Cutaneous manifestations in Hymenoptera and Diptera anaphylaxis: relationship with basal serum tryptase.

OBJECTIVES: To compare the clinical presentation of systemic anaphylaxis to Hymenoptera and Diptera with regard to basal serum tryptase (BT) and to evaluate mastocytosis in patients with elevated tryptase. PATIENTS AND METHODS: The medical records of 140 patients with a history of a systemic reaction to venom were retrospectively reviewed. Symptoms and severity of anaphylaxis and BT were recorded. Most patients with elevated tryptase were screened for mastocytosis: a dermatological examination with a skin biopsy was performed in 19 cases and a bone marrow biopsy in 14 cases. RESULTS: Tryptase was elevated in 23 patients. These patients reported fewer usual skin reactions (urticaria in 26.1% of cases with raised tryptase vs. 76.1% of cases with normal tryptase), more flushing (52.2% vs. 4.3%) and frequently did not present skin reaction (26.1% vs. 9.4%). They presented a more severe reaction (mean grade of severity: 3.48 vs. 2.69). Mastocytosis was diagnosed in seven patients with elevated tryptase: indolent systemic mastocytosis in six cases and cutaneous mastocytosis without systemic involvement in one case. In five cases, mastocytosis was previously undiagnosed. Lesions of cutaneous mastocytosis, diagnosed in five patients, consisted of urticaria pigmentosa in all cases and were often inconspicuous. CONCLUSION: These results demonstrate particular clinical features of the allergic reaction in patients with elevated BT and the higher frequency of mastocytosis in this population. In patients with a severe anaphylactic reaction without urticaria, but with flushing, tryptase should be assayed and an underlying mastocytosis should be considered.

[Two cases of papular elastorrhexis]. / Elastorrhexie papuleuse : deux cas.

BACKGROUND: Papular elastorrhexis is a rare dermatosis characterized by asymptomatic papules on the trunk and the upper extremities. Histological examination shows loss and fragmentation of elastic fibres as well as thickening of collagen bundles. PATIENTS AND METHODS: Case 1: a 46-year-old man was examined with asymptomatic papular lesions for 20 years. Firm and clearly delineated papules ranging from few millimetres to 2cm in diameter became wrinkled at their surface. They were located on the back and symmetrically on the upper limbs. The oldest of them were 15cm wide. Histological examination showed thickened collagen bundles with almost complete loss of dermal elastic fibres, fragmentation of elastic fibres around the lesion and mucin deposits. Standard laboratory tests and bone X-rays were normal. Case 2: a 34-year-old man consulted for clearly delineated asymptomatic papules on the back present for four years. Histological examination was similar to the previous patient and the laboratory tests were normal. He developed Hodgkin's lymphoma. DISCUSSION: We report these two cases because of their particularities as well as the rarity of papular elastorrhexix. The first exhibited large lesions and mucin deposits while the second was associated with Hodgkin's disease. Differential diagnosis of papular elastorrhexis includes Buschke-Ollendorff syndrome, eruptive collagenoma and elastic tissue disorders: macular anetoderma, mid-dermal elastolysis, nevus anelasticus, acne scars and pseudoxanthoma elasticum. The aetiology is unknown. There are no extracutaneous signs.

[Peripheral blood Sézary cells and the diagnosis of Sézary syndrome]. / Recherche de cellules de Sézary dans le sang périphérique: morphologie ou immunophénotype?

Sézary syndrome (SS) is a rare and aggressive cutaneous lymphoma, and its diagnosis is based both on clinical, histological and biological features. None of these criteria taken alone is specific, and the two clinical observations reported here agree with this. Peripheral blood involvement, due to the presence of a variable number of Sézary cells, has been recently fully delineated by the ISCL/EORTC international organizations, and taken into account in the diagnosis and the prognosis of this syndrome. Identification and quantification of peripheral blood Sézary cells on the blood smear is an essential criterion for the diagnosis and is sufficient when associated with relevant clinical or/and histological grounds. Flow cytometry is another tool to demonstrate Sézary cells within the peripheral blood mononuclear cells. The authors discuss about the respective advantages and limits of morphology and flow cytometry in the identification and enumeration of circulating Sézary cells. Molecular biology is helpful in peculiar situations.

[Cutaneous angiosarcoma of the leg without lymphoedema]. / Angiosarcome cutané de jambe sans lymphoedème associé.

BACKGROUND: Cutaneous angiosarcoma is a rare aggressive vascular neoplasm with a poor prognosis, seen chiefly in elderly subjects and usually on the scalp or face. The present case is original because of its localization on the leg without any chronic lymphoedema and because of the long survival period. The treatment modalities are discussed. CASE REPORT: An 87-year-old woman presented with a rapidly growing large deep-purple ulcerated tumour on the anterior aspect of the leg. In addition, two nodules with a similar aspect appeared on the outer surface of the foot. Histological examination showed vascular channels lined with atypical cells consistent with a diagnosis of angiosarcoma. Computed tomography revealed no metastases. Amputation was performed at the thigh and there was no recurrence 30 months later. DISCUSSION: The leg is a rare site of cutaneous angiosarcoma. Treatment usually consists of surgical excision with wide margins followed by radiotherapy, but in some cases amputation is unavoidable.

[Acral lichen sclerosus et atrophicus]. / Lichen scléreux acral.

BACKGROUND: Lichen sclerosus et atrophicus rarely affects the feet or hands and in this case, it is generally part of widespread cutaneous involvement. We report a case of lichen sclerosus et atrophicus involving only the extremities and the vulvar and perigenital area. PATIENTS AND METHODS: A 56-year-old woman presented with lesions of the hands and feet, with ivory white papules on the dorsal aspect of the feet and the distal phalanx of the fingers, a few small keratotic papules with central depressions in the hollow of the palms, erythema on soles and thenar and hypothenar eminences. Further examination revealed lichen sclerosus et atrophicus of the vulva and genitocrural skinfolds. Histological study of these various cutaneous lesions yielded similar results and revealed the typical features of lichen sclerosus et atrophicus. DISCUSSION: A few cases of lichen sclerosus et atrophicus confined to the hands and/or feet have been reported, involving the palms and soles or nail folds, but none has so far affected the genitalia. To our knowledge, no cases of lichen sclerosus et atrophicus involving both faces of the hands and feet and the genital region have ever been reported.

[Basal cell carcinoma of the first toenail]. / Carcinome basocellulaire de l'appareil unguéal de l'hallux.

BACKGROUND: Basal cell carcinoma is a very common form of skin cancer but its occurrence on the toenail unit is very rare. We report such a case of basal cell carcinoma localized on the proximal nail fold of the right hallux. CASE REPORT: A 67-year-old woman had a 7-year history of a non-healing ulcer on the proximal nail fold of the right hallux after antibiotics and treatment of her onychomycosis. Bowen's disease and squamous cell carcinoma were suspected. Histopathologic examination of a biopsy specimen revealed infiltrative basal cell carcinoma. The lesion was surgically excised with a 0.5 cm margin and the defect was repaired by full-thickness skin graft with good functional and cosmetic results. DISCUSSION: Basal cell carcinoma is the most common skin cancer but its localization on fingers, toes and nail units is very rare. Only six cases of basal cell carcinoma on the toe nail unit have been reported to date in the literature. Clinical aspects often mimic benign processes, resulting in misdiagnosis. Treatment requires simple excision or Mohs micrographic surgery. Our case emphasizes the value of biopsy for all nail unit lesions of atypical appearance, course or therapeutic response.

[Pemphigus vulgaris and intraepithelial neoplasia localized to the vagina]. / Pemphigus vulgaire et néoplasie intra-épithéliale de localisation vaginale.

BACKGROUND: Involvement of the vaginal mucosa in pemphigus vulgaris is a rare occurrence. Here we report an original case that resulted in discovery of intra-epithelial neoplasia at the same site. PATIENTS AND METHODS: A 63 year-old woman was followed for 18 years for pemphigus vulgaris treated with prednisone, initially in combination with azathioprine. An erosive lesion was discovered in the pouch of Douglas during routine gynecological examination and demonstrated the histological features of pemphigus, despite remission of the disease at other sites. In spite of resumption of azathioprine and prednisone, the vaginal lesion continued to spread. A further biopsy revealed intra-epithelial vaginal neoplasia together with images of suprabasal cleavage and acantholysis. Surgical removal was carried out. DISCUSSION: Intra-epithelial carcinoma associated with pemphigus vulgaris has been described in rare cases in the cervix but never in the vagina.

[Necrotic livedo after injection of buprenorphine (Subutex)]. / Livédo nécrotique après injections de buprénorphine (Subutex): rôle de l'amidon de maïs contenu dans l'excipient.

INTRODUCTION: Use of buprenorphine (Subutex) is widely used as substitution treatment in opiate addiction although its side-effects are little known. We report a case of localised necrotic livedo subsequent to injection of Subutex. OBSERVATION: A 34 year-old male drug addict on Subutex substitution treatment presented a necrotic livedo and an ulcer with clearly demarcated edges on the skin of the inner elbow. Histological analysis revealed PAS-positive particles in a hypodermic inflammatory lymphohistiocytic infiltrate that were reflective and formed a Maltese cross under polarised light and were identified as maize starch. DISCUSSION: This clinical case describes necrotic livedo lesions induced by injection of Subutex. The underlying mechanisms are associated with maize starch, an excipient used in Subutex. Unauthorised subcutaneous injection of buprenorphine (Subutex) is common among drug addicts but there is little description of the associated complications. In our patient, the presence in cutaneous biopsy samples of maize starch, an excipient of buprenorphine, provides evidence of intravenous administration of this drug.

[Malignant nodular hidradenoma and multiple nodular hidradenomas in a hypogonadic patient]. / Hidradénome nodulaire malin et hidradénomes nodulaires multiples chez un malade hypogonadique.

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.

[Idiopathic hypereosinophilic syndrome: toward a new molecular-targeted therapy and a new cytomorphological and molecular definition]. / Hyperéosinophilie essentielle: nouvelle thérapeutique ciblée et nouvelle définition cytologique et moléculaire.

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.

[Unusual presentation of infantile myofibromatosis with an ulcered plaque]. / Présentation inhabituelle d'une myofibromatose cutanée infantile en plaque unique ulcérée.

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.

[Palmoplantar lichen nitidus: a rare cause of palmoplantar hyperkeratosis]. / Lichen nitidus palmoplantaire. Une cause rare de kératodermie palmoplantaire.

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.

[Pseudolymphomatous folliculitis]. / Folliculites pseudo-lymphomateuses.

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.

[Congenital adipose plantar nodules persisting in a 28 year-old woman]. / Nodules adipeux plantaires congénitaux persistant chez une femme de 28 ans.

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.

[Cutaneous polyarteritis nodosa following hepatitis B vaccination]. / Périartérite noueuse cutanée après une vaccination contre l'hépatite B.

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.

[Treatment of pemphigus vulgaris by azathioprine and low doses of prednisone (Lever scheme)]. / Traitement du pemphigus vulgaire par le protocole Lever faible.

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.