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4. Haycox CL, Kim S, Fleckman P, et al. Trichodysplasia spinulosa: a newly described folliculocentric viral infection in an immunocompromised host. J Investig Dermatol Symp Proc. 1999;4(3):268-271. 5. Matthews MR, Wang RC, Reddick RL, Saldivar VA, Browning JC. Viral-associated trichodysplasia spinulosa: a case with electron microscopic and molecular detection of the trichodysplasia spinulosa-associated human polyomavirus. J Cutan Pathol. 2011;38(5):420-431.

cells.5 Historically, there has been debate over the classification of mucinous nevi, with some researchers classifying it as a form of primary cutaneous mucinosis, while others classify these cases as connective tissue nevi.3 Table. Clinical Findings From 22 Reported Cases of Mucinous Nevus, Including the Present Case

Mucinous Nevus: Report of a Case and Review of the Literature

Characteristic Sex

Mucinous nevus is a rare entity characterized by mucin deposition in the papillary dermis. Herein we present a case and review the available literature.

Male Female

16 6

Nevi location Back

Report of a Case | A man in his 50s presented to clinic for a second opinion regarding asymptomatic, grouped, skin-colored papules on his left chest present since his earliest memory (Figure, A). He denied a family history of similar lesions. A biopsy had previously been performed, and the findings were initially interpreted as indicating focal cutaneous mucinosis. Review of the biopsy specimens revealed loosely separated collagen fibers in the papillary dermis with increased mucinous ground substance (Figure, B). In addition, occasional stellate fibroblasts were noted. Considering the clinical and pathologic findings together, a diagnosis of mucinous nevi was rendered.

Buttock

13 2

Chest

2

Abdomen

1

Trunk

1

Back and abdomen

1

Trunk and thigh

1

Hand

1

Age of onseta Congenital (from birth through age 1 year) Adolescence (age 7-22 years) Adult (age 48 years)

Discussion | Mucinous nevi typically present as brownish papules and plaques arranged in a unilateral, linear, grouped, or dermatomal distribution. The Table summarizes the clinical findings from 22 reported cases in the literature, including the present case. Seventy-one percent of cases were reported in men, and the most common location was the back.1-4 Of note, 45% of cases were reported in Asian countries. Half of all cases were present at birth, while the remainder developed in childhood, adolescence, or early adulthood.5 The primary histopathologic feature of mucinous nevi is a diffuse, bandlike deposition of mucin in the superficial dermis with a scarce cellular component composed of spindle

Cases of Mucinous Nevus, No.1-4 (N = 22)

8 10 1

Country of patient birth Korea

a

6

Spain

5

Taiwan

3

United States

3

Italy

1

The Netherlands

1

Japan

1

Serbia

1

Portugal

1

The age of onset was unknown for 3 patients.

Figure. Clinical and Pathologic Images From a Case of Mucinous Nevus A

B

A, In the clinical presentation, grouped skin-colored papules are evident on the left chest. B, The biopsy specimen shows increased mucinous ground substance in the papillary dermis and mild rete ridge elongation (hematoxylin-eosin, original magnification ×200).

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Those who support the connective tissue origin will further divide mucinous nevi into 2 histopathologic types: a connective tissue nevus of the proteoglycan (CTNP) type and combined epidermal-CTNP type.1 In CTNP, the epidermis is normal, while in epidermal-CTNP, the epidermis shows hyperkeratosis and acanthosis with elongation of the rete ridges consistent with an epidermal nevus.1 Regardless of classification, the origin of increased mucin is unclear. Although mucin is synthesized by fibroblasts, previous studies have shown only a slight increase in activated fibroblasts. Thus, some have postulated that the upregulation of fibroblasts is responsible for the increased mucin.3 Clinically, these lesions can be hard to distinguish from connective tissue nevi such as collagenomas or elastomas. Histologic examination is essential to exclude these entities, which can be associated with congenital abnormalities. The microscopic differential diagnosis is narrow and includes focal cutaneous mucinosis and lichen myxedematosus, both of which have distinct clinical presentations. Lesions of focal cutaneous mucinosis are usually solitary papules and can be found anywhere on the body. Lichen myxedematosus presents with a slow onset of asymptomatic or slightly pruritic papules, which can be generalized or localized. Generalized subtypes of lichen myxedematosus have an associated monoclonal gammopathy, while discrete papular lichen myxedematosus, a variant of the localized subtype, has been associated with human immunodeficiency virus disease.6 Other histologic mimics, like self-healing papular mucinosis and acral persistent papular mucinosis, are easily excluded with clinical information. In our case, clinical correlation confirmed the rare diagnosis of mucinous nevus. Treatment for a mucinous nevus is not required owing to its benign nature. Surgical intervention may remove the lesion, but scarring will result.

5. Tardío JC, Granados R. The cellular component of the mucinous nevus consists of CD34-positive fibroblasts. J Cutan Pathol. 2010;37(9):1019-1020. 6. Concheiro J, Pérez-Pérez L, Peteiro C, Labandeira J, Toribio J. Discrete papular lichen myxoedematosus: a rare subtype of cutaneous mucinosis. Clin Exp Dermatol. 2009;34(8):e608-e610.

Poland Syndrome Coexisting With Blaschkolinear Congenital Melanocytic Nevi Poland syndrome is an uncommon congenital anomaly characterized by unilateral chest wall and upper extremity hypoplasia.1 It is theorized to be due to subclavian artery hypoplasia during embryogenesis.1 There have been only a few isolated reports of Poland syndrome with congenital dermatoses.2-4 Adding to this association list, we report a case of Poland syndrome with multiple congenital melanocytic nevi (CMN). Additionally, the nevi are in a Blaschkolinear arrangement, which is also rare; we know of only 2 such cases reported previously.5,6 Report of a Case | An otherwise healthy boy in his teens presented for asymptomatic hyperpigmented birthmarks on his left leg that had grown proportionately to his leg’s growth. His medical history was significant for Poland syndrome, for which he had undergone multiple left-hand reconstruction surgeries. His family history was noncontributory. Physical examination revealed oligodactyly of the left hand, underdeveloped left chest wall (Figure 1A), and multiple, evenly pigmented, bluish-black thin papules and plaques on the left posterior thigh, calf, and ankle in a Blaschkolinear distribution (Figure 1B). A

Figure 1. Clinical Images of Patient With Poland Syndrome and Blaschkolinear Congenital Melanocytic Nevi A

Gabriela Cobos, BS Inbal Braunstein, MD Katrina Abuabara, MD Emily Y. Chu, MD, PhD William James, MD

B

Author Affiliations: Perelman School of Medicine, University of Pennsylvania, Philadelphia (Cobos); Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, Maryland (Braunstein); Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia (Abuabara, Chu, James). Corresponding Author: William James, MD, Department of Dermatology, Perelman School of Medicine, 2 Maloney Bldg, 3600 Spruce St, Philadelphia, PA 19104 ([email protected]). Published Online: July 16, 2014. doi:10.1001/jamadermatol.2013.9451. Conflict of Interest Disclosures: None reported. 1. Chi CC, Wang SH, Lin PY. Combined epidermal-connective tissue nevus of proteoglycan (a type of mucinous nevus): a case report and literature review. J Cutan Pathol. 2009;36(7):808-811. 2. Perez-Crespo M, Lopez-Navarro N, Betlloch I, Herrera E, Niveiro M, Gallego E. Acquired and familial mucinous nevus. Int J Dermatol. 2011;50(10):1283-1285. 3. Song BH, Park S, Park EJ, Kwon IH, Kim KH, Kim KJ. Mucinous nevus with fat: an unusual case report and literature review. Am J Dermatopathol. 2012;34(8): e146-e148. 4. Vukicevic JS, Milobratovic DJ, Milinkovic MV, Bogdanovic Z. Extensive, adulthood inflammatory linear verrucous epidermal nevus associated with mucinous nevus. Indian J Dermatol Venereol Leprol. 2011;77(5):607-608. jamadermatology.com

A, Hypoplasia of the left chest wall and oligodactyly of the left hand. The 2 digits of left hand seen in the photograph were reconstructed from his toes. B, Blaschkolinear distribution of bluish-black thin papules and plaques on his left leg.

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