[Bullous pemphigoid in young patients: a retrospective study of 74 cases]. / Pemphigoïde du sujet jeune.

INTRODUCTION: Bullous pemphigoid usually affects elderly people. Only a few isolated cases among people younger than 65 years have been reported. OBJECTIVES: Describe the clinical and biological characteristics of patients younger than 60 years suffering from bullous pemphigoid, compare them with the usual characteristics known among elderly people and search for potential pathological associations. PATIENTS AND METHODS: Retrospective, national, multicenter study. Clinical, biological and histological characteristics were recorded with a standardised questionnaire as well as treatments and associated pathologies. RESULTS: Seventy-four cases of bullous pemphigoid diagnosed between June 1970 and March 2002 were analyzed. Mean age at the beginning of the disease was 46 +/- 11.6 years. Further explorations by indirect immunofluorescence of separated skin and/or immuno-electron microscopy and/or immunoblotting were performed for 42 patients (56.8 p. 100). Clinical characteristics among this restricted population were comparable to those found among the 32 other cases. Compared to usual data on bullous pemphigoid in elderly people, we observed a greater proportion of extensive form of disease (75 p. 100), a more frequent head and neck involvement (39.2 p. 100) and an overexpression of anti-BP180 autoantibodies (48 p. 100). Neoplasm was notified for 7 patients (9.5 p. 100), 18 (24.3 p. 100) suffered from a pathology of the basement membrane zone (6 psoriasis, 6 atopic dermatitis and 6 lichen) and 13 from neurological disease, among which 4 were bedridden. Fourty-six patients (62.2 p. 100) received drugs for the long term (mean 2.12 +/- 2.43), 4 patients were treated by PUVAtherapy and 2 by radiotherapy. DISCUSSION: Our results suggest that bullous pemphigoid among young people is more severe and more active than the usual form in the elderly. This particular form could be the result of a higher expression of anti-BP180 autoantibodies, which are considered as a marker of poor prognosis in this disease. We also found a high frequency of pathological associations and physical treatment, all responsible for damage to the basement membrane zone, which can involve auto-immunization against hemidesmosome components.

Multiple perforating and non perforating pilomatricomas in a patient with Churg-Strauss syndrome and Rubinstein-Taybi syndrome.

We report an unusual association of multiple perforating and non-perforating pilomatricomas with Churg-Strauss syndrome, and a dysmorphic syndrome evocative of Rubinstein-Taybi syndrome. These syndromes may be independent, but these rare diseases and genetic abnormalities may be linked together.

[Sneddon-Wilkinson disease. Four cases report]. / Maladie de Sneddon-Wilkinson. A propos de quatre cas.

INTRODUCTION: We report four cases of subcorneal pustular dermatosis or Sneddon-Wilkinson disease. Clinical and histological lesions and immunofluorescence data were presented. This disease is classified among neutrophilic dermatitis. PATIENTS AND METHODS: All of four patients presented with clinical and histological lesions compatible with the diagnosis of Sneddon-Wilkinson disease. Indeed, direct and indirect immuno-testing were negative. We noted an association with a benign IgA monoclonal gammapathy in one case and with a seronegative polyarthritis in one other case. Three patients correctly responded to dapsone. One of them after transient improvement was resistant to dapsone and then dramatically responded to etretinate. CONCLUSION: Subcorneal pustular dermatosis is a chronic disease, rarely described in literature. It's a pustular eruption, involving the trunck, axillae and inguinal holds. It's often associated with monoclonal gammapathy, particulary IgA. Its nosological situation is still contested, especially with IgA pemphigus sharing with it the association with IgA monoclonal gammapathy and the same efficacy of dapsone. We discuss relationships between both diseases.

[Eccrine angiomatous hamartoma: an atypical case]. / Hamartome angio-eccrine. Un cas de présentation atypique.

BACKGROUND: We report a case of eccrine angiomatous hamartoma. This rare and benign skin disease is histologically characterized by the proliferation of both eccrine glands and vascular structures. CASE REPORT: A 65 year-old man had developed during the previous 8 years post-traumatic, angiomatous, extensive and plurifocal lesions of the left lower limb. There was neither pain or local hyperhidrosis. The diagnosis of eccrine angiomatous hamartoma was made on histological evidence. DISCUSSION: This observation is original because of the onset of the disease after 50 years of age, its widespread and plurifocal character and probable triggering-off by minor local trauma. The classical surgical treatment of eccrine angiomatous hamartoma was not suitable and a laser-CO2 therapy was undertaken instead. The results were very discreet.

[Cutaneous T-cell lymphoma, cutaneous hyperpigmentation and paraneoplastic pemphigus]. / Lymphome cutané T, hyperpigmentation cutanée et pemphigus paranéoplasique.

BACKGROUND: Paraneoplastic pemphigus is an autoimmune mucocutaneous disease usually associated with neoplasia as lymphoid proliferations. We report the original case of a patient who had developed a mycosis fongoide preceded by a paraneoplastic pemphigus. To our knowledge, this association has never been reported before. Cutaneous manifestations of mycosis fongoide as pigmentary change are known. This mycosis fongoide was particular in its progressive cutaneous hyperpigmentation. CASE REPORT: A 70-year-old male patient developed a mycosis fongoide with CD30 positive cells in the dermis several months after the diagnosis of a paraneoplastic pemphigus. Simultaneously, a cutaneous hyperpigmentation was predominantly noticed on photo-exposed areas, which improved after chemotherapy. DISCUSSION: Paraneoplastic pemphigus may precede the cancer, as is shown by our present case. This paraneoplastic pemphigus is singular because of the lack of oral erosions, patient's prolonged survival and its association with a mycosis fongoide. The diagnosis of mycosis fongoide with CD30 + cells was finally established together with its relationship to the cutaneous hyperpigmentation. Indeed, a few cases of pigmentary changes in mycosis fongoide have already been reported. The pathogenesis is still unknown, but the role of mast cell and stem cell factor in hyperpigmented mycosis fongoide has been proposed.

[Acute generalized exanthematous pustulosis due to fluconazole]. / Pustulose exanthématique aiguë généralisée induite par le fluconazole.

INTRODUCTION: Fluconazole (Triflucan(R)), a systemic triazole antifungal agent is largely prescribed and some cutaneous side effects have already been described. We report the first case of acute generalized exanthematous pustulosis due to this molecule in a patient with cutaneous candidosis. CASE REPORT: A 65 year-old-woman was treated with fluconazole (200 mg/day) for a persistent cutaneous candidosis infection on the buttocks. After the third dose, the patient presented with a pustular eruption with erythema located on her trunk and in her large skin folds. The eruption was associated with fever at 39 degrees C, asthenia and neutrophilia (9,000/mm(3)). The histologic examination and the negativity of microbiological cultures were consistent with the diagnosis of acute generalized exanthematous pustulosis. The eruption cleared with local steroids in about ten days. Nineteen days later, the same pustular eruption occurred but without fever nor neutrophilia. DISCUSSION: Clinical, biological and histological manifestations were consistent with the diagnosis of acute generalized exanthematous pustulosis due to fluconazole. According to the imputability criteria of Begaud et al., intrinsic imputability of fluconazole was possible (I2). According to the classification of the EuroSCAR study, it was certain. No similar case of recurrence had already been described after the withdrawal of the molecule. We believe this is the first case of acute generalized exanthematous pustulosis due to fluconazole (extrinsic imputability: B0).

Primary hyperparathyroidism and cutaneous T-cell lymphoma: fortuitous association?

This is the third report of an association between T-cell cutaneous lymphoma (mycosis fungoides) and primary hyperparathyroidism (adenoma). Some studies support the concept that hyperparathyroidism may have promotional activity for the development of certain malignant tumors. A high risk for successive or concurrent neoplasms has been reported in patients with parathyroid adenomas. Primary hyperparathyroidism in a neoplastic context may be underreported. Patients with tumor-associated hypercalcemia should be evaluated for the possibility of primary hyperparathyroidism.

[Panniculitis revealing alpha-1 antitrypsin deficiency. Report of 3 cases]. / Panniculite révélant un déficit en alpha 1-antitrypsine.

We report panniculitis revealing alpha-1 antitrypsin deficiency in 3 patients with different Pi phenotypes. The first patient, a 16-year-old woman, had inflammatory skin lesions on the abdomen for 6 months. The lesions regressed spontaneously. Serum alph-1 antitrypsin level was normal but the Pi phenotype was MS. The second case was observed in a 56-year-old man who developed erythematous subcutaneous nodules on the abdomen, legs and buttocks in a trauma context. Serum alpha-1 antitrypsin was very low and the Pi phenotype was ZZ. The third patient was a 40-year-old woman who presented red swelling nodules on the legs. Her serum alpha-1 antitrypsin level was at the lower limit of normal and the Pi phenotype was MZ. Alpha-1 antitrypsin deficiency is an autosomic codominant inherited disorder characterized by inefficient or non-functional serum alpha-1 antitrypsin. The principal clinical manifestations are panlobular emphysema and cirrhoses. About 30 cases of panniculitis have been reported in the literature. In patients presenting panniculitis, we suggest studying the Pi phenotype to determine functional deficiency even if the serum level of alpha-1 antitrypsin is normal.

Prolonged photosensitivity following contact photoallergy to ketoprofen.

We report the third case of prolonged photosensitivity secondary to contact photoallergy to topical ketoprofen, a 2-arylpropionic acid derivative. The patient suffered from persistent photosensitivity for more than 1 year after the withdrawal of ketoprofen with recurrent eruptions on sun-exposed skin areas. This photosensitivity was associated with a persistent decrease in polychromatic and UVA minimal erythemal doses. Photobiological testing revealed cross-reactivity with fenofibrate and benzophenones. Photoallergy to ketoprofen is due to the benzophenone structure or to the very similar thiophene phenylketone of tiaprofenic acid, but not to the arylpropionic function. Thus, fenofibrate, tiaprofenic acid and benzophenones should be avoided by patients with a positive history of photocontact dermatitis to ketoprofen.