to reduce the side effects of systemic corticosteroids. PATIENTS AND METHODS: A retrospective study was conducted in 8 hospital centers. The criteria for inclusion were the clinical diagnosis of pemphigus, confirmed by histological examination and direct immunofluorescence and first line therapy using the "Lever scheme" protocol, combining 40 mg of prednisone on alternate days and 100 mg/day of azathioprine. RESULTS: Twenty-two patients, seen between January 1990 and December 2000 were included in the study. Eighteen patients (82 p. 100) exhibited complete healing of their cutaneous-buccal lesions after a mean delay of 4.3 months. The lesions of 4 patients did not heal. Three of these patients died: a bed-ridden patient, a patient exhibiting a metastatic bronchial carcinoma and a hypertensive patient who died following a hemorrhagic cerebral vascular accident. Twelve patients (54 p. 100) were weaned off treatment after a mean duration of 2.9 years. Five severe adverse events were observed: one pneumonia, 2 unbalanced diabetes, one hepatitis and one pulmonary embolism. DISCUSSION: This study showed that the healing of the cutaneous-buccal lesions was obtained using the "Lever scheme" in 18 cases out of 22 (82 p. 100). The delay to healing was relatively long in view of the delayed effect of azathioprine. This limits the use of the "Lever scheme" protocol to non-extensive and/or early stage pemphigus. The severe adverse events occurred in low-weight patients in whom the dose related to weight was the highest. Hence the doses of azathioprine and prednisone should be adapted to patients' body weight.
INTRODUCTION: We report the original case of cutaneous periarteritis nodosa that occurred one month following vaccination against hepatitis B. OBSERVATION: A 37 year-old woman, without notable past history, taking no medication, presented with a livedo that had appeared one month after the first injection of a hepatitis B vaccination and had slowly extended over the past 7 years. She did not complain of any other symptom. The histological examination of the livedo showed a necrotic vasculitis. The clinical picture, the histological image and the slow 7-year progression led to the diagnosis of cutaneous periarteritis nodosa. Treatment with hydroxychloroquine (Plaquenil) followed by colchicine had no effect on the livedo. DISCUSSION: Cutaneous periarteritis nodosa is a rare disease of unknown physiopathology. It can be distinguished from systemic periarteritis nodosa by the absence of systemic involvement and benign but prolonged progression. The association of systemic periarteritis nodosa and hepatitis B has been demonstrated. Conversely, only one case of cutaneous periarteritis nodosa associated with hepatitis B viral infection has been described. The vaccination against hepatitis B is frequently prescribed. Following vaccination, a few cases of vasculitis have been reported. The responsibility of the vaccine in the onset of vasculitis has never been formally demonstrated, nevertheless if a cause to effect exists, the physiopathological mechanism might jeopardize the circulating immune complexes containing Ag HBs.
INTRODUCTION: Congenital adipose plantar warts, a recently individualized entity, have an unknown long term evolution. Our patient, seen again aged 28 years represents the longest follow-up of the affection in the literature. OBSERVATION: A young woman presented with congenital plantar nodules. She was seen again at the age of 28: the lesions were still present and made it difficult for her to walk. DISCUSSION: Congenital adipose plantar warts are characterized by a bilateral, asymptomatic, plantar nodule of the heel, present at birth, corresponding histologically to a normal hypodermis, occasionally with interlobular fibrosis. The follow-up of published cases has only covered childhood. Our observation suggests the persistence of the affection into adulthood. It also emphasizes the possible functional problem created when walking.
BACKGROUND: Mechlorethamine is frequently used in the treatment of cutaneous lymphoma, but its application is limited in 30-80% of cases because of cutaneous intolerance. Reducing the concentration to avoid this side-effect has been only modestly successful. OBJECTIVES: To investigate whether a shorter application period could reduce the frequency of intolerance. METHODS: In an open prospective study in 39 patients with cutaneous T-cell lymphoma or parapsoriasis, mechlorethamine was applied according to the usual practices of the participating physicians (number of weekly applications, treatment confined to lesions or performed over the entire body) and then washed off after 1 h in all cases. RESULTS: Cutaneous intolerance was observed in 19 of 39 patients (49%). Six of these patients showed allergic contact dermatitis to mechlorethamine after a mean period of 9.3 weeks, while the other 13 developed irritant contact dermatitis after a longer period. Cutaneous intolerance did not differ significantly according to the number of applications per week or the extent of body area treated. The therapeutic response rate was 69%, and no difference in therapeutic efficacy was noted between daily and intermittent applications. CONCLUSIONS: Comparison with published studies showed no significant difference in the number of cases of cutaneous intolerance after short-term application, although their occurrence was delayed. Therapeutic response was decreased appreciably by short-term application as compared with results in the literature.
BACKGROUND: Lichen nitidus is a rare condition, which may be a cause of palmoplantar hyperkeratosis. We report two cases. CASE REPORTS: A 53 year-old woman presented with a dry and fissured palmoplantar hyperkeratosis. Histological examination of a biopsy showed the typical features of lichen nitidus. Significant improvement was obtained with acitretin. A few months later, multiple lichen nitidus papules appeared on the limbs and the abdomen. A 67 year-old woman was referred to us for a fissured, disabling palmoplantar hyperkeratosis refractory to topical steroids. Histological examination led to the diagnosis of lichen nitidus. Local PUVA therapy resulted in the cleaning of her lesions. Later, typical papules of lichen nitidus appeared on her elbows. DISCUSSION: Nineteen cases of palmoplantar localization of lichen nitidus have been described. The features are usually tiny yellow papules but sometimes a non-specific keratoderma resembling chronic eczema. Palmoplantar involvement of lichen nitidus may be isolated or associated with cutaneous lesions on unusual sites. Oral retinoids and local PUVA are effective treatments.
Idiopathic hypereosinophilic syndrome is characterised by chronic hypereosinophilia leading to tissue damage, and after exclusion of reactive eosinophilia. Until recently no specific or efficient therapeutic was available. In 2003, a recurrent interstitial deletion 4q12 leading to the fusion of the FIP1L1 and PDGFRA genes was detected in hypereosinophilic syndromes. The resulting protein has constitutive tyrosine kinase activity which explains clinical and cytological remission of hypereosinophilic syndrome after treatment by a specific tyrosine kinase inhibitor, imatinib mesylate or Glivec, usually used in chronic myeloid leukaemia. Here we report a patient with hypereosinophilic syndrome associated to peculiar morphology of neutrophilic series and the 4q12 deletion. He presented clinical and haematological remission since the introduction of imatinib mesylate therapy.