Case report
Primary multiple miliary osteoma cutis: an unusual Chinese case Hui-Jun Ma*, MD, PhD, Chi-Yu Jia*, MD, PhD, Yang Yang, MM, Lian-Jin Song, MM, Rong Hu, MM, and Ting-Hui Li, MM
Department of Dermatology, Plastic Surgery and Burn Repair Center, 309 Hospital of Chinese PLA, Beijing, China Correspondence Hui-Jun Ma, MD Department of Dermatology, Plastic Surgery and Burn Repair Center 309 Hospital of Chinese PLA A17 Heishanhu Road Haidian District Beijing 100091 China E-mail: [email protected] Conflicts of interest: None. *The two authors contributed equally to this work.
Introduction
Case report
Cutaneous ossification is a rare and benign dermatosis caused by the formation of bone tissue in the dermis, which occurs more frequently in women. It is classified as primary osteoma cutis when bone tissue develops in the skin without any pre-existing lesion and secondary osteoma cutis, which is more frequent and occurs when osseous tissue develops on a pre-existing lesion.1,2 In general, the primary osteoma cutis has been described as four different clinical variants: solitary, widespread, plaque-like, and multiple miliary osteomas.3 Primary multiple miliary osteoma cutis (MMOC), also named multiple military osteomas of the face (MMOF), is characterized by the gradual development of multiple hard papules, up to 3 mm in diameter, predominantly affecting facial skin.4 Herein, we presented an unusual case of MMOC in a Chinese woman, which occurred on the parietal region of her scalp in a band-like distribution. To the best of our knowledge, this is the first case of MMOC involving the scalp in the English literature.
A 25-year-old Chinese female patient presented with a 5-year history of papular lesions developing on the parietal region of her scalp. These lesions were yellow, glossy, hard, and rice-like without any rational symptom. The lesions gradually increased in number and spread peripherally over the past five years. The patient had previously been treated with carbon dioxide laser before the lesions recurred on the affected area. There was no history of acne or any other dermatological disease involving the face or scalp. She denied any history of drug and food hypersensitivity and any history of head injury. There was no family history of a similar condition. On physical examination, multiple glossy yellowish millet to mung bean-sized papules near the right parietal region and right forehead were observed (Fig. 1a). The nodules were intensively distributed in a zone 10 cm long and 5 cm wide. There were several larger nodules at the center of the area than that of surrounding site. Satellitosis was observed simultaneously. The histopathological biopsy study showed the presence of multiple ossification and
ª 2013 The International Society of Dermatology
International Journal of Dermatology 2014, 53, 73–75
73
74
Case report
Unusual case of MMOC from China
(a)
Ma et al.
(b)
(c)
Figure 1 (a) Multiple yellowish small papules on the right parietal region of the scalp and forehead. (b) Scanning power show-
ing multiple ossification and calcification foci in the full thickness of the dermis (hematoxylin and eosin; original magnification, ·40). (c) Higher magnification showing that the ossification had a lamellar pattern and contained lacunae within which were nucleated osteocytes (hematoxylin and eosin; original magnification, ·200)
calcification foci in the dermis (Fig. 1b). These foci were circumscribed and heavily calcified. The ossification had a lamellar pattern and contained lacunae within which were nucleated osteocytes (Fig. 1c). No marrow cavity, haversian canals, or osteoclasts were seen. Routine blood investigations, including serum calcium, phosphate, urea, and creatinine, were normal. Discussion Osteoma cutis is a rare benign dermatosis characterized by the presence of mature osseous nodules within the dermis or subcutis. It may be classified as primary and secondary osteoma cutis. Secondary osteoma cutis, which is responsible for 85% of cases, is considered to be a result of cutaneous and metabolic alterations or a sequel of multiple disorders, including nevi, pilomatricoma, dermatomyositis, scars, trauma, and venous stasis.1,4 Primary osteoma cutis, which is less common, may be associated with Albright’s hereditary osteodystrophy or appear with no other associated disorder, such as fibrodysplasia ossificans progressive, progressive osseous heteroplasia, isolated osteomas, widespread osteomas, MMOC, and plaque-like osteomas cutis (PLOC).2 Our case was classified as primary MMOC because there were no pre-existing lesions and the osteoma exhibited a miliary-like clinical appearance. International Journal of Dermatology 2014, 53, 73–75
Multiple miliary osteoma cutis is a rare disease, and the etiology is still unknown. There are only 51 cases that satisfy the criteria for MMOC from a wide literature research until 2010.5 These cases included 42 female and nine male patients of African, Caucasian, East Asian, and Native American origin. The osteomas started to occur at a mean age of 47 years (range 17–79) and usually appeared on the face. Extrafacial osteomas occurred in only 11 patients (mainly on the shoulder, upper check, neck, and back).5 To the best of our knowledge, this is the first case of MMOC in the English literature involving the scalp. The role of acne in MMOC formation has been widely discussed in the literature. In MMOC the osteomas appear in the same skin areas as acne lesions, most often on the face, and in 55% of the cases reviewed here, there was a history of acne vulgaris.6 It has been hypothesized that osteomas are secondary to dystrophic changes in acne scars. However, our patient had no acne lesions before, and it does not support the idea. Hopkins7 suggested that primary osteomas might arise from embryonal rests. The bones of the face and skull are formed via the process of intramembranous ossification, and it has been suggested that MMOC arise in the area where fetal intramembranous osteogenesis occurs, namely in the facial and clavicular areas. Intramembranous ectopic bone formation is also found in progressive osseous heteroplasia, ª 2013 The International Society of Dermatology
Ma et al.
Albright’s hereditary osteodystrophy, and PLOC, which are all GNAS gene mutation-based diseases. However, the negative result from the sequencing of the GNAS gene from the skin of MMOC does not support this somatic mutation theory.5 MMOC seems to be a sporadic, nonhereditary disease, and the patients represent both sexes. Leider8 suggested that all cases of MMOC might represent a metaplastic process secondary to the traumatic rigors to which the facial skin is exposed. Alternatively, Kishi et al.9 suggested that MMOC might develop as a consequence of decreased metabolic functions within the skin associated with normal aging. There have been no reports of any biochemical or endocrine abnormalities that might affect calcification and ossification in these patients, and our patient had negative results from routine biochemical investigations. The differential diagnosis is always extremely critical for dermatologists. Therefore, it is necessary to pay attention to the differential diagnosis between MMOC and other skin diseases, which often affect the scalp, including comedones, sebaceous nevus, PLOC, and cutaneous calcinosis. In our case, it was difficult to distinguish MMOC from comedones initially because they were quite similar in appearance. The lesions in our case had been misdiagnosed as comedones many times. A precise history of acne and biopsy will contribute to the differential diagnosis. When these lesions became partly confluent, the differential diagnosis of sebaceous nevus or PLOC should be kept in mind. Another rare dermatosis, which is similar to MMOC in appearance, is cutaneous calcinosis, defined by the deposition of insoluble calcium chloride in the cutaneous tissue other than bone formation. In addition, radiological imaging techniques such as computerized tomography and radiography can help to make the differential diagnosis between MMOC and the above-mentioned dermatosis to some extent. Multiple miliary osteoma cutis may be very disturbing cosmetically. To date, no curative treatment for MMOC has been discovered. Different treatments have, however, been described in the literature such as oral isotretinoin or tetracycline, surgical treatment, needle microincision– extirpation method, and carbon dioxide laser with different outcomes.10,11 Our case is unusual because most of the lesions are localized on the right parietal region of the scalp in a band-like distribution, which is a rare occurrence in MMOC. From a
ª 2013 The International Society of Dermatology
Unusual case of MMOC from China
Case report
wide English literature search, this is the first case of MMOC that occurred on the scalp. Because of its rare occurrence on this site, we should pay attention to the differential diagnosis between MMOC, PLOC, comedones, sebaceous nevus, and cutaneous calcinosis. Acknowledgments This study was supported by a common project from National Natural Science Foundation of China (No.30600538). References 1 Ayaviri NA, Nahas FX, Barbosa MV, et al. Isolated primary osteoma cutis of the head: case report. Can J Plast Surg 2006; 14: 33–36. 2 Haro R, Revelles JM, Angulo J, et al. Plaque-like osteoma cutis with transepidermal elimination. J Cutan Pathol 2009; 36: 591–593. 3 Sethuraman G, Malhotra AK, Khaitan BK, et al. Osteoma cutis in pseudohypoparathyroidism. Clin Exp Dermatol 2006; 31: 225–227. 4 Novak C, Siller G, Wood D. Idiopathic multiple miliary osteomas of the face. Australas J Dermatol 1998; 39: 109–111. 5 Myllylä RM, Haapasaari KM, Palatsi R, et al. Multiple miliary osteoma cutis is a distinct disease entity: four case reports and review of the literature. Br J Dermatol 2011; 164: 544–552. 6 Thielen AM, Stucki L, Braun RP, et al. Multiple cutaneous osteomas of the face associated with chronic inflammatory acne. J Eur Acad Dermatol Venereol 2006; 20: 321–326. 7 Hopkins JG. Multiple miliary osteomas of the skin. Arch Derm Syphilol 1928; 18: 706–715. 8 Leider M. Osteomas cutis. Arch Derm Syphilol 1950; 62: 472–473. 9 Kishi K, Kawahara K, Moriya I, et al. Clinical and radiographic study of multiple miliary osteomas of the skin. Dentomaxillofac Radiol 1984; 13: 105–108. 10 Wu M, Wang Y, Zhang D, et al. A case of giant primary osteoma cutis successfully treated with tissue expansion and surgical excision. Indian J Dermatol Venereol Leprol 2011; 77: 79–81. 11 Baskan EB, Turan H, Tunali S, et al. Miliary osteoma cutis of the face: treatment with the needle microincisionextirpation method. J Dermatolog Treat 2007; 18: 252–254.
International Journal of Dermatology 2014, 53, 73–75
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Primary multiple miliary osteoma cutis: an unusual Chinese case Hui-Jun Ma*, MD, PhD, Chi-Yu Jia*, MD, PhD, Yang Yang, MM, Lian-Jin Song, MM, Rong Hu, MM, and Ting-Hui Li, MM
Department of Dermatology, Plastic Surgery and Burn Repair Center, 309 Hospital of Chinese PLA, Beijing, China Correspondence Hui-Jun Ma, MD Department of Dermatology, Plastic Surgery and Burn Repair Center 309 Hospital of Chinese PLA A17 Heishanhu Road Haidian District Beijing 100091 China E-mail: [email protected] Conflicts of interest: None. *The two authors contributed equally to this work.
Introduction
Case report
Cutaneous ossification is a rare and benign dermatosis caused by the formation of bone tissue in the dermis, which occurs more frequently in women. It is classified as primary osteoma cutis when bone tissue develops in the skin without any pre-existing lesion and secondary osteoma cutis, which is more frequent and occurs when osseous tissue develops on a pre-existing lesion.1,2 In general, the primary osteoma cutis has been described as four different clinical variants: solitary, widespread, plaque-like, and multiple miliary osteomas.3 Primary multiple miliary osteoma cutis (MMOC), also named multiple military osteomas of the face (MMOF), is characterized by the gradual development of multiple hard papules, up to 3 mm in diameter, predominantly affecting facial skin.4 Herein, we presented an unusual case of MMOC in a Chinese woman, which occurred on the parietal region of her scalp in a band-like distribution. To the best of our knowledge, this is the first case of MMOC involving the scalp in the English literature.
A 25-year-old Chinese female patient presented with a 5-year history of papular lesions developing on the parietal region of her scalp. These lesions were yellow, glossy, hard, and rice-like without any rational symptom. The lesions gradually increased in number and spread peripherally over the past five years. The patient had previously been treated with carbon dioxide laser before the lesions recurred on the affected area. There was no history of acne or any other dermatological disease involving the face or scalp. She denied any history of drug and food hypersensitivity and any history of head injury. There was no family history of a similar condition. On physical examination, multiple glossy yellowish millet to mung bean-sized papules near the right parietal region and right forehead were observed (Fig. 1a). The nodules were intensively distributed in a zone 10 cm long and 5 cm wide. There were several larger nodules at the center of the area than that of surrounding site. Satellitosis was observed simultaneously. The histopathological biopsy study showed the presence of multiple ossification and
ª 2013 The International Society of Dermatology
International Journal of Dermatology 2014, 53, 73–75
73
74
Case report
Unusual case of MMOC from China
(a)
Ma et al.
(b)
(c)
Figure 1 (a) Multiple yellowish small papules on the right parietal region of the scalp and forehead. (b) Scanning power show-
ing multiple ossification and calcification foci in the full thickness of the dermis (hematoxylin and eosin; original magnification, ·40). (c) Higher magnification showing that the ossification had a lamellar pattern and contained lacunae within which were nucleated osteocytes (hematoxylin and eosin; original magnification, ·200)
calcification foci in the dermis (Fig. 1b). These foci were circumscribed and heavily calcified. The ossification had a lamellar pattern and contained lacunae within which were nucleated osteocytes (Fig. 1c). No marrow cavity, haversian canals, or osteoclasts were seen. Routine blood investigations, including serum calcium, phosphate, urea, and creatinine, were normal. Discussion Osteoma cutis is a rare benign dermatosis characterized by the presence of mature osseous nodules within the dermis or subcutis. It may be classified as primary and secondary osteoma cutis. Secondary osteoma cutis, which is responsible for 85% of cases, is considered to be a result of cutaneous and metabolic alterations or a sequel of multiple disorders, including nevi, pilomatricoma, dermatomyositis, scars, trauma, and venous stasis.1,4 Primary osteoma cutis, which is less common, may be associated with Albright’s hereditary osteodystrophy or appear with no other associated disorder, such as fibrodysplasia ossificans progressive, progressive osseous heteroplasia, isolated osteomas, widespread osteomas, MMOC, and plaque-like osteomas cutis (PLOC).2 Our case was classified as primary MMOC because there were no pre-existing lesions and the osteoma exhibited a miliary-like clinical appearance. International Journal of Dermatology 2014, 53, 73–75
Multiple miliary osteoma cutis is a rare disease, and the etiology is still unknown. There are only 51 cases that satisfy the criteria for MMOC from a wide literature research until 2010.5 These cases included 42 female and nine male patients of African, Caucasian, East Asian, and Native American origin. The osteomas started to occur at a mean age of 47 years (range 17–79) and usually appeared on the face. Extrafacial osteomas occurred in only 11 patients (mainly on the shoulder, upper check, neck, and back).5 To the best of our knowledge, this is the first case of MMOC in the English literature involving the scalp. The role of acne in MMOC formation has been widely discussed in the literature. In MMOC the osteomas appear in the same skin areas as acne lesions, most often on the face, and in 55% of the cases reviewed here, there was a history of acne vulgaris.6 It has been hypothesized that osteomas are secondary to dystrophic changes in acne scars. However, our patient had no acne lesions before, and it does not support the idea. Hopkins7 suggested that primary osteomas might arise from embryonal rests. The bones of the face and skull are formed via the process of intramembranous ossification, and it has been suggested that MMOC arise in the area where fetal intramembranous osteogenesis occurs, namely in the facial and clavicular areas. Intramembranous ectopic bone formation is also found in progressive osseous heteroplasia, ª 2013 The International Society of Dermatology
Ma et al.
Albright’s hereditary osteodystrophy, and PLOC, which are all GNAS gene mutation-based diseases. However, the negative result from the sequencing of the GNAS gene from the skin of MMOC does not support this somatic mutation theory.5 MMOC seems to be a sporadic, nonhereditary disease, and the patients represent both sexes. Leider8 suggested that all cases of MMOC might represent a metaplastic process secondary to the traumatic rigors to which the facial skin is exposed. Alternatively, Kishi et al.9 suggested that MMOC might develop as a consequence of decreased metabolic functions within the skin associated with normal aging. There have been no reports of any biochemical or endocrine abnormalities that might affect calcification and ossification in these patients, and our patient had negative results from routine biochemical investigations. The differential diagnosis is always extremely critical for dermatologists. Therefore, it is necessary to pay attention to the differential diagnosis between MMOC and other skin diseases, which often affect the scalp, including comedones, sebaceous nevus, PLOC, and cutaneous calcinosis. In our case, it was difficult to distinguish MMOC from comedones initially because they were quite similar in appearance. The lesions in our case had been misdiagnosed as comedones many times. A precise history of acne and biopsy will contribute to the differential diagnosis. When these lesions became partly confluent, the differential diagnosis of sebaceous nevus or PLOC should be kept in mind. Another rare dermatosis, which is similar to MMOC in appearance, is cutaneous calcinosis, defined by the deposition of insoluble calcium chloride in the cutaneous tissue other than bone formation. In addition, radiological imaging techniques such as computerized tomography and radiography can help to make the differential diagnosis between MMOC and the above-mentioned dermatosis to some extent. Multiple miliary osteoma cutis may be very disturbing cosmetically. To date, no curative treatment for MMOC has been discovered. Different treatments have, however, been described in the literature such as oral isotretinoin or tetracycline, surgical treatment, needle microincision– extirpation method, and carbon dioxide laser with different outcomes.10,11 Our case is unusual because most of the lesions are localized on the right parietal region of the scalp in a band-like distribution, which is a rare occurrence in MMOC. From a
ª 2013 The International Society of Dermatology
Unusual case of MMOC from China
Case report
wide English literature search, this is the first case of MMOC that occurred on the scalp. Because of its rare occurrence on this site, we should pay attention to the differential diagnosis between MMOC, PLOC, comedones, sebaceous nevus, and cutaneous calcinosis. Acknowledgments This study was supported by a common project from National Natural Science Foundation of China (No.30600538). References 1 Ayaviri NA, Nahas FX, Barbosa MV, et al. Isolated primary osteoma cutis of the head: case report. Can J Plast Surg 2006; 14: 33–36. 2 Haro R, Revelles JM, Angulo J, et al. Plaque-like osteoma cutis with transepidermal elimination. J Cutan Pathol 2009; 36: 591–593. 3 Sethuraman G, Malhotra AK, Khaitan BK, et al. Osteoma cutis in pseudohypoparathyroidism. Clin Exp Dermatol 2006; 31: 225–227. 4 Novak C, Siller G, Wood D. Idiopathic multiple miliary osteomas of the face. Australas J Dermatol 1998; 39: 109–111. 5 Myllylä RM, Haapasaari KM, Palatsi R, et al. Multiple miliary osteoma cutis is a distinct disease entity: four case reports and review of the literature. Br J Dermatol 2011; 164: 544–552. 6 Thielen AM, Stucki L, Braun RP, et al. Multiple cutaneous osteomas of the face associated with chronic inflammatory acne. J Eur Acad Dermatol Venereol 2006; 20: 321–326. 7 Hopkins JG. Multiple miliary osteomas of the skin. Arch Derm Syphilol 1928; 18: 706–715. 8 Leider M. Osteomas cutis. Arch Derm Syphilol 1950; 62: 472–473. 9 Kishi K, Kawahara K, Moriya I, et al. Clinical and radiographic study of multiple miliary osteomas of the skin. Dentomaxillofac Radiol 1984; 13: 105–108. 10 Wu M, Wang Y, Zhang D, et al. A case of giant primary osteoma cutis successfully treated with tissue expansion and surgical excision. Indian J Dermatol Venereol Leprol 2011; 77: 79–81. 11 Baskan EB, Turan H, Tunali S, et al. Miliary osteoma cutis of the face: treatment with the needle microincisionextirpation method. J Dermatolog Treat 2007; 18: 252–254.
International Journal of Dermatology 2014, 53, 73–75
75
