Pediatric Dermatology Vol. 31 No. 6 e160–e163, 2014
Nodular Lichen Myxedematosus During Childhood: A Case Report Rong Zeng, M.D., Min Li, M.D., Yiqun Jiang, M.D., and Weida Liu, M.D. Institute of Dermatology, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China
Abstract: Nodular lichen myxedematosus (LM) is a rare disease and is one of the five subtypes of localized LM. It is distinctly characterized by multiple nodules on the trunk and limbs with or without papular lesions. A healthy 6-year-old boy presented with complaints of “knots” on his right chest, upper limb, and thigh for 4 months. To our knowledge this is the first case of nodular LM reported in a child.
A healthy 6-year-old boy presented with multiple nodules that appeared progressively on the right chest, upper limb, and thigh. The lesions had appeared 4 months before the hospital visit. He had been treated with mometasone furoate cream once a day for 2 weeks with no improvement. He was otherwise healthy and his past medical history was unremarkable. No family history of related illnesses or infections and no remarkable travel history were found. Upon physical examination, more than 10 cutaneous nodules were observed. Two large, solid, erythematous nodules (approximately 1–2 cm) were noted on the right chest and some smaller ones were present around the larger nodules (Fig. 1A). One nodule approximately 1 cm in diameter was observed on the upper right limb (Fig. 1A) and another approximately 2 cm in diameter was seen on the right thigh (Fig. 1B). All of the lesions were asymptomatic. Routine blood cell count, erythrocyte sedimentation rate, antistreptolysin O titer, C-reactive protein,
hepatic and thyroid function tests, rheumatoid factor, antinuclear antibodies, and serum protein electrophoresis were all within the normal range. A chest radiograph was also normal. A skin biopsy was obtained from the nodule on the chest (Figs. 2A, B). Histopathologically, slight lymphocytic infiltration was observed around the vessels and adnexa, with a normal epidermis. Large quantities of mucin deposits were observed between collagen fibers, but fibroblasts were not increased in number. Alcian blue (pH 2.5) staining was positive in the above section, confirming the mucin deposition (Fig. 2C). DISCUSSION Nodular lichen myxedematosus (LM) is a rare disease first reported in 1980 by Suter et al (1). It is a subtype of localized LM with papular or nodular and plaque eruptions, without monoclonal gammopathy and thyroid disease. The lesion reveals mucin deposition with variable fibroblast proliferation (2). Table 1 shows the main clinical and histologic features of
Address correspondence to Weida Liu, M.D., 12 Jiangwangmiao Street, Nanjing 210042, China, or e-mail: Liumyco@hotmail. com. DOI: 10.1111/pde.12376
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© 2014 Wiley Periodicals, Inc.
Zeng et al: Nodular Lichen Myxedematosus During Childhood
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Figure 1. (A) Two large (1–2 cm), firm, erythematous nodules were noted on the right chest and smaller ones were present around the larger nodule. A 1-cm nodule was observed on the right upper limb. (B) A 1.5-cm nodule was found on the right thigh.
Figure 2. A skin biopsy was obtained from a nodule on the chest. (A) A slight lymphocytic infiltration was observed around vessels and adnexa, with a normal epidermis (hematoxylin and eosin, 1009). (B) Large quantities of mucin deposits were observed between collagen fibers (hematoxylin and eosin, 1009). (C) Mucinous deposition is confirmed by positive staining with Alcian blue (1009).
TABLE 1. Clinical Differential Diagnosis of the Different Types of Lichen Myxedematosus (LM) Disease
Main clinical features
Main histologic features
Discrete papular LM
Symmetrically distributed papules on trunk and limbs, not selfhealing, without systemic involvement Symmetrical papules on the extensor surface of the forearms, not self-healing, without systemic involvement
Mucinous and splay collagen bundles in the dermis Upper reticular dermis focally mucin, sparing a subepidermal zone, no increase in the number of fibroblasts Papillary dermal mucin, without overt fibroblast proliferation Reticular dermal mucin
Acral persistent papular mucinosis Papular mucinosis of infancy Nodular form Self-healing cutaneous mucinosis Generalized form Scleromyxedema without monoclonal gammopathy Localized LM with monoclonal gammopathy Localized LM with mixed features of different subtypes
Congenital or perinatal, firm papules, not self-healing, without systemic involvement Various nodules with or without a papular component, not self-healing, without systemic involvement Papules, nodules, or plaques with a predilection for the head and trunk, self-healing, without systemic involvement Generalized papular and sclerodermoid eruption, monoclonal gammopathy, usually with various of internal symptoms, absence of thyroid disorder Generalized papular and sclerodermoid eruption, no monoclonal gammopathy
Dermal mucinosis and a mild increase in fibroblasts and mast cells Microscopic triad (mucin deposition, fibroblast proliferation, fibrosis) Microscopic triad (mucin deposition, fibroblast proliferation, fibrosis)
Features of localized LM, monoclonal gammopathy
Mucin deposition with variable fibroblast proliferation
Mixed features of two or more kinds of localized LM
Mucin deposition with variable fibroblast proliferation
Data from references (2,3,6).
e162 Pediatric Dermatology Vol. 31 No. 6 November/December 2014
TABLE 2. Clinical Features of Nodular Lichen Myxedematosus Reference
Year
Age (years)
Sex
Location of nodules
Size (mm)
Itching
Treatment (outcome)
1
1980
48
Male
Upper legs
3–15
No
3
1991
32
Male
5–20
No
4
2010
18
Female
Right part of trunk, right-side proximal extremities Forearms, left cubital fossa, left thigh
Hyaluronidase injections (partial regression) None
6–10
No
Our patient
2012
6
5–20
No
Male
Right side of trunk, right-side proximal extremities
different types of LM. Localized LM is classified into five types clinically. Discrete LM is characterized by multiple symmetrically distributed 2- to 5-mm papules on the trunk and limbs. The eruptions progress slowly and rarely resolve spontaneously. Acral persistent papular mucinosis is characterized by variable numbers of 2- to 5-mm symmetrical ivory to skin-colored papules on the extensor surface of the distal forearms and dorsal aspect of the hands. The lesions are not associated with systemic manifestations and persist without spontaneous resolution. Cutaneous mucinosis of infancy is characterized by a number of firm opalescent papules on the neck, upper arms, and trunk. Neither systemic manifestations nor spontaneous resolution occur. Self-healing juvenile cutaneous mucinosis is characterized by the acute onset of deep nodules followed by spontaneous resolution in a few weeks. Nodular LM is characterized by the occurrence of various nodules, usually confined to the trunk or limbs with or without a papular component. Based on clinical and histologic features, the lesions on this boy were diagnosed as nodular LM. Clinically, nodular LM in childhood should be distinguished from other cutaneous mucinosis in young persons. Unlike cutaneous mucinosis of infancy, the age of onset of nodular LM is older and its lesions are somewhat more likely to be multiple nodules than firm opalescent papules. Mucin deposition of the former is much closer to the epidermis than the latter (3–5). For differential diagnosis, another possibility is self-healing juvenile cutaneous mucinosis (SHJCM), which is characterized by an acute eruption of multiple papules and nodules on the face, neck, scalp, abdomen, thighs, and periar-
Triamcinolone acetonide injections (regression) 0.03% tacrolimus ointment (not effective)
ticular areas. In addition, systemic symptoms (e.g., fever, muscle tenderness, arthralgias) and spontaneous resolution of lesions after a few months make it easy to distinguish from nodular LM (6–8). Our patient had no systemic symptoms and the lesions had not resolved spontaneously after 15 months. One of main histologic features of SHJCM is that mucin deposits deeply in the nodular lesions with bands of fibrosis and remarkable increases in the number of fibroblasts (8). These histopathologic changes are not found in nodular LM. Only three previous cases of nodular LM have been reported (1,9,10). These cases are summarized in Table 2. The mean age of onset of the previous three cases was 32.6 years (range 18–48 years). The current case is the only one occurring during childhood, which suggests that nodular LM may occur at a younger age than previously thought. Schneider et al (9) reported a case wherein the nodules were distributed on the right trunk and limbs, similar to the distribution in our patient. Whether this pattern is coincidental or indicates a potential mechanism of nodular LM is inconclusive. After 9 months of treatment with 0.03% tacrolimus ointment, the lesions did not show any improvement. No standard treatment for localized LM is available, although Azusa et al (10) reported regression after triamcinolone acetonide injections. More case evaluations are needed for better management of nodular LM. Our findings should prompt dermatologists to perform histologic examinations of suspect nodules in children. REFERENCES 1. Suter L, Vakilzadeh F, Macher E. Atypical tuberous myxedema Jadassohn-Dossekker. Report of a case. Dermatologica 1980;16:265–269. 2. Rongioletti F. Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg 2006;25:100–104.
Zeng et al: Nodular Lichen Myxedematosus During Childhood
3. Lum D. Cutaneous mucinosis of infancy. Arch Dermatol 1980;116:198–200. 4. Stokes KS, Rabinowitz LG, Segura AD et al. Cutaneous mucinosis of infancy. Pediatr Dermatol 1994;11: 246–251. 5. Calza AM, Masouye I, Saurat JH. An unusual case of infantile dermal mucinosis. Pediatr Dermatol 1994;11:252–255. 6. Carder KR, Fitzpatrick JE, Weston WL et al. Selfhealing juvenile cutaneous mucinosis. Pediatr Dermatol 2003;20:35–39.
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7. Aydingoz IE, Candan I, Dervent B. Self-healing juvenile cutaneous mucinosis. Dermatology 1999;199:57–59. 8. Barreau M, Dompmartin-Blanchere A, Jamous R et al. Nodular lesions of self-healing juvenile cutaneous mucinosis: a pitfall. Am J Dermatopathol 2012;34:699–705. 9. Schneider BV, Hohl D, Schnyder UW. Nodular cutaneous mucinosis—an unusual multiple type of euthyreotic focal mucinosis. Dermatologica 1991;183:73–76. 10. Ogita A, Higashi N, Hosone M et al. Nodular-type lichen myxedematosus: a case report. Case Rep Dermatol 2010;2:195–200.
Nodular Lichen Myxedematosus During Childhood: A Case Report Rong Zeng, M.D., Min Li, M.D., Yiqun Jiang, M.D., and Weida Liu, M.D. Institute of Dermatology, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China
Abstract: Nodular lichen myxedematosus (LM) is a rare disease and is one of the five subtypes of localized LM. It is distinctly characterized by multiple nodules on the trunk and limbs with or without papular lesions. A healthy 6-year-old boy presented with complaints of “knots” on his right chest, upper limb, and thigh for 4 months. To our knowledge this is the first case of nodular LM reported in a child.
A healthy 6-year-old boy presented with multiple nodules that appeared progressively on the right chest, upper limb, and thigh. The lesions had appeared 4 months before the hospital visit. He had been treated with mometasone furoate cream once a day for 2 weeks with no improvement. He was otherwise healthy and his past medical history was unremarkable. No family history of related illnesses or infections and no remarkable travel history were found. Upon physical examination, more than 10 cutaneous nodules were observed. Two large, solid, erythematous nodules (approximately 1–2 cm) were noted on the right chest and some smaller ones were present around the larger nodules (Fig. 1A). One nodule approximately 1 cm in diameter was observed on the upper right limb (Fig. 1A) and another approximately 2 cm in diameter was seen on the right thigh (Fig. 1B). All of the lesions were asymptomatic. Routine blood cell count, erythrocyte sedimentation rate, antistreptolysin O titer, C-reactive protein,
hepatic and thyroid function tests, rheumatoid factor, antinuclear antibodies, and serum protein electrophoresis were all within the normal range. A chest radiograph was also normal. A skin biopsy was obtained from the nodule on the chest (Figs. 2A, B). Histopathologically, slight lymphocytic infiltration was observed around the vessels and adnexa, with a normal epidermis. Large quantities of mucin deposits were observed between collagen fibers, but fibroblasts were not increased in number. Alcian blue (pH 2.5) staining was positive in the above section, confirming the mucin deposition (Fig. 2C). DISCUSSION Nodular lichen myxedematosus (LM) is a rare disease first reported in 1980 by Suter et al (1). It is a subtype of localized LM with papular or nodular and plaque eruptions, without monoclonal gammopathy and thyroid disease. The lesion reveals mucin deposition with variable fibroblast proliferation (2). Table 1 shows the main clinical and histologic features of
Address correspondence to Weida Liu, M.D., 12 Jiangwangmiao Street, Nanjing 210042, China, or e-mail: Liumyco@hotmail. com. DOI: 10.1111/pde.12376
e160
© 2014 Wiley Periodicals, Inc.
Zeng et al: Nodular Lichen Myxedematosus During Childhood
e161
Figure 1. (A) Two large (1–2 cm), firm, erythematous nodules were noted on the right chest and smaller ones were present around the larger nodule. A 1-cm nodule was observed on the right upper limb. (B) A 1.5-cm nodule was found on the right thigh.
Figure 2. A skin biopsy was obtained from a nodule on the chest. (A) A slight lymphocytic infiltration was observed around vessels and adnexa, with a normal epidermis (hematoxylin and eosin, 1009). (B) Large quantities of mucin deposits were observed between collagen fibers (hematoxylin and eosin, 1009). (C) Mucinous deposition is confirmed by positive staining with Alcian blue (1009).
TABLE 1. Clinical Differential Diagnosis of the Different Types of Lichen Myxedematosus (LM) Disease
Main clinical features
Main histologic features
Discrete papular LM
Symmetrically distributed papules on trunk and limbs, not selfhealing, without systemic involvement Symmetrical papules on the extensor surface of the forearms, not self-healing, without systemic involvement
Mucinous and splay collagen bundles in the dermis Upper reticular dermis focally mucin, sparing a subepidermal zone, no increase in the number of fibroblasts Papillary dermal mucin, without overt fibroblast proliferation Reticular dermal mucin
Acral persistent papular mucinosis Papular mucinosis of infancy Nodular form Self-healing cutaneous mucinosis Generalized form Scleromyxedema without monoclonal gammopathy Localized LM with monoclonal gammopathy Localized LM with mixed features of different subtypes
Congenital or perinatal, firm papules, not self-healing, without systemic involvement Various nodules with or without a papular component, not self-healing, without systemic involvement Papules, nodules, or plaques with a predilection for the head and trunk, self-healing, without systemic involvement Generalized papular and sclerodermoid eruption, monoclonal gammopathy, usually with various of internal symptoms, absence of thyroid disorder Generalized papular and sclerodermoid eruption, no monoclonal gammopathy
Dermal mucinosis and a mild increase in fibroblasts and mast cells Microscopic triad (mucin deposition, fibroblast proliferation, fibrosis) Microscopic triad (mucin deposition, fibroblast proliferation, fibrosis)
Features of localized LM, monoclonal gammopathy
Mucin deposition with variable fibroblast proliferation
Mixed features of two or more kinds of localized LM
Mucin deposition with variable fibroblast proliferation
Data from references (2,3,6).
e162 Pediatric Dermatology Vol. 31 No. 6 November/December 2014
TABLE 2. Clinical Features of Nodular Lichen Myxedematosus Reference
Year
Age (years)
Sex
Location of nodules
Size (mm)
Itching
Treatment (outcome)
1
1980
48
Male
Upper legs
3–15
No
3
1991
32
Male
5–20
No
4
2010
18
Female
Right part of trunk, right-side proximal extremities Forearms, left cubital fossa, left thigh
Hyaluronidase injections (partial regression) None
6–10
No
Our patient
2012
6
5–20
No
Male
Right side of trunk, right-side proximal extremities
different types of LM. Localized LM is classified into five types clinically. Discrete LM is characterized by multiple symmetrically distributed 2- to 5-mm papules on the trunk and limbs. The eruptions progress slowly and rarely resolve spontaneously. Acral persistent papular mucinosis is characterized by variable numbers of 2- to 5-mm symmetrical ivory to skin-colored papules on the extensor surface of the distal forearms and dorsal aspect of the hands. The lesions are not associated with systemic manifestations and persist without spontaneous resolution. Cutaneous mucinosis of infancy is characterized by a number of firm opalescent papules on the neck, upper arms, and trunk. Neither systemic manifestations nor spontaneous resolution occur. Self-healing juvenile cutaneous mucinosis is characterized by the acute onset of deep nodules followed by spontaneous resolution in a few weeks. Nodular LM is characterized by the occurrence of various nodules, usually confined to the trunk or limbs with or without a papular component. Based on clinical and histologic features, the lesions on this boy were diagnosed as nodular LM. Clinically, nodular LM in childhood should be distinguished from other cutaneous mucinosis in young persons. Unlike cutaneous mucinosis of infancy, the age of onset of nodular LM is older and its lesions are somewhat more likely to be multiple nodules than firm opalescent papules. Mucin deposition of the former is much closer to the epidermis than the latter (3–5). For differential diagnosis, another possibility is self-healing juvenile cutaneous mucinosis (SHJCM), which is characterized by an acute eruption of multiple papules and nodules on the face, neck, scalp, abdomen, thighs, and periar-
Triamcinolone acetonide injections (regression) 0.03% tacrolimus ointment (not effective)
ticular areas. In addition, systemic symptoms (e.g., fever, muscle tenderness, arthralgias) and spontaneous resolution of lesions after a few months make it easy to distinguish from nodular LM (6–8). Our patient had no systemic symptoms and the lesions had not resolved spontaneously after 15 months. One of main histologic features of SHJCM is that mucin deposits deeply in the nodular lesions with bands of fibrosis and remarkable increases in the number of fibroblasts (8). These histopathologic changes are not found in nodular LM. Only three previous cases of nodular LM have been reported (1,9,10). These cases are summarized in Table 2. The mean age of onset of the previous three cases was 32.6 years (range 18–48 years). The current case is the only one occurring during childhood, which suggests that nodular LM may occur at a younger age than previously thought. Schneider et al (9) reported a case wherein the nodules were distributed on the right trunk and limbs, similar to the distribution in our patient. Whether this pattern is coincidental or indicates a potential mechanism of nodular LM is inconclusive. After 9 months of treatment with 0.03% tacrolimus ointment, the lesions did not show any improvement. No standard treatment for localized LM is available, although Azusa et al (10) reported regression after triamcinolone acetonide injections. More case evaluations are needed for better management of nodular LM. Our findings should prompt dermatologists to perform histologic examinations of suspect nodules in children. REFERENCES 1. Suter L, Vakilzadeh F, Macher E. Atypical tuberous myxedema Jadassohn-Dossekker. Report of a case. Dermatologica 1980;16:265–269. 2. Rongioletti F. Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg 2006;25:100–104.
Zeng et al: Nodular Lichen Myxedematosus During Childhood
3. Lum D. Cutaneous mucinosis of infancy. Arch Dermatol 1980;116:198–200. 4. Stokes KS, Rabinowitz LG, Segura AD et al. Cutaneous mucinosis of infancy. Pediatr Dermatol 1994;11: 246–251. 5. Calza AM, Masouye I, Saurat JH. An unusual case of infantile dermal mucinosis. Pediatr Dermatol 1994;11:252–255. 6. Carder KR, Fitzpatrick JE, Weston WL et al. Selfhealing juvenile cutaneous mucinosis. Pediatr Dermatol 2003;20:35–39.
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7. Aydingoz IE, Candan I, Dervent B. Self-healing juvenile cutaneous mucinosis. Dermatology 1999;199:57–59. 8. Barreau M, Dompmartin-Blanchere A, Jamous R et al. Nodular lesions of self-healing juvenile cutaneous mucinosis: a pitfall. Am J Dermatopathol 2012;34:699–705. 9. Schneider BV, Hohl D, Schnyder UW. Nodular cutaneous mucinosis—an unusual multiple type of euthyreotic focal mucinosis. Dermatologica 1991;183:73–76. 10. Ogita A, Higashi N, Hosone M et al. Nodular-type lichen myxedematosus: a case report. Case Rep Dermatol 2010;2:195–200.
