title: Diffuse cutaneous bullous mastocytosis with IgM deposits at dermo-epidermal junction.
abstract: Cutaneous mastocytosis is a disease characterized by the infiltration and proliferation of mast cells in the skin. In children, the most common form of presentation is urticaria pigmentosa, while the diffuse cutaneous bullous mastocytosis is one of the rarest subtypes seen. The aim of this paper is to present a case of diffuse bullous mastocytosis with detection of IgM deposits at dermo-epidermal junction using direct immunofluorescence (DIF) microscopy. The diagnosis of diffuse bullous mastocytosis is a challenge, and DIF microscopy is necessary in order to exclude an autoimmune bullous disorder. However, IgM deposits at dermo-epidermal junction can be nonspecific, being found in a variety of skin disorders. A 6-month-old girl presented with bullous lesions and erosions on the scalp and the trunk. During hospitalization, further bullous lesions appeared, along with generalized erythrodermia. Skin biopsy revealed aspects of urticaria pigmentosa. Taking into account the clinical findings, the case was enclosed as bullous mastocytosis. Treatment included the avoidance of trigger factors, and administration of antihistamines along with a short-term course of systemic steroids. The evolution was favorable, with remission of the existing lesions and without occurrence of new ones.
authors.affiliation: Department of Pediatrics II, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
authors.forename: Kinga Cristina
authors.initials: KC
authors.lastname: Slavescu
authors.affiliation: Department of Dermatology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
authors.forename: Roxana
authors.initials: R
authors.lastname: Chiorean
authors.affiliation: Department of Dermatology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
authors.forename: Sorina
authors.identifier: http://orcid.org/0000-0003-0968-4026
authors.initials: S
authors.lastname: Danescu
authors.affiliation: Department of Pediatrics II, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
authors.forename: Madalina
authors.initials: M
authors.lastname: Bota
authors.affiliation: Department of Pathology, Emergency Clinical Hospital, Cluj-Napoca, Romania.
authors.forename: Liliana
authors.initials: L
authors.lastname: Rogojan
authors.affiliation: Department of Dermatology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
authors.forename: Adrian
authors.initials: A
authors.lastname: Baican
mesh_terms: D020405:Dermis / Q000378:metabolism / Q000473:pathology; D004817:Epidermis / Q000378:metabolism / Q000473:pathology; D005260:Female; D006258:Head and Neck Neoplasms / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism*; D007223:Infant; D034701:Mastocytosis, Cutaneous / Q000378:metabolism / Q000473:pathology; D009363:Neoplasm Proteins / Q000378:metabolism*
chemical_list: D007075:Immunoglobulin M; D009363:Neoplasm Proteins
keywords: Darier's sign; IgM deposits; cutaneous bullous mastocytosis in children; direct immunofluorescence microscopy; mast cells