Cutaneous findings in sporadic and familial autosomal dominant hyper-IgE syndrome: a retrospective, single-center study of 21 patients diagnosed using molecular analysis.

BACKGROUND: Recent identification of STAT3 mutations in autosomal dominant (AD) hyper-IgE syndrome (HIES) has improved the clinical, genetic, and molecular classification of the HIES. OBJECTIVE: We sought to characterize the cutaneous signs observed in molecularly diagnosed AD-HIES. METHODS: We conducted a retrospective study of 21 patients with AD-HIES and confirmed STAT3 mutations, treated at Necker-Enfants Malades Hospital, Paris, France. RESULTS: A papulopustular rash on the face and scalp before the age of 2 months was observed in 67% of patients. This "early rash" was distinguished from other neonatal pustular eruptions by crusted papules and pustules, rash intensity, and a continuum with chronic dermatitis. An eczematous dermatitis was almost always present before the age of 18 months (95% of patients) and was mainly confined to the face, scalp, chest, and buttocks. All patients presented with infected dermatitis (Staphylococcus aureus) and 59% had chronic candidiasis of the oral mucosa and nails. Cutaneous herpes virus infections were not unusually severe. Coarse facial skin at puberty, and sometimes at a younger age, with prominent follicular ostia resembling atrophoderma vermiculatum was not related to severe acne or facial abscesses. LIMITATIONS: This was a retrospective study with a small number of patients. CONCLUSION: When associated with serum IgE levels 10 times the age-appropriate level, a neonatal papulopustular rash progressing to a chronic impetiginized eczematous dermatitis that differs from classic atopic dermatitis is highly suggestive of AD-HIES. Early recognition is important for initiation of prophylactic antistaphylococcal and antifungal treatment.

Adverse cutaneous drug reactions to ibandronate.

Bisphosphonates are frequently used to treat bone diseases characterized by increased osteoclastic bone resorption. Adverse skin reactions to bisphosphonates are rare and range from benign to severe. Different cutaneous skin reactions have been reported with ibandronate in clinical and pharmacovigilance studies, from macula-papular rashes to toxic epidermal necrolysis. We report two new cases of erythematous and oedematous skin lesions to oral monthly ibandronate, appearing after multiple intakes of the drug. Prick tests were positive in both cases at 48 or 96 hours, and one could be confirmed histologically. Lesions did not relapse after substituting the culprit bisphosphonate with another one. A wide range of rare-to-very-rare adverse skin reactions exist with bisphosphonates, and especially ibandronate. We review the reported cases of adverse cutaneous drug reactions to bisphosphonates and illustrate the polymorphism and variety of the skin lesions. These reactions are not well known and may be misdiagnosed as they do not always suggest drug-induced eruptions. Furthermore, delays between drug intake and the first lesions can be misleading. The absence of cross-reactions among bisphosphonates allows substitution.