Bullous Mastocytosis Mimicking Congenital Epidermolysis Bullosa

Abstract

A 2-month-old female infant was referred to DebRA Mexico from the Regional Children's Hospital because of a generalized dermatosis from birth characterized by multiple blisters and erosions on the trunk, face and limbs, associated with minor trauma. A skin biopsy showing subepidermal blisters associated with a dermal infiltrate of Giemsa-positive cells and CD117-positive antibody was consistent with the diagnosis of bullous mastocytosis. Treatment with oral antihistamines, topical steroids, and antibiotics was initiated, leading to a remission of the lesions.

An infant with diffuse bullous lesions

“…When it presents with widespread bullae, DCM has a relatively poor prognosis and may be associated with life-threatening complications (flushing, anaphylactic shock, abdominal pain and gastrointestinal bleeding) 1. Therefore, it is important to differentiate from other bullous skin disorders observed in infants (bullous impetigo, epidermolysis bullosa4 and contact dermatitis) by skin biopsy.…”

Bullae on the extremities of a newborn: a case of diffuse cutaneous mastocytosis mimicking epidermolysis bullosa

“…2 About 17-25% of elderly psoriatic patients suffer from a moderate-to-severe disease, requiring a systemic treatment [2][3][4] which is often impeded due to contraindications, drug interactions and adverse events (AEs). 4 Recent real-life studies have provided data on biologics efficacy and safety in elderly psoriasis patients 5,6 with an overview survey also underling the potential of apremilast, an oral small-molecule phosphodiesterase 4 inhibitor. 7 However, real-life data on apremilast profile in elderly patients are lacking even if this drug is particularly useful in this complicated class of patients; thanks to its high-safety profile (latent tuberculosis infection, hepatitis and cancers do not represent contraindications).…”

“…Our case appeared to be the large bullous variant. Because of the rarity of DCM and a lack of typical maculopapular lesions, differentiating bullous DCM from other bullous diseases is difficult [5][6][7] . However, differentiating them is important because DCM may have a risk of shock and organ involvement.…”

A female newborn with papulovesicular lesions

“…Eine EB kam aufgrund der betroffenen Hautareale nicht in Betracht: In unserem Fall folgten die Läsionen den Blaschko-Linien, während bei der EB die am stärksten betroffenen Areale Kopf, Hände und Füße gleichzeitig auch die verletzungsanfälligsten sind [ 3 ] . Aus denselben Gründen konnten wir auch eine bullöse Mastozytose ausschließen, eine seltene Variante einer Mastzellerkrankung, die gelegentlich bei Neugeborenen beobachtet wird [ 4 ] . Die Läsionen entwickelten sich ab dem dritten Monat zu hyperpigmentierten Maculae und entsprachen so einem wichtigen Diagnosekriterium der IP [5][6][7] mit einem typischen, neonatalen, vesikulären Ausschlag.…”