J. Castanet

Porphyria cutanea tarda and antibodies to hepatitis C virus

We have studied the prevalence of hepatitis C virus antibodies (anti‐HCV) in 13 patients suffering from sporadic porphyria cutanea tarda. The sera were tested by Abbott second‐generation enzyme immunoassay; seropositivity was confirmed by Ortho second‐generation recombinant immunoblot assay. Ten cases (76.1%) were anti‐HCV positive; one patient was also seropositive for HIV. This preliminary study suggests that HCV could be a frequent triggering factor for sporadic porphyria cutanea tarda.

Follicular mycosis fungoides. A clinical and histologic variant of cutaneous T-cell lymphoma: report of two cases.

We report two cases of mycosis fungoides with marked, pleomorphic follicular manifestations. Follicular hyperkeratosis, comedo-like lesions, acquired epidermal cysts, and patchy alopecia developed in various locations in both patients. Findings of histopathologic and immunohistochemical studies showed atypical CD4+ T lymphocytes infiltrating the follicles without follicular mucinosis. Focal expression of intercellular adhesion molecule type 1 was observed within the cyst walls. These findings suggest that the follicular lesions were specific for mycosis fungoides. These manifestations represent a distinct clinical and histologic form of mycosis fungoides. This variant probably accounts for cases of mycosis fungoides with clinically suspected alopecia mucinosa in which follicular mucinosis cannot be histologically proved.

Interstitial granulomatous drug reaction with a histological pattern of interstitial granulomatous dermatitis

The interstitial granulomatous drug reaction (IGDR) is a novel drug-associated entity, characterized by violaceous plaques with a predilection for skin fold areas. Light microscopically, it resembles the incipient diffuse interstitial phase of granuloma annulare. Differentiating light microscopic features include the absence of complete collagen necrobiosis, the presence of interface dermatitis, and variable lymphoid atypia. The lack of vasculitis rules out the extravascular necrotizing granuloma (Winkelmann granuloma) associated with systemic disease. The differential diagnosis with interstitial granulomatous dermatitis with arthritis as defined by Ackerman et al. has not been studied until now. Our aim was to determine the histologic criteria allowing us to differentiate IGDR without interface dermatitis and lymphoid atypia from interstitial granulomatous dermatitis. We report three patients with IGDR triggered, in two cases by respectively angiotensin convertin enzyme (ACE) inhibitors and furosemide, and in one case by the association of an ACE inhibitor, furosemide, and fluindione. Histologic examination showed a histological pattern of interstitial granulomatous dermatitis. We found a dense, diffuse histiocytic infiltrate distributed interstitially and in palisaded array within the reticular dermis. Eosinophils and some neutrophils were scattered throughout the infiltrate. In some tiny foci, enveloped by histiocytes, thick collagen bundles associated with basophilic nuclear debris or “flame figures” were seen. Vasculitis, interface dermatitis, or lymphoid atypia were absent. Our study allowed us to expand the histological spectrum of IGDR including a histological pattern similar to interstitial granulomatous dermatitis. The lack of degenerated collagen could be a subtle clue in favor of interstitial granulomatous dermatitis triggered by a drug.

Demodicidosis in an Immunodeficient Child

Abstract: A 15‐month‐old girl developed acute lymphoblastic leukemia. Chemotherapy had induced a complete remission and she was continued on maintenance therapy. At 3 years of age, she developed an eruption consisting of excoriated papules and pustules on the face. Demodex folliculorum seemed to be the cause. Topical treatment with metronidazole applied twice a day over a period of 2 weeks resulted in partial improvement. The dermatosis finally cleared gradually with oral erythromycin therapy and one overnight application of 1% lindane cream per week for 2 successive weeks.

Visceral artery aneurysms in Von Recklinghausen's neurofibromatosis

We report the case of a patient with Von Recklinghausens neurofibromatosis in whom two visceral artery aneurysms were diagnosed: a 4 cm aneurysm originating from the common hepatic artery and a smaller aneurysm originating from the superior mesenteric artery. The hepatic artery aneurysm underwent successful embolization. Because of the patients poor general condition, the superior mesenteric aneurysm was considered inoperable and has been kept under surveillance by ultrasonography. Arterial involvement in Von Recklinghausens neurofibromatosis is a well-known but infrequent occurrence. Stenotic lesions predominate, with the renal arteries being the site of predilection. Aneurysmal defects are less common, and involvement of the visceral arteries is exceptional. Only three reports of superior mesenteric artery aneurysm in patients with Von Recklinghausens neurofibromatosis were found in the literature, and hepatic artery aneurysm has never previously been described in this disease.

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.

Cutaneous leukocytoclastic vasculitis and renal cancer: two cases

We report two cases of cutaneous leukocytoclastic vasculitis (CLV) leading to the discovery of an as yet asymptomatic, surgically curable clear cell carcinoma of the kidney. CLV causative factors or associated diseases are usually drugs, infection, or collagen vascular disease, but rarely malignancies. In such cases, these are more often malignant hematologic diseases than solid neoplasms. We believe that in apparently idiopathic CLV, a screening examination should be done to detect any underlying early-stage curable solid malignancy.

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.

Lymphoma developing in a patient with rheumatoid arthritis taking methotrexate

SummaryWe report one case of non-Hodgkin lymphoma in a patient, with a 30-year history of rheumatoid arthritis, taking low dose methotrexate weekly over a 10-month period. The mild immunosuppression that occurs with methotrexate therapy probably places patients with rheumatoid arthritis at added risk of developing lymphoproliferative diseases, but coincidence cannot be excluded.

Subacute cutaneous lupus erythematosus associated with Hodgkin's disease

SummarySimultaneous occurrence of subacute cutaneous lupus erythematosus and malignancy has rarely been reported. We report the first case of subacute cutaneous lupus erythematosus associated with Hodgkins disease. Although our case doesnot fulfill the criteria of paraneoplastic process, a relationship between the two disorders might be suggested by their simultaneous occurrence and in view of the cases of disseminated lupus erythematosus associated with lymphoma reported so far. The different hypotheses are discussed.