Tropical medicine rounds
Disseminated giant porokeratosis and porokeratosis of Mibelli in Bankura and Bardhaman districts, West Bengal, India Sankha Koley, MD, Rajesh Kumar Mandal, MD, and Chittaranjan Bar, MD
Department of Dermatology, North Bengal Medical College, Darjeeling, India Correspondence Sankha Koley MD North Bengal Medical College Subhankar Sarani Bankura 722101 West Bengal India E-mail: [email protected]
Abstract Porokeratosis of Mibelli (PM) is a rare genodermatosis. It is caused by proliferation of abnormal clones of epidermal cells in response to several stimuli, the most important of which is sunlight. Giant porokeratosis is thought to be a variant of PM. We report two cases of disseminated PM and one case of disseminated giant porokeratosis from the Bankura and Bardhaman districts of West Bengal in India; presenting to a single observer in the summer of 2012. Interestingly we have noted that the majority of cases of porokeratosis in India have been reported from West Bengal. Few patients with a single lesion of giant porokeratosis have been reported in world literature. As far as we know, this is the first case report of disseminated giant porokeratosis in world literature.
Introduction Porokeratosis of Mibelli (PM) is characterized clinically by one or more annular plaques with central atrophy and elevated hyperkeratotic ridge, called the cornoid lamella. Giant porokeratosis is considered by most authors as a morphological variant of PM. Sunlight is the most important aggravating factor in porokeratosis. Reports of disseminated lesions of PM and single lesion of giant porokeratosis have occasionally been made in Indian and other international literature. We report a rare case of disseminated giant porokeratosis and two cases of disseminated PM presenting to a single observer in Bankura and Bardhaman districts of West Bengal, India, in summer 2012. Case reports Case 1
A 44-year-old farmer from a remote village in Bankura district presented with widespread large annular plaques of variable sizes with well-defined border and grooved keratotic margin and central atrophy all over his body (Figs. 1 and 2). The initial lesion started 20 years ago as small coin-sized plaques over his leg that gradually increased in size in centrifugal expansion. After three years, a similar lesion appeared over his face and enlarged slowly to involve his right eye, leading to cicatriª 2014 The International Society of Dermatology
cial ectropion (Fig. 1). This lesion further extended to involve the scalp, with subsequent loss of hairs. Subsequently more such lesions of variable sizes appeared all over his body. He worked in fields for long hours in sunlight with bare skin without any protection. There was no family history of this disease. Based on these findings, he was given a provisional diagnosis of disseminated giant porokeratosis. Case 2
A middle-aged female laborer from a remote village in Bardhaman district presented with multiple small hyperpigmented annular plaques of different sizes, each with a grooved keratotic margin, diffusely spread all over her body (Fig. 3). The lesions started four years previously and gradually increased in number. The lesions were mostly over the extensor surface of her upper limbs, face, and upper back. Her elder sister and mother had similar lesions. As a laborer, she had to work outside under sunlight for 10 hours daily. With this clinical presentation, she was given a provisional diagnosis of disseminated PM. Case 3
A 22-year-old farmer from a remote village in Bankura district presented with multiple small hyperpigmented annular plaques of different sizes and a grooved keratotic margin over the forehead, upper chest, and upper back International Journal of Dermatology 2014, 53, 1119–1124
1119
1120
Tropical medicine rounds
Disseminated porokeratosis gigantea and Mibelli
Koley et al.
Figure 1 Case 1: giant porokeratosis causing cicatricial ectropion of right eye and scarring alopecia
Figure 2 Case 1: lesions of giant porokeratosis all over the body International Journal of Dermatology 2014, 53, 1119–1124
ª 2014 The International Society of Dermatology
Koley et al.
Disseminated porokeratosis gigantea and Mibelli
Tropical medicine rounds
Figure 3 Case 2: disseminated lesions of porokeratosis of Mibelli on face, bilateral hands, abdomen, and back
(Fig. 4). The lesions started three months prior and gradually increased in size and number. He also had a history of prolonged sunlight exposure. There was no family history of similar disease. He was given a provisional diagnosis of disseminated PM. All the patients were thoroughly investigated. All routine tests were within normal limits. None of the patients had HIV or any other kind of immunosuppression, including diabetes. Neither of them received any immunosuppressive drug for long. They were either farmer or laborer by profession and worked 8–10 hours under the sun on most days. All of them gave a history of exaggeration of lesions in summer. All the cases were clinically diagnosed by well-defined, keratotic border of lesions with the presence of gutter-like groove on their summits. They were further confirmed ª 2014 The International Society of Dermatology
histologically. Biopsy specimens revealed characteristic cornoid lamella, absent granular layer below the invagination, and mild chronic inflammatory cell infiltration in the upper dermis (Figs. 5a,b, 6a,b, and 7a,b). All of them were advised strict photoprotection, emollients in addition to treatment noted in Table 1. Comments Porokeratosis is a rare genodermatosis featuring abnormal epidermal keratinization. It is transmitted as an autosomal dominant trait with variable penetrance.1 Classically, five clinical variants are recognized; disseminated superficial porokeratosis and its actinic variant (most common), classic porokeratosis of Mibelli (second most common), punctate porokeratosis, porokeratosis palmaris et plantaris International Journal of Dermatology 2014, 53, 1119–1124
1121
1122
Tropical medicine rounds
Koley et al.
Disseminated porokeratosis gigantea and Mibelli
Figure 4 Case 3: Disseminated lesions of porokeratosis of Mibelli on forehead and upper back
Table 1 Clinical presentations of three cases Case no.
1 (Figs. 1 and 2)
2 (Fig. 3)
3 (Fig. 4)
Date of presentation Age (in years) Sex Address Occupation Distribution Duration Size (diameter) and site of larger lesions Family history Diagnosis Biopsy location Histopathology finding
April 11, 2012
April 27, 2012
June 5, 2012
44 M Susunia village, Bankura Farmer All over body 20 years – right leg; 17 years – on head 35–40 cm on right face and back of head; 16–22 cm on posterior legs
27 F Rasikpur village, Bardhaman Laborer Face, bilateral hands, abdomen and back 4 years 3–5 cm on left neck and upper back
22 M Chatna, Bankura Farmer Forehead, upper chest and back 3 months 3–4 on mid back
Absent Disseminated giant porokeratosis Right back Cornoid lamella, absent granular layer, mild chronic dermal inflammatory infiltrates Isotretinoin 20 mg per day, isotretinoin 0.05% cream at night
Similar lesion in elder sister and mother Disseminated PM Left neck Cornoid lamella with diminished granular layer and mild dermal lymphohistiocytic infiltrate Topical halobetasol 0.05% ointment daytime and isotretinoin 0.05% cream at bedtime Mild improvement after 3 months
Absent Disseminated PM Left back Cornoid lamella, diminished granular layer and mild dermal inflammatory infiltrate Topical halobetasol 0.05% ointment twice daily
Treatment
Follow-up
Lost to follow-up
Considerable improvement after 3 months
F, female; M, male.
disseminata, and linear porokeratosis.2 The border is usually more than 1 mm in height and contains a thread-like groove. Giant porokeratosis, where the lesion may vary from 10 to 20 cm in diameter and the hyperkeratotic walls raised greater than 1 cm, is considered a morphological variant of PM by most authors, while others consider it a distinct entity. International Journal of Dermatology 2014, 53, 1119–1124
There is a hypothesis that porokeratosis is triggered by proliferation of abnormal clones of epidermal cells in response to several stimuli, the most important of which are sunlight and artificial ultraviolet (UV).3 Non-familial disseminated superficial porokeratosis and single plaque PM have been documented to occur in association with immunosuppression. While sun exposure plays an important pathogenetic role ª 2014 The International Society of Dermatology
Koley et al.
Disseminated porokeratosis gigantea and Mibelli
(a)
Tropical medicine rounds
(b)
Figure 5 (a) Histopathological finding in case 1: cornoid lamella, absent granular layer below the invagination and mild chronic inflammatory cell infiltration in upper dermis (hematoxylin and eosin, 940). (b) Magnified view of (a) (hematoxylin and eosin, 9400)
(a)
(b)
Figure 6 (a) Keratin-filled invagination of epidermis with parakeratotic cells (cornoid lamella), diminished granular layer with mild dermal lymphohistiocytic infiltrate (hematoxylin and eosin, 9100). (b) Magnified view of (a) (hematoxylin and eosin, 9400)
(a)
(b)
Figure 7 (a) Cornoid lamella, diminished granular layer, and mild dermal inflammatory infiltrate (hematoxylin and eosin, 940). (b) Magnified view of (a) (hematoxylin and eosin, 9400)
in idiopathic cases, it does not seem to be essential for the development of porokeratosis in immunosuppressed patients.4 Disseminated PM has been reported following renal5 and bone marrow transplantation for leukemia, nonHodgkin’s lymphoma,6 and myelodysplastic syndrome.7 However, our patient had no history of transplantation or systemic immunosuppression. Porokeratosis may cause damage or even mutilation of body parts.8 In case 1, a lesion of giant porokeratosis had caused cicatricial ectropion of the right eye and scarring alopecia of the head. The UV index is an international standard measurement of the strength of UV radiation from the sun at a ª 2014 The International Society of Dermatology
particular place on a particular day. During the summer, scorching heat prevails over West Bengal, a state in the eastern part of India. Bankura and Bardhaman are two of the seven districts of the Burdwan Division of West Bengal. The data of the daily UV index within the last seven years (January 1, 2006, to October 31, 2012) collected from an internet website denotes that the highest UV index prevails from mid-April to mid-July.9 Last summer, mercury was as high as 47 °C in Bardhaman and Bankura districts. All three cases were presented to a single observer (SK) at the Bankura and Burdwan Medical College in summer 2012. International Journal of Dermatology 2014, 53, 1119–1124
1123
1124
Tropical medicine rounds
Disseminated porokeratosis gigantea and Mibelli
Porokeratosis of Mibelli is uncommon in the cold climate of Western countries. Disseminated PM is a rarity,10 and a dermatologist may or may not see a case in his lifetime. However, in a tropical country such as India, porokeratosis is not so rare. Varied clinical presentations have been reported. Interestingly, the majority of them were from West Bengal. Geographic location or race does not play an important role in the distribution of porokeratosis.11 The higher incidence of porokeratosis may be attributed to the higher temperature of West Bengal due to sunrays falling vertically on the tropic of Cancer. In our patients, porokeratosis might have been influenced by a patient’s occupation, i.e., their increased outdoor activities in summer without regular use of sunscreen and inadequate clothing. Incidentally, we have not registered a single case of porokeratosis in the last two years in North Bengal Medical College, Susrutanagar, Darjeeling, India, where cold climatic condition prevails. However, in the same period two cases of disseminated superficial actinic porokeratosis, three cases of PM, one case of disseminated PM, and one case of linear porokeratosis, were noted at Bankura Medical College. The first case report of hypertrophic porokeratosis in India12 as well as the rare coexistence of different morphological variants of the hypertrophic variant were made from Bankura Medical College.13 Majority of disseminated PM that have been reported in association with other variants of porokeratosis were from West Bengal.13,14 Few cases of giant porokeratosis have been reported abroad. From India, only three cases14–16 have been documented, and two of them were from West Bengal.14,16 Incidentally, each of them describe single lesions of giant porokeratosis. After a thorough search of Indian and foreign literature, we could not find a case of disseminated giant porokeratosis. These three cases, noted by a single observer in the summer season of 2012, are reported for their rarity and interesting clinical presentation. To the best of our knowledge, our case of disseminated giant porokeratosis is the first report of its kind in India and probably in the world, as well. References
Koley et al.
2 Palleshi GM, Tochia D. Porokeratosis of Mibelli and superficial disseminated porokeratosis. J Cutan Pathol 2008; 35: 253–255. 3 Chernosky ME. Porokeratosis. Arch Dermatol 1986; 122: 869–870. 4 Ponticelli C, Bencini PL. Disseminated porokeratosis in immunosuppressed patients. Nephrol Dial Transplant 1996; 11: 2353–2354. 5 Alexis AF, Busam K, Myskowski PL. Porokeratosis of Mibelli following bone marrow transplantation. Int J Dermatol 2006; 45: 361–365. 6 Knoell KA, Patterson JW, Wilson BB. Sudden onset of disseminated porokeratosis of Mibelli in a renal transplant patient. J Am Acad Dermatol 1999; 41: 830–832. 7 Cha SH, Park HJ, Lee JY, et al. Atypical porokeratosis developing following bone marrow transplantation in a patient with myelodysplastic syndrome. Ann Dermatol 2010; 22: 206–208. 8 Handjani F, Shahbaz S, Aslani FS, et al. Porokeratosis of Mibelli with mutilation: a case report. Cutis 2010; 86: 77–80. 9 Ultraviolet station data based on satellite data (SCIAMACHY, GOME-2). http://www.temis.nl/ uvradiation/SCIA/stations_uv.html (accessed on November 27, 2012). 10 Vij A, Doherty SD, Leleux TM, et al. Disseminated porokeratosis of Mibelli: a case report. Dermatol Online J 2010; 16: 12. 11 Bloom D, Abramowitz EW. Porokeratosis Mibelli: report of three cases in one family; histologic studies. Arch Dermatol 1943; 47: 1–5. 12 Das S, Banerjee G. Porokeratosis masquerading as lupus vulgaris. Indian J Dermatol 2004; 49: 212–213. 13 Koley S, Sarkar J, Choudhary S, et al. Different morphological variants of hypertrophic porokeratosis and disseminated lesions of porokeratosis of Mibelli: a rare co-existence. Indian J Dermatol Venereol Leprol 2011; 77: 199–202. 14 Sarma N, Boler AK, Bhattacharya SR. Familial disseminated plaque type porokeratosis with multiple horns and squamous cell carcinoma involving anal skin. Indian J Dermatol Venereol Leprol 2009; 75: 551. 15 Hanumanthayya K, Magavi S, Tophakhane R, et al. Coexistence of disseminated superficial and giant porokeratosis of Mibelli with squamous cell carcinoma. Indian J Dermatol Venereol Leprol 2003; 69: 296–297. 16 Raychaudhury T, Valsamma DP. Giant porokeratosis. Indian J Dermatol Venereol Leprol 2011; 77: 601–602.
1 Lever WF, Lever SG. Histopathology of the Skin, 7th edn. Philadelphia: JB Lippincott Co, 1990: 70–72.
International Journal of Dermatology 2014, 53, 1119–1124
ª 2014 The International Society of Dermatology
Disseminated giant porokeratosis and porokeratosis of Mibelli in Bankura and Bardhaman districts, West Bengal, India Sankha Koley, MD, Rajesh Kumar Mandal, MD, and Chittaranjan Bar, MD
Department of Dermatology, North Bengal Medical College, Darjeeling, India Correspondence Sankha Koley MD North Bengal Medical College Subhankar Sarani Bankura 722101 West Bengal India E-mail: [email protected]
Abstract Porokeratosis of Mibelli (PM) is a rare genodermatosis. It is caused by proliferation of abnormal clones of epidermal cells in response to several stimuli, the most important of which is sunlight. Giant porokeratosis is thought to be a variant of PM. We report two cases of disseminated PM and one case of disseminated giant porokeratosis from the Bankura and Bardhaman districts of West Bengal in India; presenting to a single observer in the summer of 2012. Interestingly we have noted that the majority of cases of porokeratosis in India have been reported from West Bengal. Few patients with a single lesion of giant porokeratosis have been reported in world literature. As far as we know, this is the first case report of disseminated giant porokeratosis in world literature.
Introduction Porokeratosis of Mibelli (PM) is characterized clinically by one or more annular plaques with central atrophy and elevated hyperkeratotic ridge, called the cornoid lamella. Giant porokeratosis is considered by most authors as a morphological variant of PM. Sunlight is the most important aggravating factor in porokeratosis. Reports of disseminated lesions of PM and single lesion of giant porokeratosis have occasionally been made in Indian and other international literature. We report a rare case of disseminated giant porokeratosis and two cases of disseminated PM presenting to a single observer in Bankura and Bardhaman districts of West Bengal, India, in summer 2012. Case reports Case 1
A 44-year-old farmer from a remote village in Bankura district presented with widespread large annular plaques of variable sizes with well-defined border and grooved keratotic margin and central atrophy all over his body (Figs. 1 and 2). The initial lesion started 20 years ago as small coin-sized plaques over his leg that gradually increased in size in centrifugal expansion. After three years, a similar lesion appeared over his face and enlarged slowly to involve his right eye, leading to cicatriª 2014 The International Society of Dermatology
cial ectropion (Fig. 1). This lesion further extended to involve the scalp, with subsequent loss of hairs. Subsequently more such lesions of variable sizes appeared all over his body. He worked in fields for long hours in sunlight with bare skin without any protection. There was no family history of this disease. Based on these findings, he was given a provisional diagnosis of disseminated giant porokeratosis. Case 2
A middle-aged female laborer from a remote village in Bardhaman district presented with multiple small hyperpigmented annular plaques of different sizes, each with a grooved keratotic margin, diffusely spread all over her body (Fig. 3). The lesions started four years previously and gradually increased in number. The lesions were mostly over the extensor surface of her upper limbs, face, and upper back. Her elder sister and mother had similar lesions. As a laborer, she had to work outside under sunlight for 10 hours daily. With this clinical presentation, she was given a provisional diagnosis of disseminated PM. Case 3
A 22-year-old farmer from a remote village in Bankura district presented with multiple small hyperpigmented annular plaques of different sizes and a grooved keratotic margin over the forehead, upper chest, and upper back International Journal of Dermatology 2014, 53, 1119–1124
1119
1120
Tropical medicine rounds
Disseminated porokeratosis gigantea and Mibelli
Koley et al.
Figure 1 Case 1: giant porokeratosis causing cicatricial ectropion of right eye and scarring alopecia
Figure 2 Case 1: lesions of giant porokeratosis all over the body International Journal of Dermatology 2014, 53, 1119–1124
ª 2014 The International Society of Dermatology
Koley et al.
Disseminated porokeratosis gigantea and Mibelli
Tropical medicine rounds
Figure 3 Case 2: disseminated lesions of porokeratosis of Mibelli on face, bilateral hands, abdomen, and back
(Fig. 4). The lesions started three months prior and gradually increased in size and number. He also had a history of prolonged sunlight exposure. There was no family history of similar disease. He was given a provisional diagnosis of disseminated PM. All the patients were thoroughly investigated. All routine tests were within normal limits. None of the patients had HIV or any other kind of immunosuppression, including diabetes. Neither of them received any immunosuppressive drug for long. They were either farmer or laborer by profession and worked 8–10 hours under the sun on most days. All of them gave a history of exaggeration of lesions in summer. All the cases were clinically diagnosed by well-defined, keratotic border of lesions with the presence of gutter-like groove on their summits. They were further confirmed ª 2014 The International Society of Dermatology
histologically. Biopsy specimens revealed characteristic cornoid lamella, absent granular layer below the invagination, and mild chronic inflammatory cell infiltration in the upper dermis (Figs. 5a,b, 6a,b, and 7a,b). All of them were advised strict photoprotection, emollients in addition to treatment noted in Table 1. Comments Porokeratosis is a rare genodermatosis featuring abnormal epidermal keratinization. It is transmitted as an autosomal dominant trait with variable penetrance.1 Classically, five clinical variants are recognized; disseminated superficial porokeratosis and its actinic variant (most common), classic porokeratosis of Mibelli (second most common), punctate porokeratosis, porokeratosis palmaris et plantaris International Journal of Dermatology 2014, 53, 1119–1124
1121
1122
Tropical medicine rounds
Koley et al.
Disseminated porokeratosis gigantea and Mibelli
Figure 4 Case 3: Disseminated lesions of porokeratosis of Mibelli on forehead and upper back
Table 1 Clinical presentations of three cases Case no.
1 (Figs. 1 and 2)
2 (Fig. 3)
3 (Fig. 4)
Date of presentation Age (in years) Sex Address Occupation Distribution Duration Size (diameter) and site of larger lesions Family history Diagnosis Biopsy location Histopathology finding
April 11, 2012
April 27, 2012
June 5, 2012
44 M Susunia village, Bankura Farmer All over body 20 years – right leg; 17 years – on head 35–40 cm on right face and back of head; 16–22 cm on posterior legs
27 F Rasikpur village, Bardhaman Laborer Face, bilateral hands, abdomen and back 4 years 3–5 cm on left neck and upper back
22 M Chatna, Bankura Farmer Forehead, upper chest and back 3 months 3–4 on mid back
Absent Disseminated giant porokeratosis Right back Cornoid lamella, absent granular layer, mild chronic dermal inflammatory infiltrates Isotretinoin 20 mg per day, isotretinoin 0.05% cream at night
Similar lesion in elder sister and mother Disseminated PM Left neck Cornoid lamella with diminished granular layer and mild dermal lymphohistiocytic infiltrate Topical halobetasol 0.05% ointment daytime and isotretinoin 0.05% cream at bedtime Mild improvement after 3 months
Absent Disseminated PM Left back Cornoid lamella, diminished granular layer and mild dermal inflammatory infiltrate Topical halobetasol 0.05% ointment twice daily
Treatment
Follow-up
Lost to follow-up
Considerable improvement after 3 months
F, female; M, male.
disseminata, and linear porokeratosis.2 The border is usually more than 1 mm in height and contains a thread-like groove. Giant porokeratosis, where the lesion may vary from 10 to 20 cm in diameter and the hyperkeratotic walls raised greater than 1 cm, is considered a morphological variant of PM by most authors, while others consider it a distinct entity. International Journal of Dermatology 2014, 53, 1119–1124
There is a hypothesis that porokeratosis is triggered by proliferation of abnormal clones of epidermal cells in response to several stimuli, the most important of which are sunlight and artificial ultraviolet (UV).3 Non-familial disseminated superficial porokeratosis and single plaque PM have been documented to occur in association with immunosuppression. While sun exposure plays an important pathogenetic role ª 2014 The International Society of Dermatology
Koley et al.
Disseminated porokeratosis gigantea and Mibelli
(a)
Tropical medicine rounds
(b)
Figure 5 (a) Histopathological finding in case 1: cornoid lamella, absent granular layer below the invagination and mild chronic inflammatory cell infiltration in upper dermis (hematoxylin and eosin, 940). (b) Magnified view of (a) (hematoxylin and eosin, 9400)
(a)
(b)
Figure 6 (a) Keratin-filled invagination of epidermis with parakeratotic cells (cornoid lamella), diminished granular layer with mild dermal lymphohistiocytic infiltrate (hematoxylin and eosin, 9100). (b) Magnified view of (a) (hematoxylin and eosin, 9400)
(a)
(b)
Figure 7 (a) Cornoid lamella, diminished granular layer, and mild dermal inflammatory infiltrate (hematoxylin and eosin, 940). (b) Magnified view of (a) (hematoxylin and eosin, 9400)
in idiopathic cases, it does not seem to be essential for the development of porokeratosis in immunosuppressed patients.4 Disseminated PM has been reported following renal5 and bone marrow transplantation for leukemia, nonHodgkin’s lymphoma,6 and myelodysplastic syndrome.7 However, our patient had no history of transplantation or systemic immunosuppression. Porokeratosis may cause damage or even mutilation of body parts.8 In case 1, a lesion of giant porokeratosis had caused cicatricial ectropion of the right eye and scarring alopecia of the head. The UV index is an international standard measurement of the strength of UV radiation from the sun at a ª 2014 The International Society of Dermatology
particular place on a particular day. During the summer, scorching heat prevails over West Bengal, a state in the eastern part of India. Bankura and Bardhaman are two of the seven districts of the Burdwan Division of West Bengal. The data of the daily UV index within the last seven years (January 1, 2006, to October 31, 2012) collected from an internet website denotes that the highest UV index prevails from mid-April to mid-July.9 Last summer, mercury was as high as 47 °C in Bardhaman and Bankura districts. All three cases were presented to a single observer (SK) at the Bankura and Burdwan Medical College in summer 2012. International Journal of Dermatology 2014, 53, 1119–1124
1123
1124
Tropical medicine rounds
Disseminated porokeratosis gigantea and Mibelli
Porokeratosis of Mibelli is uncommon in the cold climate of Western countries. Disseminated PM is a rarity,10 and a dermatologist may or may not see a case in his lifetime. However, in a tropical country such as India, porokeratosis is not so rare. Varied clinical presentations have been reported. Interestingly, the majority of them were from West Bengal. Geographic location or race does not play an important role in the distribution of porokeratosis.11 The higher incidence of porokeratosis may be attributed to the higher temperature of West Bengal due to sunrays falling vertically on the tropic of Cancer. In our patients, porokeratosis might have been influenced by a patient’s occupation, i.e., their increased outdoor activities in summer without regular use of sunscreen and inadequate clothing. Incidentally, we have not registered a single case of porokeratosis in the last two years in North Bengal Medical College, Susrutanagar, Darjeeling, India, where cold climatic condition prevails. However, in the same period two cases of disseminated superficial actinic porokeratosis, three cases of PM, one case of disseminated PM, and one case of linear porokeratosis, were noted at Bankura Medical College. The first case report of hypertrophic porokeratosis in India12 as well as the rare coexistence of different morphological variants of the hypertrophic variant were made from Bankura Medical College.13 Majority of disseminated PM that have been reported in association with other variants of porokeratosis were from West Bengal.13,14 Few cases of giant porokeratosis have been reported abroad. From India, only three cases14–16 have been documented, and two of them were from West Bengal.14,16 Incidentally, each of them describe single lesions of giant porokeratosis. After a thorough search of Indian and foreign literature, we could not find a case of disseminated giant porokeratosis. These three cases, noted by a single observer in the summer season of 2012, are reported for their rarity and interesting clinical presentation. To the best of our knowledge, our case of disseminated giant porokeratosis is the first report of its kind in India and probably in the world, as well. References
Koley et al.
2 Palleshi GM, Tochia D. Porokeratosis of Mibelli and superficial disseminated porokeratosis. J Cutan Pathol 2008; 35: 253–255. 3 Chernosky ME. Porokeratosis. Arch Dermatol 1986; 122: 869–870. 4 Ponticelli C, Bencini PL. Disseminated porokeratosis in immunosuppressed patients. Nephrol Dial Transplant 1996; 11: 2353–2354. 5 Alexis AF, Busam K, Myskowski PL. Porokeratosis of Mibelli following bone marrow transplantation. Int J Dermatol 2006; 45: 361–365. 6 Knoell KA, Patterson JW, Wilson BB. Sudden onset of disseminated porokeratosis of Mibelli in a renal transplant patient. J Am Acad Dermatol 1999; 41: 830–832. 7 Cha SH, Park HJ, Lee JY, et al. Atypical porokeratosis developing following bone marrow transplantation in a patient with myelodysplastic syndrome. Ann Dermatol 2010; 22: 206–208. 8 Handjani F, Shahbaz S, Aslani FS, et al. Porokeratosis of Mibelli with mutilation: a case report. Cutis 2010; 86: 77–80. 9 Ultraviolet station data based on satellite data (SCIAMACHY, GOME-2). http://www.temis.nl/ uvradiation/SCIA/stations_uv.html (accessed on November 27, 2012). 10 Vij A, Doherty SD, Leleux TM, et al. Disseminated porokeratosis of Mibelli: a case report. Dermatol Online J 2010; 16: 12. 11 Bloom D, Abramowitz EW. Porokeratosis Mibelli: report of three cases in one family; histologic studies. Arch Dermatol 1943; 47: 1–5. 12 Das S, Banerjee G. Porokeratosis masquerading as lupus vulgaris. Indian J Dermatol 2004; 49: 212–213. 13 Koley S, Sarkar J, Choudhary S, et al. Different morphological variants of hypertrophic porokeratosis and disseminated lesions of porokeratosis of Mibelli: a rare co-existence. Indian J Dermatol Venereol Leprol 2011; 77: 199–202. 14 Sarma N, Boler AK, Bhattacharya SR. Familial disseminated plaque type porokeratosis with multiple horns and squamous cell carcinoma involving anal skin. Indian J Dermatol Venereol Leprol 2009; 75: 551. 15 Hanumanthayya K, Magavi S, Tophakhane R, et al. Coexistence of disseminated superficial and giant porokeratosis of Mibelli with squamous cell carcinoma. Indian J Dermatol Venereol Leprol 2003; 69: 296–297. 16 Raychaudhury T, Valsamma DP. Giant porokeratosis. Indian J Dermatol Venereol Leprol 2011; 77: 601–602.
1 Lever WF, Lever SG. Histopathology of the Skin, 7th edn. Philadelphia: JB Lippincott Co, 1990: 70–72.
International Journal of Dermatology 2014, 53, 1119–1124
ª 2014 The International Society of Dermatology
