ABSTRACT
IMPORTANCE: In the literature, patients with bullous pemphigoid have been reported to have itch without blisters. Clinical observations in these patients have varied from eczematous or urticarial to papular or nodular skin lesions. Here we investigated the spectrum of clinical variants. OBSERVATIONS: Fifteen patients with itch without blisters had immunopathologic findings of bullous pemphigoid. Mean age at diagnosis was 81.7 years. No blistering occurred during the mean 2.2 years of follow-up. Mean delay of diagnosis was 2.8 years. Clinical symptoms were heterogeneous: pruritus sine materia (no primary skin lesions), eczematous, urticarial, papular, and/or nodular skin lesions were seen. Treatment with potent topical corticosteroids or methotrexate sodium led to remission in 11 patients. CONCLUSIONS AND RELEVANCE: Itch without skin lesions can be the only symptom of bullous pemphigoid. Therefore, it is important to include serologic and direct immunofluorescence in the diagnostic algorithm of itch. We propose the unifying term pruritic nonbullous pemphigoid for all patients with immunopathologic findings of bullous pemphigoid, itch, and no blisters.
Subject(s)
Glucocorticoids/therapeutic use , Methotrexate/therapeutic use , Pemphigoid, Bullous/diagnosis , Pruritus/etiology , Administration, Cutaneous , Adult , Age Factors , Aged , Aged, 80 and over , Delayed Diagnosis , Dermatologic Agents/administration & dosage , Dermatologic Agents/therapeutic use , Female , Follow-Up Studies , Glucocorticoids/administration & dosage , Humans , Male , Methotrexate/administration & dosage , Middle Aged , Pemphigoid, Bullous/pathology , Remission Induction/methods , Time Factors , Treatment OutcomeABSTRACT
We report a case of a 26 year old woman with rash, lymphadenopathy, liver enzyme abnormalities and spiking fever. She was diagnosed with drug-induced hypersensitivity syndrome (DHS) to lamotrigine. Spiking fever in relation to drug-induced hypersensitivity syndrome has not earlier been described in adults. Spiking fever is an important symptom of the wide spectrum of disease presentation. The syndrome is commonly referred to as either Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) or DHS. In accord with previous authors we see both syndromes as two ends of a spectrum, with a wide range of symptoms and presentations. Therefore we plea for unity in nomenclature.
