[Generalized granuloma annulare: remission during treatment of HIV infection]. / Granulome annulaire généralisé: guérison au cours du traitement d'une infection par le VIH.

INTRODUCTION: Granuloma annulare is a benign dermatosis characterized by pale or erythematous papules grouped in rings or in arch form figures. We report the observation combining a granuloma annulare and a Human Immunodeficiency Virus (HIV) infection, with regression of the granuloma annulare on initiation of treatment of the HIV infection. OBSERVATION: A 33 year-old man presented with an eruption of multiple, erythematous papules predominating on the trunk and limbs but sparing the face. Histology confirmed the diagnosis of generalized granuloma annulare. We diagnosed an HIV infection. Remission of the granuloma annulare was obtained concomitantly when immune restoration was obtained following the prescription of an antiretroviral tritherapy. DISCUSSION: Since 1985, several cases of atypical forms of granuloma annulare have been reported in HIV-infected patients. Nevertheless, the precise relationship between the two diseases is unknown. The case we report on raises the question of the relationship between granuloma annulare and immunodepression.

[Paraneoplastic pemphigus revealing dendritic cell sarcoma originating from Castleman's disease of the neck]. / Pemphigus paranéoplasique révélateur du développement d'un sarcome à cellules dendritiques à partir d'une tumeur de Castleman pulmonaire.

INTRODUCTION: Paraneoplastic pemphigus is associated with Castleman's disease. We report a case of paraneoplastic pemphigus at the stage of the sarcomatous transformation of Castleman's disease, present for many years but without concomitant paraneoplastic pemphigus. The pemphigus was manifested by the most unusual, exclusive, involvement of the mucosa of the mouth and lung. OBSERVATION: A 32 year-old man suffering from extensive ulceration of the oral mucosa was hospitalized in December 2000 for alteration in his general status of health and acute respiratory failure. The search for intercellular anti-substance antibodies on rat spleen was positive, corresponding to anti-envoplakin IgG and leading to the diagnosis of paraneoplastic pemphigus. The thoracic x-ray and scan revealed a hilum tumor, the histological examination of which confirmed the diagnosis of Castleman's disease concomitant to sarcomatous transformation. Following surgical treatment, the respiratory failure worsened. The patient improved with systemic corticosteroids at the dose of 2 mg/kg/d and chemotherapy was initiated. The patient died suddenly within the context of acute respiratory failure, three months after surgery. DISCUSSION: This is a case of paraneoplastic pemphigus of unusual clinical and biological expression: exclusively mucosal involvement with obliterating bronchiolitis, explained by the isolated presence of antibodies recognizing envoplakin, without anti-desmoglein. The transformation of the Castleman tumor into a sarcoma may have unmasked intra-cellular antigens (plakins), initiating the specific immune reaction.

[Genital papules revealing a Churg-Strauss syndrome]. / Angéite granulomateuse de Churg et Strauss révélée par des lésions papuleuses de la verge.

INTRODUCTION: Cutaneous lesions are rarely inaugural in Churg and Strauss syndrome. We report the case of a patient in whom recurrent papular lesions of the penis revealed a Churg-Strauss granulomatous angeitis. OBSERVATION: A 54 year-old man consulted for recurrent non-infiltrated and painless erythematous papules of the penis over the past 4 years. His past history revealed severe asthma, which had appeared at the same time, nasal polyposis and chronic sinusitis. Other than the genital organs, the clinical examination was normal. Histological exploration of a cutaneous biopsy of the penis showed a necrotic granulomatous vasculitis. The usual biological examinations were normal. Research for proteinuria, hematuria and measurement of polynuclear anticytoplasm antibodies were negative. Pulmonary X-ray revealed a bilateral interstitial syndrome with micronodules. The broncho-alveolar wash revealed moderate granulocyte alveolitis with numerous eosinophilic polynuclears. The electromyogram showed a reduction in the sensitive potential of the nerves explored in the 4 limbs. Churg-Strauss granulomatous angeitis was diagnosed. Treatment with 0.8 mg/kg/day of prednisone was initiated. The cutaneous lesions disappeared rapidly. Since three months later there had been no relapse, corticosteroid therapy was slowly and progressively reduced. Nine months later, the patient exhibited new papular lesions of the glans penis and progressive relapse of asthma. Treatment with cyclophosphamide (Endoxan(R)) combined with systemic corticosteroids was initiated. DISCUSSION: In view of the concomitance of necrotic granulomatous vasculitis in the cutaneous biopsies, severe, recent corticosteroid-dependent asthma, eosinophil-rich pulmonary infiltrate, allergic rhinitis, chronic sinusitis and the onset of a neuropathy, the diagnosis of Churg-Strauss syndrome was retained. The absence of hypereosinophilia in the blood during diagnosis can be explained by the oral corticosteroid therapy at low doses because of the asthma and sinusitis. However, hypereosinophil blood levels had been reported in several prior hemograms. Damage to the genital organ is exceptional during Churg-Strauss syndrome and is usually of granulomatous prostatitis form. Only one other case has been reported. Our observation is original in that it was isolated to the genital skin, which led to the diagnosis of Churg and Strauss granulomatous angeitis with systemic involvement.

[Paresthetic notalgia and multiple endocrine neoplasia type 2a (Sipple's syndrome): 3 cases]. / Notalgie paresthésique et néoplasie endocrinienne multiple de type IIA (syndrome de Sipple): 3 cas.

INTRODUCTION: Notalgia paresthetica is an isolated sensory mononeuropathy. Patients have a pruritus in the mid-upper back. Its association with multiple endocrine neoplasia type IIA has been reported in a few cases. We report three cases of this association. CASE REPORTS: Case n(o) 1: A 45 year-old woman had multiple endocrine neoplasia type IIA with a medullary thyroid carcinoma and a primary hyperparathyroidism; she had a mid-upper back pigmented lesion. Histological examination showed dermal melanosis and deposits of amyloid in the dermis. Case n(o) 2: A woman had a multiple endocrine neoplasia type IIA which was diagnosed at the age of 60; she had a surgical treatment for a pheochromocytoma, a medullary thyroid carcinoma, and a primary hyperparathyroidism; she had dermatological examination for a pruriginous lesion of the mid-upper back. Case n(o) 3: The daughter of the patient n(o) 2 had had a surgical cure for a medullary thyroid carcinoma and a pheochromocytoma at the age of 31; she had a papulous and pruriginous lesion in the left scapular area. Her daughter and her sister had a multiple endocrine neoplasia type IIA without notalgia paresthetica. DISCUSSION: Notalgia paresthetica is a benign cutaneous disorder which can be associated with multiple endocrine neoplasia type IIA. It can be considered that notalgia paresthetica is an early clinical marker of multiple endocrine neoplasia type IIA. Patients with a familial history of notalgia paresthetica or with an onset of notalgia paresthetica in childhood should be screened for multiple endocrine neoplasia type IIA. Patients with multiple endocrine neoplasia must also been screened for notalgia paresthetica because its finding is an argument for a familial form of multiple endocrine neoplasia type IIA. Dermatologists should be aware of this association.

[Paraneoplastic pemphigus: a pustular form during chronic lymphoid leukemia]. / Pemphigus paranéoplasique. Une forme pustuleuse au cours d'une leucémie lymphoïde chronique.

BACKGROUND: Paraneoplastic pemphigus is an autoimmune disease of the skin and mucosa described in 1990. The condition is generally associated with lymphoma or chronic lymphoid leukemia. Lesions are often misleading, masquerading as polymorphous erythema or lichen. We report a case of paraneoplastic pemphigus with pustulous skin lesions. CASE REPORT: A 52-year-old man developed over a few weeks time erosive lesions of the oral cavity and lips associated with papulous skin lesions. Secondarily, large-sized pustules, sometimes a hypopion, were observed associated with bullae. The diagnosis of paraneoplastic pemphigus was confirmed by direct immunofluorescence that evidenced IgG deposits within the keratinocytes and along the basal membrane and by indirect immunofluorescence on rat bladder that evidenced circulating antibodies. This paraneoplastic pemphigus was the inaugural sign of chronic lymphoid leukemia. DISCUSSION: Skin lesions described in paraneoplastic pemphigus include: erosion, vesicles, bullae, and psoriasiform, lichen-like, plate-like or vegetative formations. To our knowledge, this is the first report of a pustulous form; clinically similar to Hallopeau pustulous pemphigus.