Nesimi Büyükbabani

Osteoporosis and Osteopathy Markers in Patients with Mastocytosis

Objective: Osteoporosis, osteosclerosis, and lytic bone lesions have been observed in patients with systemic mastocytosis (SM). We examined bone mineral density (BMD) biochemical turnover markers and serum tryptase levels in SM, which is considered a rare disease. Materials and Methods: Seventeen adult patients (5 females, 12 males; median age: 33 years, range: 20-64) with mastocytosis were included in this study. We investigated the value of quantitative ultrasound (QUS) of the calcaneus in the assessment of BMD in SM patients, as well as BMD of the lumbar spine (L1-L4), femoral neck, and distal radius using dual energy x-ray absorptiometry (DXA) and plasma tryptase levels, biochemical markers of bone turnover. Results: At lumbar spine L1-L4, the femoral neck, and the distal radius or as calcaneus stiffness, 12 of 17 patients had T-scores of less than -1 at least at 1 site, reflecting osteopenia. Three of 17 patients had T-scores showing osteoporosis (T-score <-2.5). There was no relationship between DXA and bone lesion severity. We also found a significant positive correlation between tryptase levels and disease severity, as well as between disease severity and pyridinoline (p<0.01 by Spearman’s test). Conclusion: DXA and calcaneal QUS may not be appropriate techniques to assess bone involvement in SM patients because of the effects of osteosclerosis. This study further shows that the osteoclastic marker pyridinoline is helpful in patients with severe disease activity and sclerotic bone lesions to show bone demineralization.

Annular Erythematous Patches as the Presenting Sign of Extranodal Natural Killer/T-Cell Lymphoma

Extranodal natural killer/T-cell lymphoma (ENKTL) is a distinct type of lymphoma strongly associated with Epstein-Barr virus (EBV) infection and showing an aggressive course [1]. It usually presents as a localized disease in the upper aerodigestive tract, from the nasal cavity to the hypopharynx [2,3], but it may rapidly extend to the neighboring tissues and disseminate to various organs such as the small intestine, epiglottis, testes, adrenal gland, kidneys, and breasts [4,5]. As nasal/upper aerodigestive tract involvement may only cause nonspecific symptoms in the early period, diagnosis may be initially established based upon skin lesions [6]. We present two ENKTL patients with unusual dermatological findings.

The Dilemma of Coexisting Nevoid Hyperkeratosis of the Nipple and Areola in Mycosis Fungoides: A Report of Three Cases.

Nevoid hyperkeratosis of the nipple and areola (NHNA) is a rare clinicopathological entity showing persistent and strictly localized hyperkeratotic lesions of the nipple, areola or both with unknown etiopathogenesis. A similar clinical appearance may also be seen in different diseases with specific histopathological features. There are a few anecdotal reports on the association of NHNA with mycosis fungoides (MF), but they do not describe a uniform condition. In this report, we present 3 patients with hyperkeratotic lesions of the nipple and areola associated with MF but showing different histopathological features. We also review similar cases in the literature and discuss possibilities concerning this association. Two of our cases represent the association between MF and NHNA without histopathological features of MF on the nipple-areola complex. The other case represents hyperkeratosis of the nipple and areola with specific histological and immunohistochemical features of MF. Hence, we would like to hypothesize that MF may involve the nipple and areola and have an appearance similar to NHNA. Intriguingly, however, NHNA may occasionally also be seen in association with MF. However, this peculiar association requires further explanation.

Clinical atlas of skin tumors

BENIGN SKIN TUMORS.- Epidermal Benign Tumors.- Epidermal Precancerous Lesions and in Situ Malignancies of Skin.- Nevo-Melanocytic Benign Tumors.- Cutaneous Cysts.- Cutaneous Adnexial Tumors.- Benign Vascular Tumors Of The Skin.- Benign Neural Tissue Tumors.- Benign Fibrohistiocytic Tumors.- Mastocytosis.- Subcutaneous Fat Tissue Tumors.- MALIGNANT SKIN TUMORS.- Malignant Epithelial Tumors.- Malignant Melanoma.- Sarcomatous Tumors of Skin.- Cutaneous Lymphomas.- Histiocytosis.- Cutaneous Metastasis.- Predilection sites of skin tumors.

Epidermodysplasia Verruciformis Associated with Natural Killer/T Cell Lymphoma

Figure 3. Skin biopsy specimen showing a thickened cornified layer and epidermis along with aggregates of large, bluegray keratinocytes and a prominent granular layer. (HemaE pidermodysplasia verruciformis (EV) is an extremely rare genodermatosis characterized by verruca plana like warts that may undergo malignant transformation. We report a 12-year-old boy who had hypopigmented smooth macules and wart-like lesions for 10 years (Figure 1). He was referred for facial swelling and erythema of his right cheek. The right nasal cavity was completely filled with a smooth mass. A biopsy specimen obtained from the nasal cavity revealed high-grade malignant tumor infiltration (Figure 2). The immunophenotype of this tumor was CD56, CD3, and CD20, consistent with natural killer (NK)/T cell lymphoma. Weak staining for Epstein-Barr virus (EBV) was evident. A skin biopsy specimen from a representative lesion exhibited hyperkeratosis, acanthosis, and groups of large blue-gray keratinocytes with large nuclei and a prominent, granular layer with condensed keratohyaline granules (Figure 3). Various abnormalities of nonspecific cell-mediated immunity have been found in patients with EV. These include decreased absolute numbers of T lymphocytes and T helper cells with a reversed CD4/CD8 ratio, increased NK cell activity, and T cell–mediated cytotoxicity against human papillomavirus-harboring keratinocytes. Our patient had a normal absolute lymphocyte count. Flow cytometry demonstrated reversal of the CD4/CD8 ratio, but normal CD3 cells. Nasal NK/T cell lymphomas are almost always associated with EBV. In an immunocompromised host, EBV can proliferate and induce lymphoproliferative disorders or lymphoma. In our patient, the presence of both EV and EBV may have contributed to the development of nasal NK/T cell lymphoma. Pediatricians should be aware of this rare dermatosis, and EV should be suspected in the clinical setting of numerous verrucous lesions in early childhood. Patients should receive regular follow-up for possible cutaneous or extracutaneous malignancy. n