Two atypical cases of cutaneous gamma/delta T-cell lymphomas.

Cutaneous γ/δ T-cell lymphoma (CGD-TCL) is a recent entity described in the newly revised World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas, and is characterized by the γ/δ T-cell receptor expression on atypical lymphocytes. Only a few cases of primary CGD-TCL have been reported, with an extremely aggressive course (median survival time of 15 months). We describe 2 atypical cases of CGD-TCL. The first case was initially misdiagnosed as an inflammatory panniculitis due to the granulomatous infiltrate on the skin biopsy specimen. Diagnosis was confirmed using δ PCR that revealed γ/δ T-cell clonal expansion. The evolution was marked by predominant γ/δ T-cell infiltrate with diffuse body fat involvement as seen on positron emission tomography-computed tomography. The second case is the first described Epstein-Barr virus (EBV)-associated CGD-TCL with a rapidly fatal evolution. CGD-TCL is also a heterogeneous entity and δ PCR and EBV-encoded RNA probe to detect an EBV latent infection may help diagnose and characterize these cutaneous lymphomas.

[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.

[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.

[Crossed spinach-latex allergy revealed by exercise-induced anaphylaxis]. / Allergie croisée épinard-latex révélée par une anaphylaxie induite par l'exercice.

Diagnosis of exercise-induced anaphylaxis is based on conjunction between a specific factor: a specific or nonspecific food allergy and exercise. The authors report observation of a patient who presented with exercise-induced anaphylaxis associated with food allergy to spinach, but also with a cross reaction with latex.

[Association of hepatic adenomatosis and hepatoportal sclerosis in a woman with incontinentia pigmenti]. / Association d'une adénomatose hépatique et d'une sclérose hépatoportale chez une femme atteinte d'incontinentia pigmenti.

We report the case of the association of three uncommon diseases in a young woman: incontinentia pigmenti, portal hypertension due to hepatoportal sclerosis, and liver adenomatosis. Incontinentia pigmenti is a hereditary genodermatosis with pigmentary cutaneous lesions and dysmorphic malformations. In our patient, among liver abnormalities, there were blood biochemical alterations, portal hypertension, and initially neo hepatic nodular lesions. Histological examination of the surgical liver specimen showed several adenomas and fibrosis of the portal tracts with portal vascular changes. The etiopathogenic nature of the adenomatosis and hepatoportal sclerosis is unclear. As a general rule, hepatic adenomatosis is associated with normal liver. We hypothesize that the adenomas could be secondary to changes in hepatic vascularisation.

[Compressive dyspnea and dysphagia caused by sclerous cervical cutaneous metastasis disclosing breast carcinoma]. / Dyspnée et dysphagie compressives par métastase cutanée scléreuse cervicale révélant un carcinome mammaire.

INTRODUCTION: Cutaneous metastases from breast carcinoma are infrequently the first sign of the disease. They usually appear as cutaneous thoracic nodules. We report the case of a cutaneous sclerous metastasis from breast carcinoma revealed by acute compressive dyspnea and dysphagia. CASE REPORT: A 65 years old woman was admitted for compressive acute dyspnea and dysphagia attributed to the growth of a sclerous cervical isolated metastasis. Investigations revealed rapidly growing low-differentiated primitive breast carcinoma. Chemotherapy induced good clinical response allowing a 27 months survival, in spite of a strong chemodependance. DISCUSSION: This case-report of a breast carcinoma's metastasis is notable because of its clinical revealing signs (acute compressive dyspnea and dysphagia), clinical aspect, cervical location preceding primitive cancer's clinical expression, and long term survival with treatment. The initial submaxillary location suggest the possibility of a metastasis to submaxillary salivary glands with secondary extension to the skin.

[Pustular candidiasis in heroin addicts]. / Pustulose candidosique des heroïnomanes.

INTRODUCTION: Pustular candidiasis in heroin addicts is a rare entity in dermatology. We report a case. CASE REPORT: A 29-year-old female heroin addict developed a painful pustular growth on the scalp. There was no fever. Multiple follicular pustulae measuring 2 to 3 mm were associated with hyperesthesia of the scalp and painful cervical nodes. Biopsy showed acute ostiofolliculitis with a few blastospores and mycelial filaments. Candida albicans was isolated from the pustulae and the buccal cavity. Candida serology was positive (indirect immunofluorescence 1/100, coelectrosyneresis: 4 archs). Search for other localizations and HIV serology were negative. The last injection of brown heroin had been taken 15 days earlier; lemon had been added. Treatment with flucanazole (400 mg/d) led to improvement within 48 hours. DISCUSSION: Sudden development of pustulae or nodules in pilous zones in a heroin addict should suggest the diagnosis. Outcome depends on early treatment after diagnosis and search for other localizations. Our case presented two particular aspects: ostiofollicular localization of the pustulae and a long delay (15 days) between the (presumably) last injection and the development of the lesion. Folliculitis develops almost exclusively in addicts who use brown heroin. Contamination by Candida albicans results from the lemon used to improve solubility at injection.

[Can outcome of varicella pneumonia be predicted in a non-immunosuppressed patient?]. / Peut-on prévoir l'évolution d'une pneumopathie varicelleuse chez un sujet non-immunodéprimé?

Varicella rarely occurs in the adult but can cause multi-organ complications. The most frequent (15 to 30% involve varicella pneumonia of unpredictable outcome. In the healthy adult, outcome can be favourable in 5 days or inversely lead to respiratory distress and death in 10 to 20% of the cases. An analysis of the published cases does not reveal any factor predicting unfavourable outcome. A register of varicella pneumonia would help identify potential predictive factors.

[Schnitzler syndrome and Waldenström disease. Fatal outcome of the original case]. / Syndrome de Schnitzler et maladie de Waldenström. Evolution terminale du cas princeps.

The association between chronic urticaria, macroglobulinaemia and various other manifestations has been individualized as Schnitzler's syndrome. We report the terminal course of an original case followed up for 20 years, which ended as lymphoplasmocytic lymphoma with multiple sites, whereas no lymphomatous proliferation, meticulously looked for, had never been found hitherto. The lymphoma, associated with a macroglobulinaemia level above 5 milligrams, was diagnosed as Waldenström disease. Although most cases of Schnitzler's syndrome seem to follow a benign course (but the follow-up is not always long) a few cases have been reported showing evolution towards, or association with, lymphoma. This indicates that the follow-up of patients with Schnitzler's syndrome should be prolonged.

["Tufted angioma". A benign vascular tumor to differentiate with Kaposi sarcoma]. / Le "tufted angioma" (angiome en touffes). Une tumeur vasculaire bénigne à différencier du sarcome de Kaposi.

Among recently characterized vascular tumors, tufted angioma or angioblastoma is a benign acquired slowly progressive cutaneous tumor, which most commonly arises in the neck and upper trunk in children and young adults. This case report emphasizes the clinical and histological features of tufted angioma. Light microscopic examination reveals numerous lobules of closely packed capillaries scattered throughout the dermis. Vascular lumina are difficult to define. There are no atypical cells. Familiarity with tufted angioma should prevent this lesion from being misdiagnosed as malignant vascular tumor arising in young persons, especially Kaposi's sarcoma.

[Eruptive collagenoma. Apropos of a case with ultrastructural study]. / Le collagénome éruptif. A propos d'un cas avec étude ultrastructurale.

A 29-year-old male patient was examined for numerous asymptomatic dermal nodules symmetrically distributed over the back and the shoulders. The nodules were firm, round to oval and varied in size from a few millimeters to 2 centimeters. They had appeared in normal skin without any known previous inflammation or injury, and they has been present without modification for nine years. At histological examination a dermal accumulation of abundant and large collagen bundles was observed. Elastic fibers seemed to be less numerous than normally. Alcian blue showed no increase in acid mucopolysaccharides. An ultrastructural study displayed normal, mature collagen fibers arranged in large bundles. Cutaneous collagenoma, initially described by Colomb as "eruptive collagenoma" is a rare disorder of unknown etiology occurring in young adults as symptomless dermal nodules without previous history of illness or injury and mostly localized to the back. No change is usually observed in these nodules for several years. Histologically, the nodules are characterized by a proliferation of collagen in the dermis; elastic fibers are generally reduced in number. Our ultrastructural study showed that proliferation of normal collagen fibers is the main morphological finding.