Muckle-Wells syndrome in the setting of basal cell nevus syndrome.

Muckle-Wells syndrome (MWS) is a rare disorder inherited in an autosomal-dominant fashion that belongs to a group of hereditary periodic fever syndromes. It specifically belongs to the cryopyrin-associated periodic syndromes (CAPSs) in which there is a mutation in the NLRP3 (NLR family pyrin domain containing 3) gene that leads to overproduction of IL-1ß, the source of the multisystem inflammatory symptoms. Muckle-Wells syndrome is characterized by a recurrent urticarial eruption that is associated with episodic fever, myalgia, arthralgia, malaise, progressive sensorineural hearing loss, and amyloid nephropathy (the most severe complication). Basal cell nevus syndrome (BCNS), or Gorlin syndrome, is a rare, autosomal-dominant inherited genodermatosis linked to a mutation in the PTCH1 (patched 1) gene and is characterized by a broad range of anomalies. We report the case of a patient with MWS and BCNS in whom basal cell carcinoma (BCC) treatment was complicated by symptoms of MWS.

Eruptive Kaposi sarcoma: an unusual presentation in an HIV-negative patient.

Kaposi sarcoma (KS) is an angioproliferative disease associated with human herpes virus 8 (HHV-8) infection. Four clinical variants have been described, all unified by common histopathologic features. We report a case of KS in an elderly Italian man with an unusual clinical presentation characterized by a relatively rapid onset of lesions and an unexplained low CD4 lymphocyte count. We review KS, including the 4 recognized variants, the central histopathologic findings, and various treatment options. In addition, we briefly discuss causes of CD4 lymphocytopenia, including a cursory overview of idiopathic CD4 lymphocytopenia (ICL).