The most severe forms of cutaneous drug reactions are Stevens-Johnson SJSdrome (SJS) and toxic epidermal necrolysis (TEN).
CAPS comprises a spectrum of disease from the mildest form, familial cold autoinflammatory syndrome (FCAS), through Muckle-Wells syndrome (MWS), to the most severe form, chronic infantile neurologic cutaneous and articular syndrome (CINCA), also known as neonatal-onset multisystem inflammatory disease (NOMID).
It was, however, the significantly most frequently used drug in the subset associated with the most severe disease course (that is, diffuse cutaneous SSc).
CADR may progress to severe cutaneous adverse reactions (SCAR) if the offending drug (most often an antimicrobial) is not stopped promptly, whereas TEC often requires only supportive treatment and will resolve spontaneously.
The disease manifests in different clinical forms, ranging from cutaneous leishmaniasis (CL) to disfiguring mucosal lesions to the most severe form, visceral leishmaniasis (VL).
Cryopyrin-associated periodic syndrome (CAPS) is a group of rare autoinflammatory diseases, and of these, chronic infantile neurologic, cutaneous, and articular/neonatal-onset multisystem inflammatory disease (CINCA/NOMID) syndrome has the most severe phenotype.
One employee of American Media has died of what appears to be inhalation anthrax, the most severe form of the disease, and an assistant to NBC anchor Tom Brokaw had the milder, cutaneous form.
The mildest condition is the familial cold auto-inflammatory syndrome, patients with Muckle Wells syndrome have an intermediate phenotype, the chronic infantile neurological cutaneous and articular syndrome (CINCA) also known as neonatal onset multisystem inflammatory disease (NOMID) is the most severe disease in this spectrum [1, 2].
