Correspondence
References 1 Andres C, Puchta U, Sander CA, et al. Prevalence of Merkel cell polyomavirus DNA in cutaneous lymphomas, pseudolymphomas, and inflammatory skin diseases. Am J Dermatopathol 2010; 32: 593–598. 2 Shuda M, Arora R, Kwun HJ, et al. Human Merkel cell polyomavirus infection I. MCV T antigen expression in Merkel cell carcinoma, lymphoid tissues and lymphoid tumors. Int J Cancer 2009; 125: 1243–1249. 3 Kreuter A, Silling S, Dewan M, et al. Evaluation of four recently discovered human polyomaviruses in primary cutaneous B cell and T cell lymphoma. Arch Dermatol 2011; 147: 1449–1451. 4 Schowalter RM, Pastrana DV, Pumphrey KA, et al. Merkel cell polyomavirus and two previously unknown polyomaviruses are chronically shed from human skin. Cell Host Microbe 2010; 7: 509–515. 5 Mirvish JJ, Pomerantz RG, Falo LD Jr, et al. Role of infectious agents in cutaneous T cell lymphoma: facts and controversies. Clin Dermatol 2013; 31: 423–431.
Acne keloidalis nuchae in Latin American women
Acne keloidalis nuchae (AKN) is a chronic inflammatory disease that involves the hair follicles of the occipital base of the head and neck, leading to the formation of keloidal papules or plaques.1 It is caused by the entrapment of hair into the connective tissue, causing a foreign body reaction.2 It affects mostly those of African, Southeast Asian, and Hispanic descent. Its incidence is low, representing 0.45% of all African dermatoses. Few cases in women have been reported.2–5 The following report consists of three cases of AKN in mestizo women and one in an African-American woman. Case 1, a 42-year-old mestizo woman with curly hair, presented with a 10 9 5 cm scarring alopecic plaque studded with firm follicular papules and several hair shafts emerging from them. It was located on the nape of her neck and had evolved over six years. Her physical
(a)
examination was relevant for acanthosis nigricans on her neck and both armpits, keratosis pilaris in the cheeks and lateral arm, and ulerythema ophryogenes in her eyebrows (Fig. 1). Her abdominal circumference measured 90 cm, and body mass index (BMI) was 28.4. Laboratory tests were within normal limits, except for insulin (25 mU/ml) and homeostatic model assessment estimated insulin resistance (HOMA-IR) (5.5). Case 2 is a 20-year-old mestizo female patient with straight hair and Down syndrome. She was evaluated for an alopecic, pruritic area of 7 9 5 cm located in her occiput with follicular papules, which had been present for over six months. She had acanthosis nigricans in flexural areas, striae distensae in arms and thighs, and keratosis pilaris (Fig. 2). The abdominal circumference was 98 cm, and BMI was 31.4. Her laboratory tests revealed cholesterol and triglyceride levels slightly above the upper limit of normal, fasting glucose 89 mg/dl, insulin 20 mU/ml, and HOMA 4.3. Case 3, a 46-year-old mestizo female patient with curly hair, was evaluated for an alopecic plaque that measured approximately 4 9 3 cm with few erythematous follicular papules in the occipital region that had been present for over four years (Fig. 3a). The abdominal circumference was 90 cm and BMI 28. Laboratory tests revealed a fasting glucose of 98 mg/dl, insulin 20.6 mU/ml, and HOMA 5.5. Case 4, a 40-year-old black female patient with curly hair, had a 6-year history of a large, painful, scarring alopecic plaque located in the nape of the neck (Fig. 3b). The abdominal circumference measured 70 cm and BMI 22.3. The laboratory tests were within normal values. AKN is almost exclusively reported in males.1 This suggests a pathophysiological mechanism related to male sex hormones (i.e., increase in testosterone levels, augmented sensitivity of hair follicles to androgens, or high levels of free androgens), occupation, sports, and shaving habits, which are less commonly observed in women. There are a few reports of AKN in women that showed no relation-
(b)
Figure 1 (a) Alopecic area in the neck with follicular papules, through which emerges hair shafts and scar plates. (b) Keratotic follicular papules on erythematous areas located distally in both eyebrows ª 2015 The International Society of Dermatology
International Journal of Dermatology 2015, 54, e182–e196
e183
e184
Correspondence
(a)
(b)
Figure 2 (a) Alopecic area in the neck and pruritic follicular papules. (b) Acanthosis nigricans, striae distensae and keratotic papules in arms
(a)
(b)
Figure 3 (a) Alopecic area with few erythematous follicular papules in occipital region corresponding to case 3. (b) Scarred alopecic plaque in the neck corresponding to case 4.
ship with these predisposing factors.3–6 In the cases described, race and normal testosterone levels suggest the existence of other mechanisms, which may be causing this disorder. Three of our patients had an elevated BMI, elevated insulin levels, and HOMA index consistent with insulin resistance (HOMA ≥ 4.30). Verma and Wollina2 suggested AKN as another sign of metabolic syndrome. Most cases in our country are not related to curly hair, black race, or chronic irritation of the occipital area. Knable et al.7 studied AKN in American football players, reporting a high prevalence in players of African descent. The risk of developing type 2 diabetes, cardiovascular disease, and metabolic syndrome is very high, mainly in linemen, who tend to have a higher BMI.8 The repeated association of acanthosis nigricans found in them could generate the hypothesis that AKN not only occurs in American football players because of their race or helmet use but also because of their anthropometric characteristics. One of our cases had ulerythema ophryogenes. This condition has been previously reported in keratosis follicularis spinulosa decalvans, which had been associated with AKN.9 We were not able to find any previous reports of AKN in patients with Down syndrome.10 However, the association of Down syndrome with diabetes, International Journal of Dermatology 2015, 54, e182–e196
insulin resistance, and acanthosis nigricans may explain its appearance. Our cases suggest that AKN should be investigated as a multifactorial process, where environmental factors, metabolic, follicle occlusive dermatosis, and racial characteristics are involved. Enrique Loayza, MD Tania Cazar, MD Veronica Uraga, MD Andrea Lubkov, MD Juan Carlos Garces, MD Department of Dermatology Hospital Luis Vernaza Guayaquil Ecuador E-mail: [email protected] Conflicts of interest: None of the authors have any disclosures to declare.
References 1 Adegbidi H, Atadokpede F, do Ango-Padonou F, et al. Keloid acne of the neck: epidemiological studies ª 2015 The International Society of Dermatology
Correspondence
2
3
4 5
6
7
8
9
10
over 10 years. Int J Dermatol 2005; 44(Suppl 1): 49–50. Verma SB, Wollina U. Acne keloidalis nuchae: another cutaneous symptom of metabolic syndrome, truncal obesity, and impending/overt diabetes mellitus? Am J Clin Dermatol 2010; 11: 433–436. Ogunbiyi A, George A. Acne keloidalis in females: case report and review of literature. J Natl Med Assoc 2005; 97: 736–738. Dinehart SM, Tanner L, Mallory SB, et al. Acne keloidalis in women. Cutis 1989; 44: 250–252. Akaberi AA, Kafaie P, Noorbala MT, et al. Acne keloidalis nuchae in a Caucasian woman. J Pak Assoc Dermatol 2011; 21: 66–68. Kafaie P, Akaberi A, Hajihoseini H, et al. Acne keloidalis nuchae in a white woman. Iran J Dermatol 2009; 12: 69–70. Knable AL Jr, Hanke CW, Gonin R. Prevalence of acne keloidalis nuchae in football players. J Am Acad Dermatol 1997; 37: 570–574. Wilkerson GB, Bullard JT, Bartal DW. Identification of cardiometabolic risk among collegiate football players. J Athl Train 2010; 45: 67–74. Goh MS, Magee J, Chong AH. Keratosis follicularis spinulosa decalvans and acne keloidalis nuchae. Australas J Dermatol 2005; 46: 257–260. Fonseca CT, Amaral DM, Ribeiro MG, et al. Insulin resistance in adolescents with Down syndrome: a cross-sectional study. BMC Endocr Disord 2005; 5: 6.
Severe atopic dermatitis associated with transient hypogammaglobulinemia of infancy
Atopic dermatitis (AD) is a common chronic inflammatory skin disease, which, in infants, is usually treated with topical steroids and dietary restriction. However, these treatments are often ineffective. In infants, as the expression of maternally derived IgG decreases, serum IgG levels reduce,
(a)
resulting in physiological hypogammaglobulinemia. These levels reach a nadir at the age of 3–4 months and subsequently increase when infant IgG production commences. Transient hypogammaglobulinemia in infancy (THI) is an extension of this physiological hypogammaglobulinemia.1 We report two cases of severe AD associated with THI, both of which improved after the serum IgG levels increased. Case 1 was a 6-month-old boy that developed erythema on both cheeks at two months of age (Fig. 1a), which subsequently spread throughout the body. He was treated with herbal medicine only at another clinic; however, the erythema progressed to scaling and eczema. Therefore, the boy was admitted to our hospital and was treated with topical and intravenous corticosteroids. Case 2 was a 4-month-old girl that developed erythroderma with severe eczema on the face and body and was treated with emollient only for two months at a pediatric clinic. However, the eczema progressed, and she developed phlegmon in the middle finger of the left hand. Thus, she was referred to our hospital, where she was administered topical corticosteroids, intravenous antibiotics, and corticosteroids. As IgE antibodies specific to certain food allergens were elevated in both patients (Table 1), the implicated foods were subsequently avoided. Both patients were diagnosed with AD according to the Japanese guidelines for the management of AD.2 Both patients shared common clinical features, had a similar clinical course, and hypogammaglobulinemia but had no family history of allergy (Table 1). Their eruptions resolved during hospitalization but relapsed after discharge, despite the application of topical corticosteroids. At 10 months of age, their erythema and eczema began to resolve as their serum IgG levels increased. At 16 months of age, the eczema had completely resolved
(b)
Figure 1 Clinical features of the patient (case 1). (a) Clinical features at the age of 6 months. Erythema with scaling and eczema was noted. (b) Clinical features at the age of 16 months. No eczema or erythema was noted ª 2015 The International Society of Dermatology
International Journal of Dermatology 2015, 54, e182–e196
e185
References 1 Andres C, Puchta U, Sander CA, et al. Prevalence of Merkel cell polyomavirus DNA in cutaneous lymphomas, pseudolymphomas, and inflammatory skin diseases. Am J Dermatopathol 2010; 32: 593–598. 2 Shuda M, Arora R, Kwun HJ, et al. Human Merkel cell polyomavirus infection I. MCV T antigen expression in Merkel cell carcinoma, lymphoid tissues and lymphoid tumors. Int J Cancer 2009; 125: 1243–1249. 3 Kreuter A, Silling S, Dewan M, et al. Evaluation of four recently discovered human polyomaviruses in primary cutaneous B cell and T cell lymphoma. Arch Dermatol 2011; 147: 1449–1451. 4 Schowalter RM, Pastrana DV, Pumphrey KA, et al. Merkel cell polyomavirus and two previously unknown polyomaviruses are chronically shed from human skin. Cell Host Microbe 2010; 7: 509–515. 5 Mirvish JJ, Pomerantz RG, Falo LD Jr, et al. Role of infectious agents in cutaneous T cell lymphoma: facts and controversies. Clin Dermatol 2013; 31: 423–431.
Acne keloidalis nuchae in Latin American women
Acne keloidalis nuchae (AKN) is a chronic inflammatory disease that involves the hair follicles of the occipital base of the head and neck, leading to the formation of keloidal papules or plaques.1 It is caused by the entrapment of hair into the connective tissue, causing a foreign body reaction.2 It affects mostly those of African, Southeast Asian, and Hispanic descent. Its incidence is low, representing 0.45% of all African dermatoses. Few cases in women have been reported.2–5 The following report consists of three cases of AKN in mestizo women and one in an African-American woman. Case 1, a 42-year-old mestizo woman with curly hair, presented with a 10 9 5 cm scarring alopecic plaque studded with firm follicular papules and several hair shafts emerging from them. It was located on the nape of her neck and had evolved over six years. Her physical
(a)
examination was relevant for acanthosis nigricans on her neck and both armpits, keratosis pilaris in the cheeks and lateral arm, and ulerythema ophryogenes in her eyebrows (Fig. 1). Her abdominal circumference measured 90 cm, and body mass index (BMI) was 28.4. Laboratory tests were within normal limits, except for insulin (25 mU/ml) and homeostatic model assessment estimated insulin resistance (HOMA-IR) (5.5). Case 2 is a 20-year-old mestizo female patient with straight hair and Down syndrome. She was evaluated for an alopecic, pruritic area of 7 9 5 cm located in her occiput with follicular papules, which had been present for over six months. She had acanthosis nigricans in flexural areas, striae distensae in arms and thighs, and keratosis pilaris (Fig. 2). The abdominal circumference was 98 cm, and BMI was 31.4. Her laboratory tests revealed cholesterol and triglyceride levels slightly above the upper limit of normal, fasting glucose 89 mg/dl, insulin 20 mU/ml, and HOMA 4.3. Case 3, a 46-year-old mestizo female patient with curly hair, was evaluated for an alopecic plaque that measured approximately 4 9 3 cm with few erythematous follicular papules in the occipital region that had been present for over four years (Fig. 3a). The abdominal circumference was 90 cm and BMI 28. Laboratory tests revealed a fasting glucose of 98 mg/dl, insulin 20.6 mU/ml, and HOMA 5.5. Case 4, a 40-year-old black female patient with curly hair, had a 6-year history of a large, painful, scarring alopecic plaque located in the nape of the neck (Fig. 3b). The abdominal circumference measured 70 cm and BMI 22.3. The laboratory tests were within normal values. AKN is almost exclusively reported in males.1 This suggests a pathophysiological mechanism related to male sex hormones (i.e., increase in testosterone levels, augmented sensitivity of hair follicles to androgens, or high levels of free androgens), occupation, sports, and shaving habits, which are less commonly observed in women. There are a few reports of AKN in women that showed no relation-
(b)
Figure 1 (a) Alopecic area in the neck with follicular papules, through which emerges hair shafts and scar plates. (b) Keratotic follicular papules on erythematous areas located distally in both eyebrows ª 2015 The International Society of Dermatology
International Journal of Dermatology 2015, 54, e182–e196
e183
e184
Correspondence
(a)
(b)
Figure 2 (a) Alopecic area in the neck and pruritic follicular papules. (b) Acanthosis nigricans, striae distensae and keratotic papules in arms
(a)
(b)
Figure 3 (a) Alopecic area with few erythematous follicular papules in occipital region corresponding to case 3. (b) Scarred alopecic plaque in the neck corresponding to case 4.
ship with these predisposing factors.3–6 In the cases described, race and normal testosterone levels suggest the existence of other mechanisms, which may be causing this disorder. Three of our patients had an elevated BMI, elevated insulin levels, and HOMA index consistent with insulin resistance (HOMA ≥ 4.30). Verma and Wollina2 suggested AKN as another sign of metabolic syndrome. Most cases in our country are not related to curly hair, black race, or chronic irritation of the occipital area. Knable et al.7 studied AKN in American football players, reporting a high prevalence in players of African descent. The risk of developing type 2 diabetes, cardiovascular disease, and metabolic syndrome is very high, mainly in linemen, who tend to have a higher BMI.8 The repeated association of acanthosis nigricans found in them could generate the hypothesis that AKN not only occurs in American football players because of their race or helmet use but also because of their anthropometric characteristics. One of our cases had ulerythema ophryogenes. This condition has been previously reported in keratosis follicularis spinulosa decalvans, which had been associated with AKN.9 We were not able to find any previous reports of AKN in patients with Down syndrome.10 However, the association of Down syndrome with diabetes, International Journal of Dermatology 2015, 54, e182–e196
insulin resistance, and acanthosis nigricans may explain its appearance. Our cases suggest that AKN should be investigated as a multifactorial process, where environmental factors, metabolic, follicle occlusive dermatosis, and racial characteristics are involved. Enrique Loayza, MD Tania Cazar, MD Veronica Uraga, MD Andrea Lubkov, MD Juan Carlos Garces, MD Department of Dermatology Hospital Luis Vernaza Guayaquil Ecuador E-mail: [email protected] Conflicts of interest: None of the authors have any disclosures to declare.
References 1 Adegbidi H, Atadokpede F, do Ango-Padonou F, et al. Keloid acne of the neck: epidemiological studies ª 2015 The International Society of Dermatology
Correspondence
2
3
4 5
6
7
8
9
10
over 10 years. Int J Dermatol 2005; 44(Suppl 1): 49–50. Verma SB, Wollina U. Acne keloidalis nuchae: another cutaneous symptom of metabolic syndrome, truncal obesity, and impending/overt diabetes mellitus? Am J Clin Dermatol 2010; 11: 433–436. Ogunbiyi A, George A. Acne keloidalis in females: case report and review of literature. J Natl Med Assoc 2005; 97: 736–738. Dinehart SM, Tanner L, Mallory SB, et al. Acne keloidalis in women. Cutis 1989; 44: 250–252. Akaberi AA, Kafaie P, Noorbala MT, et al. Acne keloidalis nuchae in a Caucasian woman. J Pak Assoc Dermatol 2011; 21: 66–68. Kafaie P, Akaberi A, Hajihoseini H, et al. Acne keloidalis nuchae in a white woman. Iran J Dermatol 2009; 12: 69–70. Knable AL Jr, Hanke CW, Gonin R. Prevalence of acne keloidalis nuchae in football players. J Am Acad Dermatol 1997; 37: 570–574. Wilkerson GB, Bullard JT, Bartal DW. Identification of cardiometabolic risk among collegiate football players. J Athl Train 2010; 45: 67–74. Goh MS, Magee J, Chong AH. Keratosis follicularis spinulosa decalvans and acne keloidalis nuchae. Australas J Dermatol 2005; 46: 257–260. Fonseca CT, Amaral DM, Ribeiro MG, et al. Insulin resistance in adolescents with Down syndrome: a cross-sectional study. BMC Endocr Disord 2005; 5: 6.
Severe atopic dermatitis associated with transient hypogammaglobulinemia of infancy
Atopic dermatitis (AD) is a common chronic inflammatory skin disease, which, in infants, is usually treated with topical steroids and dietary restriction. However, these treatments are often ineffective. In infants, as the expression of maternally derived IgG decreases, serum IgG levels reduce,
(a)
resulting in physiological hypogammaglobulinemia. These levels reach a nadir at the age of 3–4 months and subsequently increase when infant IgG production commences. Transient hypogammaglobulinemia in infancy (THI) is an extension of this physiological hypogammaglobulinemia.1 We report two cases of severe AD associated with THI, both of which improved after the serum IgG levels increased. Case 1 was a 6-month-old boy that developed erythema on both cheeks at two months of age (Fig. 1a), which subsequently spread throughout the body. He was treated with herbal medicine only at another clinic; however, the erythema progressed to scaling and eczema. Therefore, the boy was admitted to our hospital and was treated with topical and intravenous corticosteroids. Case 2 was a 4-month-old girl that developed erythroderma with severe eczema on the face and body and was treated with emollient only for two months at a pediatric clinic. However, the eczema progressed, and she developed phlegmon in the middle finger of the left hand. Thus, she was referred to our hospital, where she was administered topical corticosteroids, intravenous antibiotics, and corticosteroids. As IgE antibodies specific to certain food allergens were elevated in both patients (Table 1), the implicated foods were subsequently avoided. Both patients were diagnosed with AD according to the Japanese guidelines for the management of AD.2 Both patients shared common clinical features, had a similar clinical course, and hypogammaglobulinemia but had no family history of allergy (Table 1). Their eruptions resolved during hospitalization but relapsed after discharge, despite the application of topical corticosteroids. At 10 months of age, their erythema and eczema began to resolve as their serum IgG levels increased. At 16 months of age, the eczema had completely resolved
(b)
Figure 1 Clinical features of the patient (case 1). (a) Clinical features at the age of 6 months. Erythema with scaling and eczema was noted. (b) Clinical features at the age of 16 months. No eczema or erythema was noted ª 2015 The International Society of Dermatology
International Journal of Dermatology 2015, 54, e182–e196
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