Viewpoints in dermatology • Correspondence
Correspondence
Eosinophilic angiocentric fibrosis presenting as asymptomatic cutaneous nodules
A 74-year-old woman presented with a 1-month history of an asymptomatic right-sided breast lump. A mammogram was performed and showed an area of increased density in the right breast with an additional high-density lesion in the left breast. An ultrasonography scan revealed focal areas of high vascularity suspicious of carcinoma. Two biopsies were taken of the suspicious foci under ultrasound guidance. The histology of each was reported as inflammatory myofibroblastic tumour and the lesions were then excised. The patient presented to dermatology for a second opinion as further asymptomatic nodules were palpable on her left and right upper arms and right upper chest. The histology was re-evaluated and revealed breast parenchyma with widespread chronic inflammation and fibrosis. No epithelial hyperplasia, atypia or malignancy was seen. Fibrosis was a feature common to all biopsies and excision specimens. The fibrosis included foci having a regional concentric pattern around small blood vessels. This was admixed with numerous eosinophils. Based on
the histological features, a diagnosis of eosinophilic angiocentric fibrosis (EAF) was made. EAF is a rare fibrosing disease of unknown aetiology, first described by Holmes and Panje in 1983.1 Histopathologically, EAF shares features with granuloma faciale (GF), and 13% of the EAF cases in the English literature had concurrent GF.2 The mean age at diagnosis of EAF is 48 years, with a range age of 19–79 years.3 In total, 53 cases of EAF have been described in the English literature. All were described as arising in the sinus or respiratory tracts, except for 6 cases described in the orbit. To our knowledge, this is the first case involving the skin and subcutis of the arms and breasts. The diagnosis of EAF is based on the distinctive histological features. In the early phase, a dense inflammatory infiltrate is seen with numerous eosinophils (Fig. 1). In the later phase, it is characterised by a perivascular concentric pattern of fibrosis (Fig. 2). The relationship between EAF and GF is unclear, but the histological similarities and concurrence of the two diseases in the same patient, albeit rare, suggest a close relationship. Given the diagnostic difficulty in the present case, together with the relatively subtle histological diagnostic clues, it is quite likely that other cases of EAF
Figure 1 Fibrosis and an inflammatory cell infiltrate including
Figure 2 Regional concentric fibrosis was evident, centered
numerous eosinophils was seen across all biopsies taken.
around blood vessels.
doi: 10.1111/ced.12416
ª 2014 British Association of Dermatologists
Clinical and Experimental Dermatology (2015) 40, pp85–90
85
Correspondence
affecting uncommon sites have gone unreported. The rarity of the disease means that a consensus on treatment strategies is yet to be reached. Most reported cases in the literature had surgical excision, as did our patient. In summary, we report a 74-year-old woman presenting with multiple asymptomatic lumps on her breasts and arms, which were confirmed histologically as EAF fibrosis. This is the first case in English literature to involve the skin and subcutis of sites other than the upper and lower respiratory tract or orbit. E. Rashidghamat, R. Groves, and A. Robson St John’s Institute of Dermatology and Dermatopathology, Guy’s and St Thomas’ NHS Trust, London, SE17EH, UK E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 13 March 2014
References 1 Holmes DK, Panje WR. Intranasal granuloma faciale. Am J Otolaryngol 1983; 4: 184–6. 2 Nigar E, Dhillon R, Matin RN. Eosinophilic angiocentric fibrosis and extrafacial granuloma annulare. Histopathology 2007; 51: 729–31. 3 Sunde J, Alexander KA, Reddy VV, Woodworth BA. Intranasal eosinophilic angiocentric fibrosis: a case report and review. Head Neck Pathol 2010; 4: 246–8.
dermis (Fig. 2a). One month later, the patient requested a skin biopsy, as the histopathological examination returned similar results, although there were more melanocytes in the dermis. Immunohistochemical analysis revealed that the pigmented cells were positive for S-100 (Fig. 2b) and Melan A (Fig. 2c) staining, with a smattering of HMB-45-positive cells. Although the aetiology of ADM is currently unknown, several hypotheses have been proposed. According to the most widely accepted theory, proposed by Mizushima,2 ADM occurs when pre-existing immature dermal melanocytes are reactivated by factors such as oestrogen, sunlight and inflammation. The histopathological findings of ADM include spindle-shaped dendritic cells containing melanin in the dermis.3 The pigmented cells test positive for S-100 and HMB-45. This raises the possibility that, although there is no melanin in normal cells, there may be an undetectable ‘melanin potential’ in the cells of people predisposed to this condition, with detection being possible only when the melanin is activated.
(a)
Acquired dermal melanocytosis on the back doi: 10.1111/ced.12432 Acquired dermal melanocytosis (ADM) is a pigmentary disorder characterized clinically by blue–brown or grey– blue patches, first described in the literature by Mevorah in 1977.1 We report a rare case of acquired dermal melanocytosis on the back. In April 2013, a 46-year-old Chinese woman presented with a non-palpable grey–blue hyperpigmented patch on her lower left back. The lesion, which was asymptomatic, had appeared approximately 1 year previously. Two hospitals had diagnosed the patient with haemangioma despite normal results on magnetic resonance imaging scans. On physical examination, we found a grey-blue, nonpalpable patch, measuring 150 9 250 mm, on the left side of the patient’s back (Fig. 1). There were no other unusual findings, and the patient was otherwise healthy. She reported no previous inflammation, significant sun exposure or other skin disease. There was no family history of abnormal cutaneous pigmentation. Histopathological examination revealed scattered, spindle-shaped dendritic cells containing melanin in the
86
Clinical and Experimental Dermatology (2015) 40, pp85–90
(b)
Figure 1 Physical examination. Grey-blue, nonpalpable patch,
measuring 150 9 250 mm, with red scar visible at the biopsy site.
ª 2014 British Association of Dermatologists
Correspondence
Eosinophilic angiocentric fibrosis presenting as asymptomatic cutaneous nodules
A 74-year-old woman presented with a 1-month history of an asymptomatic right-sided breast lump. A mammogram was performed and showed an area of increased density in the right breast with an additional high-density lesion in the left breast. An ultrasonography scan revealed focal areas of high vascularity suspicious of carcinoma. Two biopsies were taken of the suspicious foci under ultrasound guidance. The histology of each was reported as inflammatory myofibroblastic tumour and the lesions were then excised. The patient presented to dermatology for a second opinion as further asymptomatic nodules were palpable on her left and right upper arms and right upper chest. The histology was re-evaluated and revealed breast parenchyma with widespread chronic inflammation and fibrosis. No epithelial hyperplasia, atypia or malignancy was seen. Fibrosis was a feature common to all biopsies and excision specimens. The fibrosis included foci having a regional concentric pattern around small blood vessels. This was admixed with numerous eosinophils. Based on
the histological features, a diagnosis of eosinophilic angiocentric fibrosis (EAF) was made. EAF is a rare fibrosing disease of unknown aetiology, first described by Holmes and Panje in 1983.1 Histopathologically, EAF shares features with granuloma faciale (GF), and 13% of the EAF cases in the English literature had concurrent GF.2 The mean age at diagnosis of EAF is 48 years, with a range age of 19–79 years.3 In total, 53 cases of EAF have been described in the English literature. All were described as arising in the sinus or respiratory tracts, except for 6 cases described in the orbit. To our knowledge, this is the first case involving the skin and subcutis of the arms and breasts. The diagnosis of EAF is based on the distinctive histological features. In the early phase, a dense inflammatory infiltrate is seen with numerous eosinophils (Fig. 1). In the later phase, it is characterised by a perivascular concentric pattern of fibrosis (Fig. 2). The relationship between EAF and GF is unclear, but the histological similarities and concurrence of the two diseases in the same patient, albeit rare, suggest a close relationship. Given the diagnostic difficulty in the present case, together with the relatively subtle histological diagnostic clues, it is quite likely that other cases of EAF
Figure 1 Fibrosis and an inflammatory cell infiltrate including
Figure 2 Regional concentric fibrosis was evident, centered
numerous eosinophils was seen across all biopsies taken.
around blood vessels.
doi: 10.1111/ced.12416
ª 2014 British Association of Dermatologists
Clinical and Experimental Dermatology (2015) 40, pp85–90
85
Correspondence
affecting uncommon sites have gone unreported. The rarity of the disease means that a consensus on treatment strategies is yet to be reached. Most reported cases in the literature had surgical excision, as did our patient. In summary, we report a 74-year-old woman presenting with multiple asymptomatic lumps on her breasts and arms, which were confirmed histologically as EAF fibrosis. This is the first case in English literature to involve the skin and subcutis of sites other than the upper and lower respiratory tract or orbit. E. Rashidghamat, R. Groves, and A. Robson St John’s Institute of Dermatology and Dermatopathology, Guy’s and St Thomas’ NHS Trust, London, SE17EH, UK E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 13 March 2014
References 1 Holmes DK, Panje WR. Intranasal granuloma faciale. Am J Otolaryngol 1983; 4: 184–6. 2 Nigar E, Dhillon R, Matin RN. Eosinophilic angiocentric fibrosis and extrafacial granuloma annulare. Histopathology 2007; 51: 729–31. 3 Sunde J, Alexander KA, Reddy VV, Woodworth BA. Intranasal eosinophilic angiocentric fibrosis: a case report and review. Head Neck Pathol 2010; 4: 246–8.
dermis (Fig. 2a). One month later, the patient requested a skin biopsy, as the histopathological examination returned similar results, although there were more melanocytes in the dermis. Immunohistochemical analysis revealed that the pigmented cells were positive for S-100 (Fig. 2b) and Melan A (Fig. 2c) staining, with a smattering of HMB-45-positive cells. Although the aetiology of ADM is currently unknown, several hypotheses have been proposed. According to the most widely accepted theory, proposed by Mizushima,2 ADM occurs when pre-existing immature dermal melanocytes are reactivated by factors such as oestrogen, sunlight and inflammation. The histopathological findings of ADM include spindle-shaped dendritic cells containing melanin in the dermis.3 The pigmented cells test positive for S-100 and HMB-45. This raises the possibility that, although there is no melanin in normal cells, there may be an undetectable ‘melanin potential’ in the cells of people predisposed to this condition, with detection being possible only when the melanin is activated.
(a)
Acquired dermal melanocytosis on the back doi: 10.1111/ced.12432 Acquired dermal melanocytosis (ADM) is a pigmentary disorder characterized clinically by blue–brown or grey– blue patches, first described in the literature by Mevorah in 1977.1 We report a rare case of acquired dermal melanocytosis on the back. In April 2013, a 46-year-old Chinese woman presented with a non-palpable grey–blue hyperpigmented patch on her lower left back. The lesion, which was asymptomatic, had appeared approximately 1 year previously. Two hospitals had diagnosed the patient with haemangioma despite normal results on magnetic resonance imaging scans. On physical examination, we found a grey-blue, nonpalpable patch, measuring 150 9 250 mm, on the left side of the patient’s back (Fig. 1). There were no other unusual findings, and the patient was otherwise healthy. She reported no previous inflammation, significant sun exposure or other skin disease. There was no family history of abnormal cutaneous pigmentation. Histopathological examination revealed scattered, spindle-shaped dendritic cells containing melanin in the
86
Clinical and Experimental Dermatology (2015) 40, pp85–90
(b)
Figure 1 Physical examination. Grey-blue, nonpalpable patch,
measuring 150 9 250 mm, with red scar visible at the biopsy site.
ª 2014 British Association of Dermatologists
