Boala de Meleda este o keratodermie palmo-plantara difuza ereditara. Principalele caracteristici sunt: transmiterea autosomal recesiva, progredienta si transgradienta. Boala se manifesta din primele saptamani de viata si avanseaza spre regiunile vecine sau la distanta (coate, genunchi). Microscopia electronica nu deceleaza anomalii majore ale keratinizarii, dar se evidentiaza o incetinire a cornificarii terminale. Tratamentul nu poate decat sa amelioreze. Se folosesc keratolitice locale, agenti emlienti si de hidratare, retinoizi (topic, sistemic).

Prezentam cazul unei femei de 49 de ani internata cu keratodermie palmo-plantara simetrica. Leziunile se extind pe fetele dorsale ale mainilor si picioarelor si spre articulatii (pumn, glezna) avand aspect eritemato-scuamos. La nivelul degetului cinci exista benzi constrictive, ainhumoide. In plus, prezinta modificari unghiale si eritem perioral. Nu exista anomalii ale parului, dintilor sau modificari psihice. Neaga cosangvinitate in familie. Singura sora prezinta acelasi tip de leziuni. S-a folosit un tratament local simptomatic cu hidratante si keratolitice (uree, acid salicilic). Nu au fost administrati retinoizi, desi efectul lor spectaculos asupra hiperkeratozei este cunoscut, deoarece s-au luat in consideratie necesitatea administrarii indelungate, posibilitatea efectului de rebound la intrerupere si reactiile adverse asupra metabolismului lipidic (au fost decelate hiperlipemie si hipercolesterolemie).

Comentarii. Cazul de fata permite trecerea in revista a principalelor keratodermii palmo-plantare ereditare.

Mal de Meleda is a recessive, transgressive palmoplantar keratoderma. The disease appears in the very first weeks of life and progresses to the adjacent parts or at distance (elbows, knees). There are no major anomalies of keratinization, but the electronic microscopy shows a slowing down of terminal cornification. The treatment can only improve. Use topical keratolytic agents, hydration agents, retinoids (topically or sistemically).

We report a case of a 49 years old woman hospitalized for symmetric palms' and soles' lesions with diffuse, thick, yellow, keratotic deposits. The lesions with erythema and scaling progress to the dorsa of the hands and feet and to the joints (ankle, fist). The fingers are moderate fixed in flexion. There are some ainhum-like constrictions at the 5th fingers. Also presents nail changes and perioral erythema. There are no anomalies of teeth, hair or psychic changes. She denies cosangvinity. The only sister has the same type of lesions. It has been used a local keratolytic and hydrating treatment (urea and salicilic acid). Retinoids have not been used because of the necessity of a long-term administration, rebound phenomena and adverse reactions on the lipidic metabolism (she had high levels of serum cholesterol and lipids).

Commentaries: this case permit us a review of the inherited palmo-plantar keratodermas.