[Diffuse cutaneous mastocytosis of an infant: A case report]. / Mastocytose cutanée diffuse du nourrisson : à propos d'un cas.

Mastocytosis is a group of diseases related to abnormal accumulation and proliferation of mast cells in one or more organs. They may be associated with an acquired point mutation and the activation of the receptor tyrosine-kinase c-KIT of CFS (mast cell growth factor). The clinical manifestations are varied and secondary to the release of mast cell mediators and/or infiltration of various organs. There are two main types of mastocytosis: pure cutaneous mastocytosis and systemic mastocytosis when more than two organs are involved in mast cell infiltration (bone marrow, gastrointestinal tract, bone, liver and spleen, lymph nodes). Mastocytosis affects children in two thirds of cases, most frequently as an isolated cutaneous form. The most common clinical form in children is urticaria pigmentosa and solitary mastocytoma; bullous diffuse mastocytosis is rare. We report the case of an 8-month-old infant who presented with a diffuse pruritic bullous eruption. The histology and immunohistochemistry results were suggestive of mastocytosis. A serum tryptase test yielded positive results. Laboratory investigations did not identify systemic involvement. The patient was given antihistamine H1 medication and local care. Advice regarding the disease was offered to the parents. The course of the disease was marked by a decrease in the number of blisters and attenuation of the pruritus at the 6-month follow-up. This observation emphasizes the importance of awareness of this rare entity, which should be considered in all cases of bullous dermatosis in children, thereby allowing for early treatment.

Systemic paraneoplastic vasculitis secondary to papillary carcinoma of the thyroid.

Systemic vasculitis secondary to thyroid carcinomas is exceptional. We report the case of a 55-year-old woman who presented with systemic vasculitis involving leucocytoclastic cutaneous vasculitis and renal disorders secondary to papillary thyroid carcinoma (PTC). Her symptoms resolved completely after total thyroidectomy. Other causes of vasculitis were excluded. To our knowledge, this is only the second case reported of systemic vasculitis associated with PTC in a paraneoplastic manner.

[Multiple facial nodules revealing disseminated cryptococcosis in an immunocompetent patient]. / Nodules multiples du visage révélant une cryptococcose disséminée chez un patient immunocompétent.

BACKGROUND: Cryptococcosis is a potentially severe infection that usually occurs in a setting of immunosuppression. Its occurrence outside of this context is rare. We report a case of disseminated cryptococcosis revealed by a spectacular skin disease in an immunocompetent patient. PATIENTS AND METHODS: A 40-year-old male patient had been presenting multiple nodules and tumors on his face for one month in a context of asthenia and intermittent fever. Histological examination of a skin biopsy revealed encapsulated yeasts strongly suggestive of Cryptococcus neoformans. Mycological examination of the skin biopsy and cerebrospinal fluid isolated Cryptococcus gattii. The blood cultures were positive. Brain MRI demonstrated cryptococcal parenchymal involvement. Screening for primary or secondary immunodeficiency was negative. The patient received amphotericin B 1mg/kg/day and fluconazole 600mg/day but died 2months after diagnosis. DISCUSSION: Cryptococcosis is a potentially severe infection caused by C. neoformans. This rare condition occurs most commonly in patients with profound deficiency in terms of cellular immunity. Although rare, the occurrence of cryptococcosis in immunocompetent patients is possible, and in this event the signs are highly polymorphic, which usually makes it very difficult to diagnose. The diagnosis of cryptococcosis is based on the identification by direct examination and after staining with India ink of encapsulated yeasts of the Cryptococcus genus. Culture on Sabouraud medium is essential for identification of the species. Treatment for disseminated cryptococcosis involves amphotericin B, often associated with flucytosine IV. In the event of meningitis infection in non-HIV patients, mortality continues to be around 15%, despite adequate medical treatment. CONCLUSION: Although rare, cryptococcosis can occur in immunocompetent subjects. The prognosis is severe even after treatment.

[Recurrence of cutaneous sarcoidosis during immune reconstitution syndrome in an HIV-infected patient]. / Récidive de sarcoïdose cutanée lors du syndrome de restauration immunitaire chez une patiente infectée par le VIH.

BACKGROUND: Association of sarcoidosis and HIV can occur in the context of immune reconstitution syndrome (IRS) after initiation of antiretroviral therapy (ART). Herein we report a case of cutaneous sarcoidosis in remission in an HIV-infected patient but relapsing during IRS associated with initiation of ART. PATIENTS AND METHODS: A 33-year-old female HIV-infected patient from Cameroon was treated with triple therapy with good efficacy. The patient previously had a small nodular lesion on her left cheek which disappeared spontaneously 2 months before the diagnosis of HIV infection. Three months after initiation of triple ART, the patient consulted again for recurrence of the lesion, which had gradually increased in size. Clinical examination revealed a purplish-red nodular plaque of lupoid appearance under vitropression, located between the inner corner of the eye, the nasal wing and the left cheek. A skin biopsy revealed giant-cell epithelioid dermal granulomas without caseous necrosis. Blood angiotensin-converting enzyme levels were elevated and intradermal reaction to tuberculin was negative. A diagnosis was made of cutaneous sarcoidosis. The patient was treated with chloroquine 200mg/day for 3 months, resulting in total subsidence of the lesions. No recurrence was observed at 1 year. DISCUSSION: Introduction of ART has changed the dermatological aspect of HIV infection. In addition to specific dermatological signs specific to HIV and to immunosuppression, there are the cutaneous adverse effects of antiretroviral drugs and skin disorders indicating reconstituted immunity during IRS. Schematically, three forms of IRS may be distinguished: the paradoxical form, the infectious form, and the inflammatory form. The latter corresponds to the onset or exacerbation of inflammatory conditions or autoimmune diseases after the start of ART. Thirty cases of association between sarcoidosis and HIV have been described, of which two-thirds occurred during IRS. The central role of CD4 in sarcoidosis explains its occurrence in HIV patients during reconstitution of the CD4 count. CONCLUSION: In HIV-infected patients treated with anti-retroviral treatment, certain skin diseases such as sarcoidosis may be related to IRS.

[Kaposi's disease: an unusual side-effect of topical corticosteroids]. / Maladie de Kaposi : un effet secondaire inhabituel des dermocorticoïdes.

BACKGROUND: Kaposi's sarcoma is an angiogenic tumour associated with HHV8 infection. There are several clinical forms, including those related to acquired immunosuppression. We report a case of nodular lesions in Kaposi's disease that appeared during treatment of bullous pemphigoid with topical corticosteroids. PATIENTS AND METHODS: A 74-year-old female patient presented bullous pemphigoid lesions on all four limbs that were treated with clobetasol cream, a topical corticosteroid. After 2 months of treatment, the patient noted the appearance of several asymptomatic purple, erythematous, papulonodular lesions of elastic consistency on her right thigh and both legs. The histological examination revealed Kaposi's disease with positive immunolabelling for HHV8 in the tumour cells. HIV serology was negative. The lesions disappeared 4 months after cessation of corticosteroid therapy and the patient was still in remission one year later. COMMENT: Authentic "Mediterranean" cutaneous Kaposi's disease was apparently revealed in this patient during treatment with extremely potent topical corticosteroids, before regressing completely on discontinuation of the medication. We discuss the various mechanisms that could have contributed to this side-effect of dermal corticosteroids, whose sustained application over a wide area caused HHV8 antigen reactivation.

[Pemphigus vulgaris: an unusual clinical presentation]. / Pemphigus vulgaire de présentation inhabituelle.

BACKGROUND: Pemphigus vulgaris is a bullous auto-immune disease affecting the skin and mucosa. It is characterised by acantholysis that results in the formation of intraepithelial bullous lesions. Herein we report a case distinguished by its unusual clinical presentation. PATIENTS AND METHODS: A 45-year-old man, a chronic smoker, consulted for hyperkeratotic lesions of the toes on the right foot present for 5 months. Examination revealed a violet colour of the toes associated with localised yellowish keratoderma on the sole of the foot and impaired toe nails, as well as impairment on the nails of the first, third and fourth fingers on the right hand. In addition, two hyperkeratotic plaques with crusts were noted on the patient's forehead. Histological examination of a biopsy sample taken from the nail bed of the fourth toe on the right foot showed suprabasal acantholysis with a characteristic tombstone appearance. Direct immunofluorescence confirmed the diagnosis of pemphigus. Oral corticosteroid therapy was initiated consisting of prednisone 1.5mg/kg per day. Improvement of the nail lesions and subsidence of the lesions on the patient's forehead occurred after three months of treatment. DISCUSSION: During the course of pemphigus, the nail findings most commonly reported in the literature are paronychia, onychomadesis and onycholysis. However, these signs are generally seen in patients with known pemphigus vulgaris and only rarely indicate bullous disease. The case we report illustrates a special situation in which pemphigus vulgaris was revealed by unusual skin and nail lesions.

[Specific tongue involvement revealing sarcoidosis]. / Atteinte linguale spécifique révélatrice d'une sarcoïdose.

BACKGROUND: Sarcoidosis is a multisystem granulomatous disease of unknown aetiology. Tongue involvement is extremely rare. Herein we report a new case. PATIENTS AND METHODS: A 65-year-old woman presented with a 6-month history of painless multiple nodules on the tongue. The patient also complained of xerostomia. Besides nodules on the tongue, physical examination revealed erythematous nodules on the dorsum of the hands and feet. A diagnosis of sarcoidosis with tongue, cutaneous and salivary gland involvement was made on the basis of these findings and further investigations performed. The patient's condition showed improvement with partial regression of tongue lesions after oral steroid therapy. DISCUSSION: Tongue involvement in sarcoidosis is exceedingly rare. It may be isolated or part of a generalized multisystem sarcoidosis. Nodules, indurations and swelling are the most common clinical manifestations. Management of tongue sarcoidosis is not consensual. Oral corticosteroids appear to be the most effective therapy. CONCLUSION: Sarcoidosis of the tongue should be considered when chronic unexplained nodules are noted. Diagnosis can easily be confirmed by a histological examination of mucosal biopsy.

Clinical features and histological findings are potential indicators of activity in lesions of common vitiligo.

BACKGROUND: Unstable vitiligo lesions are usually considered to be contraindications for surgical treatment. Unfortunately, in the majority of common vitiligo cases, neither accurate clinical signs nor routine blood tests are available to determine whether or not the disease is active. OBJECTIVES: To establish a direct correlation between the clinical aspect of vitiligo lesions and their activity. METHODS: This was a prospective observational study that took place over 1 year. For each patient, a standardized evaluation included both a careful daylight and Wood's lamp examination, pictures, biopsies performed at the marginal area and histological and immunohistological studies. A second examination to assess the activity of the lesions correctly was performed 1 year after inclusion in the study. Both the clinical changes and the histological features of the lesions in actively spreading vitiligo were compared with those in stable vitiligo. RESULTS: This study included 50 patients. The lesions were classified as hypomelanotic with poorly defined borders (HPDB, 29 cases) or amelanotic with sharply demarcated borders (ASDB, 21 cases). One year after the biopsy, of the 48 patients still in the study, 20 had lesions that were considered to be stable and 28 had active lesions. Correlations were successfully obtained between clinical aspects, histological findings and vitiligo activity. The HPDB and ASDB lesions were correlated respectively with active and stable status (P < 0·001). CONCLUSIONS: A simple clinical examination including a Wood's lamp examination may allow reliable and efficient evaluation of the stability of vitiligo lesions.