Steatocystoma multiplex generalisata partially suppurativa--case report.
Steatocystoma multiplex is a rare inherited disorder with an autosomal dominant mode of transmission, but sometimes it may appear sporadically. Usually the onset tends to occur during adolescence or early adult life. A clinical case of a 27-year-old male patient is presented. Since the age of 8, he had been presenting with multiple, asymptomatic, round-to-oval, well-defined, smooth-surfaced, yellow to skin-colored, 5- to 22-mm diameter cysts and nodules initially scattered on the trunk and lately disseminated all over the body with less lesions on the lower extremities. At one of follow-up visits, he presented with high fever, pain with tumefaction of the small and medium size joints of the palms and soles, and deteriorated general status with polymorphous skin lesions. Based on the clinical and paraclinical features, the diagnosis of steatocystoma multiplex generalisata partially suppurativa was made. He was treated with oral isotretinoin (1 mg/kg per day) for 14 weeks, antibiotics and local treatments. The lesions healed slowly, with local disfigurement, hyperpigmentation and unpleasant scars. Isotretinoin usually does not eradicate the condition but could be effective in suppurative abscesses. Steatocystoma multiplex generalisata is considered rare; the true incidence of the disease is unknown. In the disease evolution, the severe inflammatory variant, steatocystoma multiplex suppurativa, may appear at any time.
Fekete
Gyula László
GL
Department of Dermatology, University of Medicine and Pharmacy Târgu-Mureş, Bulevardul 1 Decembrie 1918, nr.211/27, Târgu-Mureş, Romania. dermafek@yahoo.com
Fekete
Júlia Edit
JE
D000328:Adult; D006801:Humans; D008297:Male; D053549:Pachyonychia Congenita / Q000150:complications / Q000473:pathology* / Q000628:therapy