[Panniculitis revealing alpha-1 antitrypsin deficiency. Report of 3 cases].

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.

[Intravascular papillary endothelial hyperplasia: multiple lesions appearing in the course of treatment with interferon beta].

Introduction: Intravascular papillary endothelial hyperplasia is a benign and rare vascular lesion. We report a case of multiple vascular lesions of the hand following 3 months treatment with beta-interferon injections for multiple sclerosis.

Observation: A 50 year-old man had multiple vascular nodules of the hands.

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.

[Multiple cutaneous reticulohistiocytosis].

Background: Multicentric reticulohistiocytosis is a non Langerhans cell histiocytosis. This rare disease is characterized by cutaneous papules and nodules and a destructive polyarthritis; multisystem involvement may occur. Multiple and diffuse cutaneous reticulohistiocytosis have been more rarely reported.

Paraneoplastic pemphigus associated with Castleman's tumour.

We present a case of paraneoplastic pemphigus associated with Castleman's disease. Our patient had stomatitis and vulvar erosions followed by a cutaneous polymorphous eruption. Investigations for neoplasia disclosed a Castleman's tumour suggesting the diagnosis of paraneoplastic pemphigus.

Pili torti with congenital deafness (Bjornstad syndrome): a case report.

We report Bjornstad syndrome in a 5-year-old girl with severe bilateral congenital loss of hearing and pili torti. The mode of inheritance of this rare syndrome seems to be heterogeneous. A maternal uncle of the patient was deaf from birth and his hair had shown the same abnormalities at the same age; an autosomal recessive transmission can be assumed.

Cutaneous manifestations of relapsing polychondritis in a patient receiving goserelin for carcinoma of the prostate.

Relapsing polychondritis is a chronic rheumatologic disorder of unknown etiology. Cutaneous manifestations occur in nearly half of the patients and often precede cartilaginous involvement. We present the case of a man with a history of prostatic adenocarcinoma who underwent monthly injections of goserelin (Zoladex), an LH-RH analogue.

Contact allergy to 3-(4' methylbenzylidene) camphor and contact and photocontact allergy to 4-isopropyl dibenzoylmethane.

We report a clinical case of a 15-year-old boy who presented a photodistributed eruption after having used a sunscreen cream for 8 days. Photobiological testing showed a contact allergy to 3-(4' methylbenzylidene) camphor (Eusolex 6300) included in his sunscreen cream associated with a contact and photocontact allergy to 4-isopropyl-dibenzoylmethane (Eusolex 8020) not included.

Localized lipoatrophia with persistent circulating autoantibodies and partial immunoglobulin A deficiency in a child.

A 7-year-old Caucasian girl developed lipoatrophic areas on the abdomen and left thigh. Laboratory tests showed a partial IgA deficiency, circulating autoantibodies (ANAs, double- and single-stranded anti-DNAs, rheumatoid factor). The clinical aspect resembles lipodystrophia centrifugalis abdominalis infantilis, but autoimmune abnormalities suggest connective tissue panniculitis which underlines the overlap between the different forms of localized lipoatrophias.

[Systemic sensitization to 17-beta estradiol induced by transcutaneous administration].

Introduction: Transdermal administration of drugs to obtain a systemic effect may lead to allergic sensitization and compromise subsequent use via another administration route.

Observation: A 52-year-old woman presented contact eczema with the transdermal therapeutic system Estraderm TTS50. Generalized eczema developed later after oral administration of an oestrogen derivative.

Acquired localized elastolysis associated with varicose veins.

Localized elastolysis refers to a group of heterogeneous conditions which includes cutis laxa. We report a form of localized and non-inflammatory elastolysis evocative of cutis laxa, localized to the anteromedial aspect of the left thigh and knee, acquired at the age of 8 years and associated with unilateral adjacent venous dilatations appearing at the age of 39 years. Our case is clinically and ultrastructurally compatible with cutis laxa.

Photosensitive and phototriggered pityriasis rubra pilaris.

We report a 59-year-old man with photosensitive and photodistributed pityriasis rubra pilaris. The phototriggering of the lesions was obtained by the iterative polychromatic phototest (3 times the minimal erythema dose (MED) on 3 consecutive days) and was also observed on the supra MED sites, which began the 10th day and persisted several months. Our results are comparable to those already obtained in photosensitive psoriasis, which helped us to identify the clinical similarities of photosensitivity in these two instances of dermatosis.

[Cutaneous carcinoma and papillomavirus 5. Discovered during prolonged UVA photochemotherapy].

Introduction: HPV 5 is an opportunistic oncogenic virus found mainly in patients with verruciform epidermodysplasia or in certain immunodepressed patients.

Case: We report the case of a 48-year-old patient who developed, over a 5 year period, 30 skin carcinomas after prolonged puvatreatment. HPV 5 was identified.

[Vulvar lichen sclerosus].

Lichen sclerosus's pathogeny, the most frequent vulvar dystrophy predominant at the start of menopause, is still enigmatic. Its repercussions on the functional level can be disabling. Its clinical sides include atrophy and sclerosis.