ABSTRACT
The spectrum of Stevens-Johnson syndrome/toxic epidermal necrolysis (TEN) is the most severe form of cutaneous adverse reactions to drugs. We report a case of a HIV-positive man with TEN who presented a very good response to a single dose of intravenous immunoglobulins and a short pulse of corticosteroids, together with intensive supportive care. Although the largest study on the management of this type of patients reported to date suggests a scheme of three doses of intravenous immunoglobulins together with glucocorticoids, we implemented a single dose of immunoglobulins due to lack of availability.
Subject(s)
Adrenal Cortex Hormones/administration & dosage , HIV Infections/complications , Immunoglobulins, Intravenous/administration & dosage , Stevens-Johnson Syndrome/drug therapy , Adrenal Cortex Hormones/adverse effects , Drug-Related Side Effects and Adverse Reactions , Humans , Immunoglobulins, Intravenous/adverse effects , Male , Middle Aged , Stevens-Johnson Syndrome/etiologyABSTRACT
Porokeratosis is a heterogeneous group of dermatoses with alterations of keratinization. Histologically, they are characterized by the presence of cornoid lamellae. Eruptive pruritic papular porokeratosis (EPPP) or the inflammatory form of disseminated superficial porokeratosis (or eruptive disseminated porokeratosis) is an infrequent variant, characterized by pruritic erythematous papules or annular lesions. We present a 72-year-old woman with EPPP, exhibited by pruritic lesions on the extremities and back, and review the literature concerning this condition. We found 32 cases of EPPP or inflammatory disseminated superficial porokeratosis (including the current case) reported in the literature, with a median age of 66 years (range, 13-84); 59.3% were men. Eruptive pruritic papular porokeratosis was associated with various neoplasms in 31.2% of cases. Six patients had an associated viral infection. Response to treatment was poor in most cases. Eruptive pruritic papular porokeratosis resolved spontaneously in 75% of cases. Median time to resolution was 6 months (range, 1-24). Eruptive pruritic papular porokeratosis (or inflammatory disseminated superficial porokeratosis/eruptive disseminated porokeratosis) is an infrequent variant of porokeratosis characterized by intense pruritus and spontaneous resolution in most individuals. Eruptive pruritic papular porokeratosis can be associated with neoplasms and screening for malignancies is recommended if clinically indicated.
