PubMed Health⌕ Search

Biomedical subjects

N Hilty

Publications and source records attributed to N Hilty.

5 recordsLinked to original sources

Mycosis fungoides with mucinosis follicularis in childhood.

Mycosis fungoides is a cutaneous T-cell lymphoma (CTCL) usually observed in mid to late adulthood. It occurs only rarely during childhood. Follicular mucinosis is a chronic dermatosis involving the sebaceous glands and outer root sheaths. It is normally differentiated into a juvenile benign form and an adult form possibly associated with mycosis fungoides. We report a 12-year-old boy who presented with an 8-month history of erythematous mucinous plaques on the scalp. Three months later, he developed erythematous patches and plaques on his whole body, accompanied by cervical lymphadenopathy. A biopsy showed follicular mucinosis and epidermotropism of the lymphocytic infiltrate. Immunophenotyping and a PCR clonality test were consistent with CTCL. The patient received PUVA treatment and local steroids, resulting in partial remission. Mycosis fungoides should be considered in the differential diagnosis of chronic, scaling dermatoses in childhood. Moreover, follicular mucinosis in childhood can be associated with mycosis fungoides.

Child↗

[Scurvy in trisomy 21].

We report a case of Down's syndrome in a 22-year-old woman who developed vitamin C deficiency with subsequent appearance of the characteristic joint pains and cutaneous and mucosal lesions of scurvy. A low intake of vitamin C due to peculiar eating habits and reduced absorption due to cardiac insufficiency and treatment with a platelet-aggregation inhibitor were considered to have caused the deficiency. Follicular purpura is a diagnostic skin sign of scurvy.

Adult↗

[Hypervascularization syndrome of the penis following arterialization of the dorsal penile vein for erectile impotence].

A hypervascularization syndrome following arterialization of the deep dorsal vein of the penis to amend venous erectile impotence is a rare cause of penile ulcers that has not previously been described in the dermatological literature. Arterialization is performed by installing a shunt from the inferior epigastric artery or a venous bypass from the femoral artery to the deep dorsal vein of the penis, resulting in a blockage of venous outflow and in retrograde inflow into the corpora cavernosa. Complications arise as a result of persistently elevated blood pressure in the deep venous system and the erectile tissue in 10-20% of cases and are most often linked to dilatation of the shunt: the consequences are enlargement and induration of the glans, hazard of phimosis and paraphimosis, pulsation of the penis, micturation difficulties and, ultimately, ulceration of the glans. Surgical reduction of the arterial inflow ("banding" of the shunt) is the only therapeutic procedure that reduces hypervascularization without compromising the newly gained erectile function.

Arteriovenous Shunt, Surgical↗