Letters to the Editor
dermatitis, asthma, allergic rhinitis, food allergy, allergic conjunctivitis and eosinophilic esophagitis.3 However, little is known about TSLP expression in inflammatory PSS. We report a 3-week-old girl affected by inflammatory PSS who had a homozygous large deletion in the 6p21.3 region encompassing the CDSN gene, which abrogated corneodesmosin expression. Her clinical and pathophysiological data have been reported elsewhere.4 She developed generalized erythematous rash immediately after birth, followed by superficial peeling and ichthyosiform erythroderma. Laboratory studies revealed marked eosinophilia and a rapid elevation of total serum immunoglobulin (Ig)E levels during early infancy (Fig. 1a, b). Although allergen-specific IgE were initially all negative, the patient was sensitized with multiple inhalant and food allergens with age.4 Eosinophil counts were gradually decreased after improvement of the initial skin lesions. The patient developed IgE-mediated fish allergy and atopic dermatitis. These findings were consistent with the susceptibility to allergic diseases in inflammatory PSS. To elucidate the underlying mechanism, TSLP expression was analyzed by immunohistochemical staining. As shown in Figure 1(c), a skin biopsy specimen from a normal control showed negative staining in the granular layers, whereas TSLP was highly expressed in the patient’s skin biopsy specimen obtained at 3 weeks of age. The staining pattern was similar to those found in atopic dermatitis and Netherton syndrome.5 These findings suggest that increased expression of epidermal TSLP in inflammatory PSS may result in a Th2 adaptive immune response to environmental allergens like atopic dermatitis and Netherton syndrome. Further studies will be necessary to assess induction and regulation of TSLP expression and its downstream molecules in inflammatory PSS. The characterization of barrier-related defects and local immune phenotype
may lead to a better understanding of human disorders associated with skin barrier defects.
ACKNOWLEDGEMENTS: This work was supported by a Grant-in-Aid for Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology of Japan; and a grant from the Ministry of Health, Labor and Welfare of Japan, Tokyo. CONFLICT OF INTEREST:
None declared.
Taizo WADA,1 Tomoko TOMA,1 Yasuhito HAMAGUCHI,2 Akihiro YACHIE1 1
Departments of Pediatrics, and 2 Dermatology, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan doi: 10.1111/1346-8138.12449
REFERENCES 1 Oji V, Eckl KM, Aufenvenne K et al. Loss of corneodesmosin leads to severe skin barrier defect, pruritus, and atopy: unraveling the peeling skin disease. Am J Hum Genet 2010; 87: 274–281. 2 De Benedetto A, Kubo A, Beck LA. Skin barrier disruption: a requirement for allergen sensitization? J Invest Dermatol 2012; 132: 949– 963. 3 Ziegler SF. Thymic stromal lymphopoietin and allergic disease. J Allergy Clin Immunol 2012; 130: 845–852. 4 Wada T, Matsuda Y, Muraoka M et al. Alu-mediated large deletion of the CDSN gene as a cause of peeling skin disease. Clin Genet 2013; (in press). 5 Soumelis V, Reche PA, Kanzler H et al. Human epithelial cells trigger dendritic cell mediated allergic inflammation by producing TSLP. Nat Immunol 2002; 3: 673–680.
Unilateral linear punctate palmar keratoderma Dear Editor, Palmoplantar keratodermas (PPKs) are a heterogeneous group of disorders characterised by thickening of the epidermis of the palms and soles. They are genetically and clinically heterogeneous. Autosomal recessive, autosomal dominant, X-linked, and acquired forms have been described. Clinically, three forms can be seen: diffuse, focal, and punctate. Punctate linear palmoplantar keratoderma is a rare entity.1–3 Unilateral disease with involvement of only one hand has not been reported yet, to our knowledge. Here we present a 15-year-old boy who was enrolled at our outpatient clinic with palmar linear hyperkeratosis. In a dermatologic examination there was a linear yellow-red palmar hyperkeratotic plaque in the hypothenar area (Fig. 1a,b). The lesion developed when the patient was 4 years old and had
grown over time. There were no similar lesions on other parts of the body. The boy’s complaint was its appearance and itchiness. There was no involvement in the right hand, or right and left soles. Complete blood count, urea, creatinine, liver function tests, stool for occult blood, sedimentation and chest x-ray were normal. There were no similar lesions among the patient’s family members. We took two biopsies for confirmation and the result was keratoderma. In the biopsy, compact orthohyperkeratosis, hypergranulosis, and slight acanthosis were seen (Fig. 1c). In the differential diagnosis linear punctate porokeratosis, arsenical keratosis, striate PPK was considered. Our case was diagnosed histopathologically. Linear punctate PPK can be seen in all races and its rates are the same in both sexes.4 Generally, it is seen between the ages of 10-30 years.1–3 PPKs can be inherited or acquired.
Correspondence: Dilek Biyik Ozkaya Adnan, Menderes Bulvarı, Vatan caddesi 34093 Bezmialem Vakıf Universitesi Tıp Fak€ ultesi Deti ve Zuhrevi Hastalıkları Istanbul,Turkey. Email: [email protected]
© 2014 Japanese Dermatological Association
449
Letters to the Editor
(a)
(c)
(b)
Figure 1. (a, b) Clinical presentation of the lesion. (c) Prominent compact orthohyperkeratosis, hypergranulosis, and slight acanthosis of the epidermis, consistent with keratoderma (H&E 9 20).
While its aetiology is unknown, external factors such as trauma may trigger the disease.1,3 Generally, the involvement is bilateral. Sharma et al. reported a case of unilateral linear PPK, which was the first such case.1 In their case, only the left sole and hand were involved. Treatment is generally symptomatic. Topical keratolytics (e.g. salicylic acid, lactic acid), steroids, tretinoin, and psoralens can be used. Effective treatment with acitretin has been reported. Our case did not want to use systemic therapy. We treated him with 0.025% topical tretinoin for 1 month; the lesion got thinner but did not completely heal. The lesion recurred after 1 month. The patient didn’t want to use topical treatments and he didn’t use them regularly, so we suggested oral retinoids to our patient. The aetiology of this disease is unknown but genetic and environmental factors may trigger the disease. It has been described in a few autosomal dominant pedigrees. Martinez et al. reported that they have identified the locus for type I punctate PPK on chromosome 15q22–q24.5 In the family members of our patient there was no known skin disease, so our patient may have a mutation. PPKs can be associated with malignancies, so PPK patients must be examined and followed up carefully. Our patient had no other disease, and none of his relatives had similar lesions. Our patient’s laboratory tests and clinical examination were normal. All patients must be followed up carefully because of the association with malignancies.1
CONFLICT OF INTEREST:
None.
Dilek BIYIK OZKAYA,1 Betul TAS,2 Ozlem SU,1 Alkım UNAL CAKITER,1 Zeynep TOSUNER,3 Cuyan DEMIRKESEN,4 Nahide ONSUN1 1
Department of Dermatovenerology, Medical Faculty, Bezmialem Vakif University, 2Department of Dermatovenerology, Bagcılar Research and Training Hospital, 3Department of Pathology, Medical Faculty, Bezmialem Vakif University, and 4Department of Pathology, Istanbul University Cerrahpasßa Medical Faculty, Istanbul, Turkey doi: 10.1111/1346-8138.12485
REFERENCES 1 Sharma S, Barman KD, Garg VK, Jain S. Unilateral linear punctate palmoplantar keratoderma. Indian J Dermatol Venereol Leprol 2012; 78: 85–8. 2 O’Toole A, O’Malley M. Unilateral keratoderma in a mother and her son. J Cutan Med Surg 1012; 16: 288–90. PubMed 3 Oztas P, Alli N, Polat M et al. Punctate palmoplantar keratoderma (Brauer-Buschke-Fischer syndrome). Am J Clin Dermatol 2007; 8: 113–6. PubMed 4 Erkek E, Erdogan S, Tuncez F et al. Type I hereditary punctate keratoderma associated with widespread lentigo simplex and successfully treated with low-dose oral acitretin. Arch Dermatol 2006; 142: 1076– 7. PubMed 5 Martinez-Mir A, Zlotogorski A, Londono D et al. Identification of a locus for type 1 punctate palmoplantar keratoderma on chromosome 15q22-q24. J Med Genet 2003; 40: 872–8.
Late-onset self-healing Langerhans cell histiocytosis in a patient with atopic dermatitis Dear Editor, Self-healing Langerhans cell histiocytosis (SHLCH) is a variant of cutaneous Langerhans cell (LC) histiocytosis initially seen at
birth or just after birth. SHLCH is characterized by eruptions of multiple papules and nodules that resolve spontaneously without involvement of other organs.1 In 1973, Hashimoto and
Correspondence: Takeshi Nakahara, M.D., Ph.D., Division of Skin Surface Sensing, Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. Email: [email protected]
450
© 2014 Japanese Dermatological Association
dermatitis, asthma, allergic rhinitis, food allergy, allergic conjunctivitis and eosinophilic esophagitis.3 However, little is known about TSLP expression in inflammatory PSS. We report a 3-week-old girl affected by inflammatory PSS who had a homozygous large deletion in the 6p21.3 region encompassing the CDSN gene, which abrogated corneodesmosin expression. Her clinical and pathophysiological data have been reported elsewhere.4 She developed generalized erythematous rash immediately after birth, followed by superficial peeling and ichthyosiform erythroderma. Laboratory studies revealed marked eosinophilia and a rapid elevation of total serum immunoglobulin (Ig)E levels during early infancy (Fig. 1a, b). Although allergen-specific IgE were initially all negative, the patient was sensitized with multiple inhalant and food allergens with age.4 Eosinophil counts were gradually decreased after improvement of the initial skin lesions. The patient developed IgE-mediated fish allergy and atopic dermatitis. These findings were consistent with the susceptibility to allergic diseases in inflammatory PSS. To elucidate the underlying mechanism, TSLP expression was analyzed by immunohistochemical staining. As shown in Figure 1(c), a skin biopsy specimen from a normal control showed negative staining in the granular layers, whereas TSLP was highly expressed in the patient’s skin biopsy specimen obtained at 3 weeks of age. The staining pattern was similar to those found in atopic dermatitis and Netherton syndrome.5 These findings suggest that increased expression of epidermal TSLP in inflammatory PSS may result in a Th2 adaptive immune response to environmental allergens like atopic dermatitis and Netherton syndrome. Further studies will be necessary to assess induction and regulation of TSLP expression and its downstream molecules in inflammatory PSS. The characterization of barrier-related defects and local immune phenotype
may lead to a better understanding of human disorders associated with skin barrier defects.
ACKNOWLEDGEMENTS: This work was supported by a Grant-in-Aid for Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology of Japan; and a grant from the Ministry of Health, Labor and Welfare of Japan, Tokyo. CONFLICT OF INTEREST:
None declared.
Taizo WADA,1 Tomoko TOMA,1 Yasuhito HAMAGUCHI,2 Akihiro YACHIE1 1
Departments of Pediatrics, and 2 Dermatology, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan doi: 10.1111/1346-8138.12449
REFERENCES 1 Oji V, Eckl KM, Aufenvenne K et al. Loss of corneodesmosin leads to severe skin barrier defect, pruritus, and atopy: unraveling the peeling skin disease. Am J Hum Genet 2010; 87: 274–281. 2 De Benedetto A, Kubo A, Beck LA. Skin barrier disruption: a requirement for allergen sensitization? J Invest Dermatol 2012; 132: 949– 963. 3 Ziegler SF. Thymic stromal lymphopoietin and allergic disease. J Allergy Clin Immunol 2012; 130: 845–852. 4 Wada T, Matsuda Y, Muraoka M et al. Alu-mediated large deletion of the CDSN gene as a cause of peeling skin disease. Clin Genet 2013; (in press). 5 Soumelis V, Reche PA, Kanzler H et al. Human epithelial cells trigger dendritic cell mediated allergic inflammation by producing TSLP. Nat Immunol 2002; 3: 673–680.
Unilateral linear punctate palmar keratoderma Dear Editor, Palmoplantar keratodermas (PPKs) are a heterogeneous group of disorders characterised by thickening of the epidermis of the palms and soles. They are genetically and clinically heterogeneous. Autosomal recessive, autosomal dominant, X-linked, and acquired forms have been described. Clinically, three forms can be seen: diffuse, focal, and punctate. Punctate linear palmoplantar keratoderma is a rare entity.1–3 Unilateral disease with involvement of only one hand has not been reported yet, to our knowledge. Here we present a 15-year-old boy who was enrolled at our outpatient clinic with palmar linear hyperkeratosis. In a dermatologic examination there was a linear yellow-red palmar hyperkeratotic plaque in the hypothenar area (Fig. 1a,b). The lesion developed when the patient was 4 years old and had
grown over time. There were no similar lesions on other parts of the body. The boy’s complaint was its appearance and itchiness. There was no involvement in the right hand, or right and left soles. Complete blood count, urea, creatinine, liver function tests, stool for occult blood, sedimentation and chest x-ray were normal. There were no similar lesions among the patient’s family members. We took two biopsies for confirmation and the result was keratoderma. In the biopsy, compact orthohyperkeratosis, hypergranulosis, and slight acanthosis were seen (Fig. 1c). In the differential diagnosis linear punctate porokeratosis, arsenical keratosis, striate PPK was considered. Our case was diagnosed histopathologically. Linear punctate PPK can be seen in all races and its rates are the same in both sexes.4 Generally, it is seen between the ages of 10-30 years.1–3 PPKs can be inherited or acquired.
Correspondence: Dilek Biyik Ozkaya Adnan, Menderes Bulvarı, Vatan caddesi 34093 Bezmialem Vakıf Universitesi Tıp Fak€ ultesi Deti ve Zuhrevi Hastalıkları Istanbul,Turkey. Email: [email protected]
© 2014 Japanese Dermatological Association
449
Letters to the Editor
(a)
(c)
(b)
Figure 1. (a, b) Clinical presentation of the lesion. (c) Prominent compact orthohyperkeratosis, hypergranulosis, and slight acanthosis of the epidermis, consistent with keratoderma (H&E 9 20).
While its aetiology is unknown, external factors such as trauma may trigger the disease.1,3 Generally, the involvement is bilateral. Sharma et al. reported a case of unilateral linear PPK, which was the first such case.1 In their case, only the left sole and hand were involved. Treatment is generally symptomatic. Topical keratolytics (e.g. salicylic acid, lactic acid), steroids, tretinoin, and psoralens can be used. Effective treatment with acitretin has been reported. Our case did not want to use systemic therapy. We treated him with 0.025% topical tretinoin for 1 month; the lesion got thinner but did not completely heal. The lesion recurred after 1 month. The patient didn’t want to use topical treatments and he didn’t use them regularly, so we suggested oral retinoids to our patient. The aetiology of this disease is unknown but genetic and environmental factors may trigger the disease. It has been described in a few autosomal dominant pedigrees. Martinez et al. reported that they have identified the locus for type I punctate PPK on chromosome 15q22–q24.5 In the family members of our patient there was no known skin disease, so our patient may have a mutation. PPKs can be associated with malignancies, so PPK patients must be examined and followed up carefully. Our patient had no other disease, and none of his relatives had similar lesions. Our patient’s laboratory tests and clinical examination were normal. All patients must be followed up carefully because of the association with malignancies.1
CONFLICT OF INTEREST:
None.
Dilek BIYIK OZKAYA,1 Betul TAS,2 Ozlem SU,1 Alkım UNAL CAKITER,1 Zeynep TOSUNER,3 Cuyan DEMIRKESEN,4 Nahide ONSUN1 1
Department of Dermatovenerology, Medical Faculty, Bezmialem Vakif University, 2Department of Dermatovenerology, Bagcılar Research and Training Hospital, 3Department of Pathology, Medical Faculty, Bezmialem Vakif University, and 4Department of Pathology, Istanbul University Cerrahpasßa Medical Faculty, Istanbul, Turkey doi: 10.1111/1346-8138.12485
REFERENCES 1 Sharma S, Barman KD, Garg VK, Jain S. Unilateral linear punctate palmoplantar keratoderma. Indian J Dermatol Venereol Leprol 2012; 78: 85–8. 2 O’Toole A, O’Malley M. Unilateral keratoderma in a mother and her son. J Cutan Med Surg 1012; 16: 288–90. PubMed 3 Oztas P, Alli N, Polat M et al. Punctate palmoplantar keratoderma (Brauer-Buschke-Fischer syndrome). Am J Clin Dermatol 2007; 8: 113–6. PubMed 4 Erkek E, Erdogan S, Tuncez F et al. Type I hereditary punctate keratoderma associated with widespread lentigo simplex and successfully treated with low-dose oral acitretin. Arch Dermatol 2006; 142: 1076– 7. PubMed 5 Martinez-Mir A, Zlotogorski A, Londono D et al. Identification of a locus for type 1 punctate palmoplantar keratoderma on chromosome 15q22-q24. J Med Genet 2003; 40: 872–8.
Late-onset self-healing Langerhans cell histiocytosis in a patient with atopic dermatitis Dear Editor, Self-healing Langerhans cell histiocytosis (SHLCH) is a variant of cutaneous Langerhans cell (LC) histiocytosis initially seen at
birth or just after birth. SHLCH is characterized by eruptions of multiple papules and nodules that resolve spontaneously without involvement of other organs.1 In 1973, Hashimoto and
Correspondence: Takeshi Nakahara, M.D., Ph.D., Division of Skin Surface Sensing, Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. Email: [email protected]
450
© 2014 Japanese Dermatological Association
