Neonatal bullous mastocytosis

I am reporting a 17-day old female neonate attended in an emergency with a history of generalized multiple bullous lesions all over the body with spontaneous ruptures of these bullous lesions resulting in erosions and healing with dark pigmentations. Provisional clinical diagnoses were bullous mastocytosis or congenital syphilis. Hematological investigations were against the diagnosis of congenital syphilis. The skin lesion’s biopsy with an array of histopathological staining and immunohistochemical typing confirmed the diagnosis of diffuse cutaneous erythrodermic mastocytosis. The attending clinician should know the process of evoking bullous mastocytosis in front of pruritic blisters associated with dermographism. Enlarged bullous form of the disease should be considered an emergency and dealt with urgency.

Bullous Mastocytosis in a Filipino Infant: A Case Study

Introduction. Mastocytosis is a disease defined by the proliferation of mast cells in organs, most commonly the skin. It may affect any age group but is usually found in children in the first year of life. We present a case of diffuse cutaneous mastocytosis manifesting in the rare bullous form. Case. A 4-month-old Filipino male presented with multiple bullae on the head, trunk, and extremities after applying chamomile oil. Biopsy of the skin demonstrated numerous mast cells, confirming the diagnosis of bullous mastocytosis. The patient was treated with oral antihistamines and corticosteroids, which was followed by a good response. Conclusion. Diagnosis of diffuse cutaneous mastocytosis may be challenging due to its rarity. Proper management requires preventive measures, symptomatic treatment, as well as communication of prognosis with the stakeholders.

Bullous Mastocytosis In Infants: A Case Report

Cutaneous mastocytosis is one of the diseases that will be considered in the differential diagnosis of patients presenting with urticarial plaques and common bullae. Its differential diagnosis should be made among other potential conditions such as bullous impetigo, bullous pemphigoid or eczematous eruptions accompanying immune deficiencies. The close follow up of evolution of specific lesions with detailed anamnesis, and physical examination the diagnosis of cutanous mastocytosis should be considered in priority. Detection of Darier’s sign is also helpful during examination. Definitive diagnosis can only be established after performing a skin biopsy. Here, we present a 2.5 month-old infant with cutanous mastocytosis and discuss differential diagnosis and therapeutic features in light of current literature.

Diffuse Cutaneous Bullous Mastocytosis and Disseminated Intravascular Coagulation Postvaccination

Background: Diffuse cutaneous bullous mastocytosis is the most rare subtype of cutaneous mastocytosis, characterized by generalized skin infiltration with mast cells and blistering. Objective: To increase the awareness of the natural history and potential adverse complications of this rare cutaneous condition. Method and Results: We report a case of a male diagnosed on day 7 of life with follow-up of his progression over 6 years. When he was 2.5 months old, he was admitted to hospital postvaccinations with a flare of his blistering that was complicated by disseminated intravascular coagulation and polyuric acute tubular necrosis. Blistering ceased at 3 years, but at 6 years, extensive urtication continued in response to known triggers and a suboptimal dose of mast cell membrane stabilizers and histamine-1 and -2 receptor antagonists. Conclusion: This case discusses the progression of this rare condition over 6 years and highlights the importance of reaching optimal pharmacologic blockage of histamine-1 and -2 receptors and stabilization of mast cell membranes in patients persistently experiencing ongoing pruritus, urtication, and flushing symptoms.

Indirect Immunofluorescence for the Detection of Autoimmune Urticaria

Background: An autoimmune basis is believed to be responsible for about half of chronic spontaneous urticaria (CSU) cases. The autologous serum skin test is used as a possible indicator, but there is currently no test that directly indicates an autoimmune etiology. In this study, an indirect immunofluorescence was used to identify patients with autoantibodies directed at mast cells. Methods: Two substrates were used including paraffin embedded sections of skin biopsies from an infant with bullous mastocytosis and cord blood–derived mast cells (CBMC). Sera from 76 patients with CSU were incubated with substrates and conjugated with human IgG. Results: Using the bullous mastocytosis preparations, positive indirect immunofluorescence was found in 46% (n = 76), while the CBMC substrate was positive in 39% (n = 70). Conclusion: The IgG autoantibodies directed at mast cells could be detected in about half the patients with CSU. Indirect immunofluorescence should be considered as an indicator of the autoimmune form of CSU.