C. Perrin

Giant oral aphthous ulcers induced by nicorandil.

tests, liver function tests and ultrasound of the abdomen were normal or negative. Quantitative analysis of porphyrins in urine (separated into fractions of uroporphyrins, coproporphyrins and carboxylic porphyrins) was performed by standard methods. Porphyrin levels were normal. Phototests and photopatch tests performed as described by Fujimoto et al. were negative. The histopathological features of a blister on the back of the hand were characterized as a subepidermal blister with denuded dermal papillae protruding into the blister cavity. Only occasional lymphocytes were seen in the dermis. Direct immunofluorescence was repeatedly negative. A diagnosis of pseudoporphyria associated with antiandrogen therapy was considered. Flutamide was replaced with bicalutamide, a new androgen-receptor antagonist, 50 mg/ day, in addition to goserelin, leading to permanent healing of the lesions. Pseudoporphyria is a condition characterized by clinical changes resembling PCT, in the absence of any demonstrable abnormality in porphyrin metabolism. It may be associated with several drugs, whose withdrawal is often curative. Differential diagnosis includes epidermolysis bullosa acquisita, in which direct immunofluorescence is positive for IgG deposits at the dermal– epidermal junction in perilesional skin. In contrast, direct immunofluorescence is not specific in pseudoporphyria, with occasional positivity around the blood vessels, as in PCT. Flutamide is a non-steroidal derivative of toluidine which blocks the action of both testosterone and dihydrotestosterone bycompeting with androgen receptors. The antiandrogenactivity of flutamide probably depends on conversion to 2-hydroxyflutamide in the liver. Cutaneous side-effects have rarely been described, with the exception of two reports of photosensitive dermatitis. In our patient, phototests and photopatch tests were negative. Replacement of flutamide with bicalutamide led to permanent healing. The mechanism of flutamide in inducing blisters is unknown, but it is well-known that cutaneous lesions of pseudoporphyria appear to be precipitated or aggravated by exposure to sunlight. A relevant factor in this case, as in the case with naproxen described by Burns, is that blisters developed only in areas of vitiligo on the dorsa of the hands, while normal skin was not affected. The absorption spectrum of flutamide has been reported to be 292 nm. The presence of melanin in normal skin may thus be sufficient to prevent flutamide-induced pseudoporphyria.

FZD6 encoding the Wnt receptor frizzled 6 is mutated in autosomal-recessive nail dysplasia

Background  Isolated nail dysplasia is rare and has been reported in only a small number of families.

Pseudo-dermatomyositis induced by long-term hydroxyurea therapy: report of two cases.

1 Tsui |KC, Hisen A. Stocssl A] el al. Double-blind study of hotulinum loxin in spasmodic torticollis. lMiicel 1986: ii: 245-7. 2 (Sirliinda 1*. Vita (.!. NJcolosi C el iiL Botulinum toxin therapy: distant effects on neuromuscular transmission. / Neurol Neursurg Fsfichiatrij 1992: S5:K44-5. J Sthnider P. Bricbta A, Schmied M, Auff E. Gallbladder dysfunction induced hy botulinum A toxin. iMiiret 1993: 342: 811-12 (letter). 4 iiushara KO. Park DM. Botulinum roxin and sweating. / Neurol FsfichitUrn 1994: 57: 14i7-S.

Acral hyperpigmented macules and longitudinal melanonychia in AIDS patients

Two patients with AIDS are described who developed acral hyperpigmented macules of the fingers, palms and soles, buccal mucosa and genitalia, associated with longitudinal melanonychia. These pigmentary changes seemed to be independent of zidovudine and were associated in one with diffuse melanoderma and elevated levels of α‐MSH. Histological and ultrastructural studies showed an increase of the dendrites and pigmentation of the melanocytes and few melanosomes in the keratinocytes.

Juvenile pityriasis rubra pilaris associated with hypogammaglobulinaemia and furunculosis

We report a case of follicular keratosis with inflammatory changes, consistent with a diagnosis of atypical juvenile pityriasis rubra pilaris. An unusual feature was the occurrence of severe Staphylococcus aureus folliculitis and furunculosis, a phenomenon rarely encountered in pityriasis rubra pilaris and the other follicular keratoses. Standard antibiotic and antiseptic treatment for chronic S. aureus infection was ineffective. The patient was subsequently found to have hypogammaglobulinaemia, and treatment with human polyvalent immunoglobulin infusions was successful in eradicating the sepsis. It is therefore probable that the hypogammaglobulinaemia played a pathogenic role in the development of cutaneous sepsis.

Altered expression of the hemidesmosome‐anchoring filament complex proteins in basal cell carcinoma: possible role in the origin of peritumoral lacunae

Basal cell carcinoma (BCC) is a frequent skin cancer with low metastalic potential. Expression of the anchoring filament proteins, native laminin‐5 and its individual α3, β3 and γ2 chains, uncein, and linear IgA antigen was examined by immunostaining in 17 BCC with different histological subtypes. Immunoreactivity of the hemidesmosomal proteins, integrin α6β4. 230‐kDa bullous pemphigoid antigen (BP‐230 Ag) and plectin/HD‐1, and that of dermal‐epidermal junction (DEJ) components, integrin α2β1, laminin‐1, collagen IV, and collagen VII was also analysed. Around tumour nests, the labelling of laminin‐5 was absent or markedly reduced in 12 BCC (comprising eight solid BCC, three adenoid BCC and one keratotic BCC) and strong in five BCC (comprising three adenoid BCC, one keratotic BCC and one adenoid and keratotic BCC). Intriguingly, in tumour cells of 12 BCC including laminin‐5 negative tumours, a cytoplasmic reactivity of the laminin 72 chain was detected, but not that of the α3 and β3 chains. In the basement membrane of the epidermis overlying tumour nests, the labelling of laminin‐5 was always strong. Uncein, linear IgA disease antigen, and integrin α6β4 were absent in solid BCC and weakly expressed in adenoid or keratotic BCC. For plectin/HD‐1 and BP‐230 Ag, a cytoplasmic reactivity was detected in the majority of the tumour cells. The labelling of integrin α2β1, laminin‐1, collagen IV and collagen VII indicated no alteration in the synthesis of these proteins. In peritumoral lacunae, immunoreactivity of hemidesmosome and anchoring filament proteins was absent, except for plectin/HD‐1 on the tumour side and sometimes for laminin‐5 on the stromal side, while laminin‐1, collagen IV and collagen VII were detected on the stromal side. These findings suggest that the components of the hemidesmosome‐anchoring filament complex are not synthetized or assembled properly in BCC, and that the alteration of these adhesion structures may be the cause of peritumoral lacunae.

Blastic plasmacytoid dendritic cell neoplasm: a report of four cases and review of the literature.

Background  Blastic plasmacytoid dendritic cell neoplasm (BPDCN), formerly known as agranular CD4+/CD56+ haematodermic neoplasm (CD4/CD56 HN), is a rare distinct form of lymphoma‐like entity known of dermatologists because of its marked predilection for cutaneous involvement, and its aggressive behaviour. Moreover, the association or the evolution to an acute leukaemia entity that still expresses CD4 and CD56 markers is almost systematic. This new described entity of ‘CD4+/CD56+ leukaemia’ or ‘leukaemia of plasmacytoid dendritic cell lineage’ has a poor prognostic and may lead to include haematopoietic stem cell transplantation in the treatment strategy as early as possible.

Traitement du mycosis fongoïde par le laser excimer à 308 nm : étude anatomo-clinique chez dix malades

Resume Introduction Le mycosis fongoide est un lymphome cutane T epidermotrope. Les stades precoces ont un bon pronostic mais posent des problemes therapeutiques par leur chronicite et les recidives frequentes. Nous avons realise une etude retrospective anatomo-clinique sur 10 malades atteints de mycosis fongoide afin d’evaluer l’efficacite du laser excimer a 308 nm dans cette indication. Methodes Dix malades dont le diagnostic de mycosis fongoide avait ete confirme histologiquement, ont ete inclus dans l’etude. Un traitement par laser excimer a 308 nm a ete realise sur un total de 29 lesions dont 25 lesions peu ou pas infiltrees, trois plaques infiltrees et un nodule. Apres determination de la dose erythemateuse minimale, les seances ont ete debutees au rythme de deux par semaine avec une augmentation progressive des doses en fonction de la tolerance. Un suivi clinique a ete ensuite realise. Des biopsies cutanees ont ete realisees, lorsque cela etait possible, avant, a la fin et a distance du traitement afin de realiser une etude histologique et immuno-histochimique. Resultats Quatre-vingt-six pour cent des lesions peu ou pas infiltrees ou en plaques etaient en remission complete a la fin du traitement et 14 p. 100 en remission partielle avec un taux global de reponse de 100 p. 100. Le nombre moyen de seances a ete de 15 (6 a 46 seances), soit une duree moyenne de traitement de 2 mois, et la dose cumulee de 5 J/cm 2 (1,3 a 16,1 J/cm 2 ). Dix-neuf lesions ont pu etre suivies a distance du traitement avec une duree mediane de suivi de 15 mois (8 a 26 mois). Une reponse complete s’est maintenue pour 13 lesions (12 lesions peu ou pas infiltrees et une plaque) sur 19, soit 68 p. 100. Une reponse partielle s’est maintenue pour trois lesions (une lesion peu ou pas infiltree et deux plaques) sur 19, soit 16 p. 100. Une rechute est survenue pour deux lesions (deux lesions peu ou pas infiltrees) sur 19, soit 11 p. 100. Ces deux rechutes ont toutes deux ete observees a 7 mois. Le seul nodule traite n’a pas repondu au traitement. Les resultats histologiques et immuno-histochimiques concordaient avec l’aspect clinique montrant une guerison histologique en cas de guerison clinique hormis un cas qui a montre la persistance de quelques cellules de type mycosis en exocytose alors que la lesion paraissait guerie cliniquement. Discussion Cette etude montre l’efficacite du laser excimer dans les mycosis fongoides au stade Ia. Compte tenu de la faible disponibilite de ce traitement et de son caractere onereux, le laser excimer a 308 nm reste indique en seconde intention apres echec ou recidive d’un traitement par dermocorticoides bien conduit.

Efficacité des immunoglobulines intraveineuses dans le traitement d’une mucinose papuleuse généralisée

BACKGROUND Scleromyxoedema is a rare disorder of unknown pathogenesis that is very difficult to treat. We report a case resistant to corticosteroid treatment but controlled by intravenous gammaglobulin (IVIG). CASE REPORT A 60-year-old woman presented progressive generalized papular eruption with infiltrated and itchy lesions of between 2 and 5mm in diameter. Otherwise, the clinical examination was normal. Monoclonal gammopathy of the IgG lambda type was found. Histology confirmed the diagnosis of scleromyxoedema. The disease continued to progress despite oral corticosteroids (0.5mg/kg per day). Thalidomide was introduced but was discontinued after 2 months due to side effects. Treatment comprising six monthly infusions of IVIG (2g/kg on 5 days) resulted in a marked reduction (>50%) in lesions. Two months after discontinuation of IVIG, recurrence was observed and maintenance infusions of IVIG every 6 weeks were needed to control the disease. DISCUSSION The course of scleromyxoedema is unpredictable and treatment is extremely difficult. Successful therapy with IGIV has been reported but this approach seems to afford only temporary relief and maintenance infusions are required, as confirmed by the initial efficacy of treatment in our patient and the rapid recurrence of lesions following withdrawal.