Cutaneous mastocytosis, problems of clinical diagnosis of four cases

Vol. 55 No. 3 Suppl., 2014
This supplement was not sponsored by Outside Organizations.

ROMANIAN JOURNAL of MORPHOLOGY and EMBRYOLOGY

Virgil Patrascu, Andreea-Oana Enache, Raluca Niculina Ciurea, Corneliu Cristian Georgescu, Alina Maria Vilcea, Loredana Elena Stoica, Marius Eugen Ciurea

Mastocytosis is a rare disease characterized by a pathological increased of mast cells in one or more tissues, particularly in the skin, bone marrow, liver, spleen, lymph nodes and gastrointestinal tract. Cutaneous mastocytosis represents over 90% of cases found with predilection in children. The aim of the paper was to summarize the authors clinical, histopathological and immunohistochemical observations on patients with cutaneous mastocytosis. We present four cases of cutaneous mastocytosis, sporadic form, customized by clinical presentation and age of onset: two installed in the neonatal period, a case with onset in infancy and another in adulthood. For the assessment of the severity and the effectiveness of the treatment, we used SCORMA Index. We performed in each patient histopathological examination of the skin (Hematoxylin-Eosin and Giemsa stains), the dosage of mediators (serum tryptase level, serum histamine levels, urinary histamine metabolites) and the balance of expansion (complete blood cell count, liver biological investigations, abdominal ultrasound, skeletal radiography, chest radiography). For the adult with mastocytosis, we performed abdominal scanner and cytological study of the bone marrow. Following investigations carried out in each case, we mentioned the diagnosis of cutaneous mastocytosis, and also excluded several diseases confounded by clinically and histologically aspect. Considering the fact that the balance expansion was negative, we excluded the diagnosis of systemic mastocytosis. The presence of anemia and protein energetic malnutrition in children with mastocytosis involves carrying out balance extension for the exclusion of a systemic form of the disease. Histopathological examination of the skin using special stains, the dosage of mediators (serum tryptase level, serum histamine levels, urinary histamine metabolites) and balance expansion establish the diagnosis of cutaneous mastocytosis and also exclude many confusions because of the clinical presentation.

Corresponding author: Virgil Patrascu, Associate Professor, MD, PhD; e-mail: vm.patrascu@gmail.com

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ROMANIAN JOURNAL of MORPHOLOGY and EMBRYOLOGY

Silviu Horia Morariu, Mircea Suciu, Mihai Dorin Vartolomei, Mihail Alexandru Badea, Ovidiu Simion Cotoi

Aneurysmal dermatofibroma (AD) or aneurysmal fibrous histiocytoma (AFH) is a relatively rare form of histiocytoma representing less than 2% of total cases. It shares many clinical and dermoscopic similarities with skin tumors, especially malignant melanoma and Kaposi s sarcoma, which can make differentiation problematic. We report the case of a 53-year-old man, who presents with a black nodular tumor with increased consistency, edges infiltrated from the surface to depth, spontaneous and sensitive to touch that shows rapid growth in the last three months. Dermoscopically, the central region consists of intricate areas colored in red, violet, blue-white and black. On the periphery stand two rings, centrally white and peripherally pigmented, with an abundance of polymorphic capillaries. To clarify the diagnosis, the lesion was widely excised and histopathological examination was performed, which revealed immunophenotypical tumor cells negative for HMB-45 and S100, and numerous CD68 macrophages between tumor cells. This aneurysmal fibrous histiocytoma shows both clinical and dermoscopically discrete differential criteria, which are not specific and that make it difficult to distinguish from malignant melanoma and Kaposi s sarcoma, and required performing histopathology and immunohistochemistry._x000D_

Corresponding author: Mircea Suciu, Associate Professor, MD, PhD; e-mail: suciu.mircea@umftgm.ro

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