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Non-Commercial Use Only Dermatology Reports 2021; volume 13:9021 Diffuse cutaneous mastocytosis urticaria pigmentosa, diffuse cutaneous masquerading as linear IgA mastocytosis (DCM), mastocytoma, Correspondence: Tuntas Rayinda, Department and teleangiectacia macularis eruptive of Dermatology and Venereology, Faculty of bullous dermatosis of child- Medicine, Public Health, and Nursing, perstans.4,5 Diffuse cutaneous mastocy- hood Universitas Gadjah Mada, Yogyakarta, tosis is rare form of pediatric mastocy- Indonesia. tosis, and can manifest as an eruption E-mail: [email protected] Tuntas Rayinda, Dyah Ayu Mira of papules, erythematous plaques, bul- Oktarina, Retno Danarti lae, and erythroderma followed by skin Key words: cutaneous mastocytosis, bullous Department of Dermatology and 6 mastocytosis, chronic bullous disease of child- thickening and pigmentation changes. hood, linear IgA dermatosis of childhood. Venereology, Faculty of Medicine, Public The diagnosis of DCM in children, Health and Nursing, Universitas Gadjah especially those presenting as bullous Acknowledgements: Authors would like to Mada, Yogyakarta, Indonesia eruption (namely, diffuse bullous cuta- thank the staff in Klinik Bahasa for the proof- neous mastocytosis), is a challenge for reading and language editing. clinicians and symptoms can mimic Contributions: The authors contributed equally. Abstract other bullous eruptions. Other than DCM, the bullous eruption in infants Conflict of interest: The authors declare no Diffuse cutaneous mastocytosis is a potential conflict of interest. rare form of cutaneous mastocytosis and children can also develop in either that can appear in heterogeneous clini- acquired or inherited bullous disorders, Funding: None. cal presentations, including eruption of such as linear IgA bullous dermatosis in papules, erythematous plaques, blisters, childhood (LABD), staphylococcal Ethics approval: Approved. scalded skin syndrome, juvenile bul- and erythroderma. We report a 1.5- Consent to publication: Received. year-old boy who presented with itchy lous pemphigoid, and epidermolysis only wheals and blisters spreading on his bullosa simplex. Availability of data and materials: Available body. The patient was initially man- We present a DCM case in a child from the authors. aged as a linear IgA bullous dermatosis initially managed as LABD because of Please cite this article as: Rayinda T, of childhood (LABD) because of the its similar clinical symptoms and fea- tures of linear IgA deposits on the base-useOktarina DAM, Danarti R. Diffuse cutaneous similarity of clinical symptoms and the mastocytosis masquerading as linear IgA bul- presenting of linear IgA deposits at the ment membrane in direct immunofluo- lous dermatosis of childhood. Dermatol Rep basement membrane. Due to the devel- rescence (DIF) examinations. This case 2021;13:9021. opment of urticarial plaque after the report emphasizes the importance of comprehensive clinical, histological Received for publication: 21 November 2020. resolution of the blisters, the diagnosis Revision received: 18 January 2021. of diffuse cutaneous mastocytosis was and DIF examinations in managing Accepted for publication: 20 January 2021. made based on clinical, histopathologi- children with acquired bullous erup- cal (hematoxylin-eosin, Giemsa, and tion. Moreover, clinicians should con- This work is licensed under a Creative sider DCM as one of the differential Commons Attribution-NonCommercial 4.0 toluidine blue staining), and direct International License (CC BY-NC 4.0). immunofluorescent examinations (IgA, diagnoses of infants or children pre- IgG, IgM, C3). The symptoms were senting with bullous lesions. ©Copyright: the Author(s), 2021 improved following antihistamines and Licensee PAGEPress, Italy Dermatology Reports 2021; 13:9021 oral corticosteroid treatment. doi:10.4081/dr.2021.9021 Case Report Non-commercial urticaria with unknown causes. There Introduction A 1.5-year-old boy was brought to was no family history with a similar Mastocytosis is a group of diseases our center with blisters on his face and condition. with clinical symptoms caused by mas- body, starting three days before the Dermatological examination sive infiltration of mast cells in various admission. Six days earlier, the patient revealed tense blisters with clear fluid tissues, including skin, blood, gastroin- had fever, cough, and runny nose. The in various sizes scattered on the face testinal, cardiovascular, and muscu- patient was treated with paracetamol and back. Several skin-colored papules loskeletal systems.1 Nettleship first and amoxicillin for two days by a gen- and skin erosions were also found on described the disease entity in 1889 as eral practitioner (GP). After the fever his body (Figure 1). Nikolsky’s sign, a rare form of urticaria.2 Mastocytosis receded, flaccid blisters developed on Darier sign, and dermographism were has a prevalence of 1 in 10,000 popula- his face and body. negative during examination. Neither tion, and the incidence ranges from 5 to In past medical history, the patient lymph node enlargement nor 10 cases per million individuals per had experienced a similar complaint hepatosplenomegaly were found. year in the United States.3 one month before. At that time, the Based on clinical examination, the Cutaneous mastocytosis is the most complaint was not treated and he self- working diagnosis established at that common form of mastocytosis in chil- improved within two weeks. The time was LABD, and the therapy given dren and has several forms, including patient also had a history of recurrent was normal saline (0.9% sodium chlo- [page 4] [Dermatology Reports 2021; 13:9021] Case Report ride) gauze dressing on the top of bul- lae followed by silver sulfadiazine 1% cream applied twice daily to areas experiencing erosion. At this appoint- ment, skin biopsy was considered, but the parents refused. Four months later, the patient came back with the appear- ance of rashes that felt very itchy. The lesions had emerged on the chest and spread to the back and extremities. At this appointment, no new blisters had developed. Dermatology examination revealed wheals and hyperpigmented patches, distributed on the face, chest, back, and abdomen (Figure 1). The patient was still managed as LABD and was given methylprednisolone 4 mg tablets twice daily for seven days and cetirizine 2.5 mg daily. Two weeks later, the symptoms were improved, but there were still new Figure 1. Clinical picture of the patient at initial visit: blisters and erosion distributed on erythematous wheals on the back, face, the trunk (A), and the development of new urticarial plaques after blisters resolution (B). and chest. During the examination, only use Non-commercial Figure 2. Histopathology staining with hematoxylin-eosin (A1, A2), Toluidine blue (B1, B2), and Giemsa (C1, C2), showed massive infiltration of mast cells in the epidermis and dermis. [Dermatology Reports 2021; 13:9021] [page 5] Case Report more erythematous patches and wheals linear IgA deposits the basement mem- mature mast cells and triggers the were found with hyperpigmented brane. The staining for IgM, IgG, and release of mast cells’ mediators. In patches distributed on the face, chest, C3 were negative (Figure 3). about 60% to 80% of mastocytosis back, and abdomen. There were no Based on the clinical and support- cases, somatic mutations of the gene signs of Darier nor dermographism. At ing examination, the diagnosis of cuta- encoding the Kit protein cause this point, the parents agreed to give neous mastocytosis was made in this autocrine dysregulation and activation consent for the skin biopsy procedure patient. The therapy given was ceti- of Kit in the absence of the SCF for the patient. rizine 2.5 mg per day and topical mois- ligand.7,8 The excessive activation of Histopathological examination with turizer. Two weeks later, there were no Kit protein leads to the involvement of hematoxylin-eosin (HE) staining from new lesions had developed, and the internal organs other than the skin.7 urticarial lesions taken from the back treatment was continued with the appli- Most of the patients with cutaneous area showed basket weave-type ortho- cation of topical moisturizer twice mastocytosis do not meet all of the cri- keratosis, spongiosis, acanthosis, and daily. However, new blisters reap- teria for systemic mastocytosis, in elongation of rete-ridge in the epider- peared one week later. The patient went which there is a massive infiltration of mis. Infiltration of inflammatory cells, to a GP and received oral prednisone mast cell in various organs, including consisting of lymphocytes, a few 2.5 mg per day for one month. Up to the bone marrow and liver. Although eosinophils, histiocytes, and a large two weeks after the patient stopped tak- internal organ involvement is rare, number of mast cells, was found in the ing oral prednisone, there was no any cutaneous mastocytosis is often accom- upper dermis, perivascular, and sur- sign of relapse. panied by gastrointestinal symptoms rounding the skin appendages. Neither and anaphylaxis.7 We did not find any a subepidermal cleft nor any neutrophil sign of internal organ involvement in were seen. Additional staining using Discussion and Conclusions our case. Giemsa and toluidine blue showed a Skinonly biopsy is crucial in diagnosing Cutaneous mastocytosis is a disease large number of mast cells in the der- cutaneous mastocytosis. caused by the release of mast cell medi- mis layer (Figure 2). These findings Histopathological examination of
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