Letters to the Editor
Bullous prurigo pigmentosa in a pregnant woman with hyperemesis gravidarum Dear Editor, Prurigo pigmentosa is a relatively rare inflammatory dermatosis, mainly seen in Japanese patients, and is clinically characterized by pruritic erythematous papules and subsequent reticular pigmentation. As an atypical presentation, prurigo pigmentosa with bullous formation has been reported in several cases.1–3 We herein describe a case of prurigo pigmentosa with vesiculobullous formation occurring in a pregnant woman with severe hyperemesis gravidarum and ketonuria. A 30-year-old woman was hospitalized due to severe hyperemesis gravidarum. She was referred to the dermatology department, complaining of itchy eruptions, when she was 10 weeks pregnant. She had lost 7 kg in bodyweight compared with pre-pregnancy, and her body mass index was 18.5. Physical examination revealed erythematous plaques on the abdomen and back. Furthermore, reticular erythema with tense vesicles were scattered on the nuchal region (Fig. 1a,b). A skin biopsy revealed subcorneal bulla containing dyskeratotic cells and prominent neutrophils, and liquefaction degeneration of the basal layer (Fig. 1c). Infiltration by a number of mononuclear cells in the subepidermal layers was also
(a)
(c)
(b)
(d)
observed. Laboratory examination showed that complete blood count, liver and kidney function, C-reactive protein, antinuclear antibody, anti-desmogleins and anti-BP180 were all within normal ranges, and hemoglobin A1c was 5.0%. However, urinary examination revealed ketone of 4+. Along with the improvement of her general condition by fluid therapy and bedrest, ketonuria disappeared, and the skin lesions were also improved leaving reticular pigmentation after 1 month (Fig. 1d). Although the etiology of prurigo pigmentosa is still unknown, several papers have suggested the role of ketonuria associated with diabetes mellitus, diet and fasting. By contrast, only two cases of prurigo pigmentosa in association with pregnancy have been reported.4,5 Both cases developed prurigo pigmentosa 13 weeks into their pregnancies. One of the cases suffered from severe vomiting and lost 7% of bodyweight. Examination of this patient revealed elevated urine ketones,4 which is very similar to our case. In the present case, inadequate oral intake due to severe hyperemesis gravidarum led to ketosis, which subsequently caused prurigo pigmentosa. To date, no similar cases have been reported. Prurigo pigmentosa is usually treated with minocycline, dapsone and sulfamethoxazole. By contrast, our case improved by treatment with rest and fluid therapy along with disappearance of ketonuria, which suggests that the development of prurigo pigmentosa is attributable to ketosis due to severe hyperemesis gravidarum. There have been several reported cases of bullous prurigo pigmentosa.1–3 Extensive inflammation with intercellular and intracellular edema, basal liquefaction and papillary dermal edema may result in vesiculobullous lesions. The pathomechanism of bullous formation is unknown, however, marked interand intracellular edema may induce bullous changes. Bullous lesion of prurigo pigmentosa may be induced by intense rubbing, which however is unlikely in our case because there was no scratch mark and bullous lesions were seen on sites where the fingers are difficult to reach. This is the first case of bullous prurigo pigmentosa in close association with ketosis. Prurigo pigmentosa should be recognized as a cutaneous disorder which occurs during pregnancy.
CONFLICT OF INTEREST: Figure 1. (a) Multiple erythematous papules on the nuchal region and upper back, (b) containing bulla. (c) Intraepidermal bulla with a number of neutrophils, liquefaction and mononuclear cell infiltrates in the papillary dermis (hematoxylin–eosin, original magnification 9 200). (d) Reticular pattern pigmentation after improvement.
None declared.
Yuka HANAMI, Toshiyuki YAMAMOTO Department of Dermatology, Fukushima Medical University, Fukushima, Japan doi: 10.1111/1346-8138.12795
Correspondence: Yuka Hanami, Ph.D., M.D., Department of Dermatology, School of Medicine, Fukushima Medical University, 1 Hikarigaoka, Fukushima, Fukushima 960-1295, Japan. Email: [email protected]
436
© 2015 Japanese Dermatological Association
Letters to the Editor
REFERENCES 1 Kubota Y, Koga T, Nakayama J. Bullous prurigo pigmentosa and diabetes. Eur J Dermatol 1998; 8: 439–441. 2 De Francesco V, Quinkenstein E, Mariuzzi L, Frattasio A, Pillon B, Patrone P. Bullous prurigo pigmentosa. Eur J Dermatol 2006; 16: 184–186.
3 Kim SK, Kang HY, Lee ES. Bullous prurigo pigmentosa. Int J Dermatol 2007; 46: 888–890. 4 Leone L, Colato C, Girolomoni G. Prurigo pigmentosa in a pregnant woman. Int J Gynaecol Obstet 2007; 98: 261–262. 5 Jang MS, Baek JW, Kang DY, Kang JS, Kim ST, Suh KS. Successful treatment with narrowband UVB phototherapy in prurigo pigmentosa associated with pregnancy. Eur J Dermatol 2011; 21: 634–635.
Unilateral keratosis pilaris occurring on linear hypopigmentation patches: A new variant of keratosis pilaris in an Asian? Dear Editor, Keratosis pilaris (KP) is a common inherited disorder of follicular hyperkeratosis, which is characterized by small, folliculocentric, keratotic papules that may have surrounding erythema.1 In this study, we describe a rare Asian case of unilateral keratosis pilaris occurring on linear hypopigmentation patches. A 25-year-old patient presented with asymptomatic, rough, follicular papules occurring on pre-existent hypopigmentations distributed along Blaschko’s lines on the right lateral chest wall since his early months of infancy (Fig. 1a). His past medical history was unremarkable. The patient had no indication of atopy and was taking no medications. The patient’s father had similar lesions on the bilateral extensor surfaces of the upper arms. Physical examination revealed normal dermatoglyph and no epidermal atrophy. Quite a few small papules had perifollicular erythematous halo and a circular hair shaft embedded in the superficial epidermis (Fig. 1b). A skin biopsy specimen revealed hyperkeratosis, reduction of melanocytes from the basal lamina region (Fig. 1c,d), a dilated follicular infundibulum with a keratotic plug surrounded by a sparse lymphohistiocytic infiltrate and discrete fibrosis (Fig. 1e). Based on the clinical manifestations and histopathological features, the diagnosis
(a)
Figure 1. (a) Follicular papules occurring on linear hypopigmentation patches. (b) Perifollicular erythematous halo and circular hair shafts. (c) Hyperkeratosis and dilated follicular infundibulum with a keratotic plug (hematoxylin–eosin [HE], original magnification 940). (d) Reduction of melanocytes from the basal lamina region (HE, 9400). (e) Perifollicular sparse lymphohistiocytic infiltrate (HE, 9400).
was unilateral keratosis pilaris occurring on linear hypopigmentation patches following the lines of Blaschko. Keratosis pilaris is the most common form of follicular keratosis. It occurs so frequently that it is often considered a normal variant, making prevalence estimates difficult.2 It can be associated with various diseases, commonly with atopic dermatitis and ichthyosis vulgaris, but also with metabolic and endocrine diseases such as malnutrition, obesity, diabetes mellitus, hypothyroidism, hyperandrogenism and hyperadrenocorticism.3 KP is also associated with a number of syndromes such as Noonan, Greither and Down syndromes, although it is not the dominant feature.2 In this case, the most distinctive presentation is unilateral KP occurring on linear hypopigmentation patches. It is universally acknowledged that the lesions of KP originate from normal skin-colored surface. To this day and to our knowledge, the phenomena has not been reported before. There is very limited published work describing the etiopathogenesis of KP. Thomas and Khopkar4 proposed that a disorder of the keratinocytes caused by a mutation in the filaggrin gene is responsible for inducing both hyperkeratosis and inflammatory changes in KP. But this hypothesis could not explain skin hypopigmentation in this case. Kiritsi et al. reported a case of severe
(b)
(d)
(c)
(e)
Correspondence: Wei Lai, M.D., Department of Dermatology, the Third Affiliated Hospital, Sun Yat-sen University, 600 Tianhe Road, Guangzhou 510630, China. Email: [email protected]
© 2015 Japanese Dermatological Association
437
Bullous prurigo pigmentosa in a pregnant woman with hyperemesis gravidarum Dear Editor, Prurigo pigmentosa is a relatively rare inflammatory dermatosis, mainly seen in Japanese patients, and is clinically characterized by pruritic erythematous papules and subsequent reticular pigmentation. As an atypical presentation, prurigo pigmentosa with bullous formation has been reported in several cases.1–3 We herein describe a case of prurigo pigmentosa with vesiculobullous formation occurring in a pregnant woman with severe hyperemesis gravidarum and ketonuria. A 30-year-old woman was hospitalized due to severe hyperemesis gravidarum. She was referred to the dermatology department, complaining of itchy eruptions, when she was 10 weeks pregnant. She had lost 7 kg in bodyweight compared with pre-pregnancy, and her body mass index was 18.5. Physical examination revealed erythematous plaques on the abdomen and back. Furthermore, reticular erythema with tense vesicles were scattered on the nuchal region (Fig. 1a,b). A skin biopsy revealed subcorneal bulla containing dyskeratotic cells and prominent neutrophils, and liquefaction degeneration of the basal layer (Fig. 1c). Infiltration by a number of mononuclear cells in the subepidermal layers was also
(a)
(c)
(b)
(d)
observed. Laboratory examination showed that complete blood count, liver and kidney function, C-reactive protein, antinuclear antibody, anti-desmogleins and anti-BP180 were all within normal ranges, and hemoglobin A1c was 5.0%. However, urinary examination revealed ketone of 4+. Along with the improvement of her general condition by fluid therapy and bedrest, ketonuria disappeared, and the skin lesions were also improved leaving reticular pigmentation after 1 month (Fig. 1d). Although the etiology of prurigo pigmentosa is still unknown, several papers have suggested the role of ketonuria associated with diabetes mellitus, diet and fasting. By contrast, only two cases of prurigo pigmentosa in association with pregnancy have been reported.4,5 Both cases developed prurigo pigmentosa 13 weeks into their pregnancies. One of the cases suffered from severe vomiting and lost 7% of bodyweight. Examination of this patient revealed elevated urine ketones,4 which is very similar to our case. In the present case, inadequate oral intake due to severe hyperemesis gravidarum led to ketosis, which subsequently caused prurigo pigmentosa. To date, no similar cases have been reported. Prurigo pigmentosa is usually treated with minocycline, dapsone and sulfamethoxazole. By contrast, our case improved by treatment with rest and fluid therapy along with disappearance of ketonuria, which suggests that the development of prurigo pigmentosa is attributable to ketosis due to severe hyperemesis gravidarum. There have been several reported cases of bullous prurigo pigmentosa.1–3 Extensive inflammation with intercellular and intracellular edema, basal liquefaction and papillary dermal edema may result in vesiculobullous lesions. The pathomechanism of bullous formation is unknown, however, marked interand intracellular edema may induce bullous changes. Bullous lesion of prurigo pigmentosa may be induced by intense rubbing, which however is unlikely in our case because there was no scratch mark and bullous lesions were seen on sites where the fingers are difficult to reach. This is the first case of bullous prurigo pigmentosa in close association with ketosis. Prurigo pigmentosa should be recognized as a cutaneous disorder which occurs during pregnancy.
CONFLICT OF INTEREST: Figure 1. (a) Multiple erythematous papules on the nuchal region and upper back, (b) containing bulla. (c) Intraepidermal bulla with a number of neutrophils, liquefaction and mononuclear cell infiltrates in the papillary dermis (hematoxylin–eosin, original magnification 9 200). (d) Reticular pattern pigmentation after improvement.
None declared.
Yuka HANAMI, Toshiyuki YAMAMOTO Department of Dermatology, Fukushima Medical University, Fukushima, Japan doi: 10.1111/1346-8138.12795
Correspondence: Yuka Hanami, Ph.D., M.D., Department of Dermatology, School of Medicine, Fukushima Medical University, 1 Hikarigaoka, Fukushima, Fukushima 960-1295, Japan. Email: [email protected]
436
© 2015 Japanese Dermatological Association
Letters to the Editor
REFERENCES 1 Kubota Y, Koga T, Nakayama J. Bullous prurigo pigmentosa and diabetes. Eur J Dermatol 1998; 8: 439–441. 2 De Francesco V, Quinkenstein E, Mariuzzi L, Frattasio A, Pillon B, Patrone P. Bullous prurigo pigmentosa. Eur J Dermatol 2006; 16: 184–186.
3 Kim SK, Kang HY, Lee ES. Bullous prurigo pigmentosa. Int J Dermatol 2007; 46: 888–890. 4 Leone L, Colato C, Girolomoni G. Prurigo pigmentosa in a pregnant woman. Int J Gynaecol Obstet 2007; 98: 261–262. 5 Jang MS, Baek JW, Kang DY, Kang JS, Kim ST, Suh KS. Successful treatment with narrowband UVB phototherapy in prurigo pigmentosa associated with pregnancy. Eur J Dermatol 2011; 21: 634–635.
Unilateral keratosis pilaris occurring on linear hypopigmentation patches: A new variant of keratosis pilaris in an Asian? Dear Editor, Keratosis pilaris (KP) is a common inherited disorder of follicular hyperkeratosis, which is characterized by small, folliculocentric, keratotic papules that may have surrounding erythema.1 In this study, we describe a rare Asian case of unilateral keratosis pilaris occurring on linear hypopigmentation patches. A 25-year-old patient presented with asymptomatic, rough, follicular papules occurring on pre-existent hypopigmentations distributed along Blaschko’s lines on the right lateral chest wall since his early months of infancy (Fig. 1a). His past medical history was unremarkable. The patient had no indication of atopy and was taking no medications. The patient’s father had similar lesions on the bilateral extensor surfaces of the upper arms. Physical examination revealed normal dermatoglyph and no epidermal atrophy. Quite a few small papules had perifollicular erythematous halo and a circular hair shaft embedded in the superficial epidermis (Fig. 1b). A skin biopsy specimen revealed hyperkeratosis, reduction of melanocytes from the basal lamina region (Fig. 1c,d), a dilated follicular infundibulum with a keratotic plug surrounded by a sparse lymphohistiocytic infiltrate and discrete fibrosis (Fig. 1e). Based on the clinical manifestations and histopathological features, the diagnosis
(a)
Figure 1. (a) Follicular papules occurring on linear hypopigmentation patches. (b) Perifollicular erythematous halo and circular hair shafts. (c) Hyperkeratosis and dilated follicular infundibulum with a keratotic plug (hematoxylin–eosin [HE], original magnification 940). (d) Reduction of melanocytes from the basal lamina region (HE, 9400). (e) Perifollicular sparse lymphohistiocytic infiltrate (HE, 9400).
was unilateral keratosis pilaris occurring on linear hypopigmentation patches following the lines of Blaschko. Keratosis pilaris is the most common form of follicular keratosis. It occurs so frequently that it is often considered a normal variant, making prevalence estimates difficult.2 It can be associated with various diseases, commonly with atopic dermatitis and ichthyosis vulgaris, but also with metabolic and endocrine diseases such as malnutrition, obesity, diabetes mellitus, hypothyroidism, hyperandrogenism and hyperadrenocorticism.3 KP is also associated with a number of syndromes such as Noonan, Greither and Down syndromes, although it is not the dominant feature.2 In this case, the most distinctive presentation is unilateral KP occurring on linear hypopigmentation patches. It is universally acknowledged that the lesions of KP originate from normal skin-colored surface. To this day and to our knowledge, the phenomena has not been reported before. There is very limited published work describing the etiopathogenesis of KP. Thomas and Khopkar4 proposed that a disorder of the keratinocytes caused by a mutation in the filaggrin gene is responsible for inducing both hyperkeratosis and inflammatory changes in KP. But this hypothesis could not explain skin hypopigmentation in this case. Kiritsi et al. reported a case of severe
(b)
(d)
(c)
(e)
Correspondence: Wei Lai, M.D., Department of Dermatology, the Third Affiliated Hospital, Sun Yat-sen University, 600 Tianhe Road, Guangzhou 510630, China. Email: [email protected]
© 2015 Japanese Dermatological Association
437
