Poland Syndrome Coexisting with Blaschkolinear Congenital Melanocytic Nevi

Letters

Those who support the connective tissue origin will fur- 5. Tardío JC, Granados R. The cellular component of the mucinous nevus ther divide mucinous nevi into 2 histopathologic types: a con- consists of CD34-positive fibroblasts. J Cutan Pathol. 2010;37(9):1019-1020. nective tissue nevus of the proteoglycan (CTNP) type and com- 6. Concheiro J, Pérez-Pérez L, Peteiro C, Labandeira J, Toribio J. Discrete 1 papular lichen myxoedematosus: a rare subtype of cutaneous mucinosis. Clin bined epidermal-CTNP type. In CTNP, the epidermis is normal, Exp Dermatol. 2009;34(8):e608-e610. while in epidermal-CTNP, the epidermis shows hyperkerato- sis and acanthosis with elongation of the rete ridges consis- 1 tent with an epidermal nevus. Regardless of classification, the Poland Syndrome Coexisting With Blaschkolinear origin of increased mucin is unclear. Although mucin is syn- Congenital Melanocytic Nevi thesized by fibroblasts, previous studies have shown only a Poland syndrome is an uncommon congenital anomaly slight increase in activated fibroblasts. Thus, some have pos- characterized by unilateral chest wall and upper extremity tulated that the upregulation of fibroblasts is responsible for hypoplasia.1 It is theorized to be due to subclavian artery the increased mucin.3 hypoplasia during embryogenesis.1 There have been only a Clinically, these lesions can be hard to distinguish from con- few isolated reports of Poland syndrome with congenital nective tissue nevi such as collagenomas or elastomas. Histo- dermatoses.2-4 Adding to this association list, we report a case logic examination is essential to exclude these entities, which of Poland syndrome with multiple congenital melanocytic can be associated with congenital abnormalities. The micro- nevi (CMN). Additionally, the nevi are in a Blaschkolinear scopic differential diagnosis is narrow and includes focal cuta- arrangement, which is also rare; we know of only 2 such cases neous mucinosis and lichen myxedematosus, both of which have reported previously.5,6 distinct clinical presentations. Lesions of focal cutaneous mucinosis are usually solitary papules and can be found anywhere Report of a Case | An otherwise healthy boy in his teens pre- on the body. Lichen myxedematosus presents with a slow on- sented for asymptomatic hyperpigmented birthmarks on his set of asymptomatic or slightly pruritic papules, which can be left leg that had grown proportionately to his leg’s growth. His generalized or localized. Generalized subtypes of lichen myx- medical history was significant for Poland syndrome, for which edematosus have an associated monoclonal gammopathy,while he had undergone multiple left-hand reconstruction surger- discrete papular lichen myxedematosus, a variant of the local- ies. His family history was noncontributory. Physical exami- ized subtype, has been associated with human immunodefi- nation revealed oligodactyly of the left hand, underdevel- ciency virus disease.6 Other histologic mimics, like self-healing oped left chest wall (Figure 1A), and multiple, evenly pigmented, papular mucinosis and acral persistent papular mucinosis, are bluish-black thin papules and plaques on the left posterior thigh, easily excluded with clinical information. In our case, clinical cor- calf, and ankle in a Blaschkolinear distribution (Figure 1B). A relation 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 le- Figure 1. Clinical Images of Patient With Poland Syndrome sion, but scarring will result. and Blaschkolinear Congenital Melanocytic Nevi

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

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. 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. 4. Vukicevic JS, Milobratovic DJ, Milinkovic MV, Bogdanovic Z. Extensive, B, Blaschkolinear distribution of bluish-black thin papules and plaques on his adulthood inflammatory linear verrucous epidermal nevus associated with left leg. mucinous nevus. Indian J Dermatol Venereol Leprol. 2011;77(5):607-608.

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tion between the 2 conditions seems to be coincidental, as in Figure 2. Lesional Biopsy Specimen Obtained From the Left Thigh previously reported cases of Poland syndrome with other congenital dermatologic findings.

Thomas Lam, BA Yongxue Yao, MD, PhD Joanne Trockman, MD

Author Affiliations: Department of Dermatology, Indiana University School of Medicine, Indianapolis. Corresponding Author: Yongxue Yao, MD, PhD, Department of Dermatology, Indiana University School of Medicine, 545 Barnhill Dr, EH139, Indianapolis, IN 46202 ([email protected]). Published Online: July 9, 2014. The photograph shows a junctional nevus with congenital features doi:10.1001/jamadermatol.2013.9687. (hematoxylin-eosin, original magnification ×200). Conflict of Interest Disclosures: None reported. 1. Fokin AA, Robicsek F. Poland’s syndrome revisited. Ann Thorac Surg. 2002;74 (6):2218-2225. skin biopsy specimen from the left posterior thigh showed char- 2. Vélez A, Moreno J. Poland’s syndrome and recessive X-linked ichthyosis in acteristics consistent with CMN (Figure 2). two brothers. Clin Exp Dermatol. 2000;25(4):308-311. 3. Riyaz N, Riyaz A. Poland syndrome (anomaly) with congenital hemangioma: Discussion | Poland syndrome, named after the British surgeon a new association. Indian J Dermatol Venereol Leprol. 2006;72(3):222-223. Alfred Poland who first described it in 1841, is an uncommon, 4. Gocmen H, Akkas Y, Doganay S. Poland syndrome: rare presentation in two sporadic, and very rarely inherited birth defect characterized cases. N Z Med J. 2010;123(1321):71-77. by unilateral chest wall hypoplasia (often right-sided) and 5. Effendy I, Happle R. Linear arrangement of multiple congenital melanocytic ipsilateral hand deformity (most often syn-brachydactyly nevi. J Am Acad Dermatol. 1992;27(5 Pt 2):853-854. and less often oligodactyly).1 Poland syndrome affects boys 2 6. Droitcourt C, Adenis-Lamarre E, Ezzedine K, Taïeb A, Jouary T. Blaschkolinear congenital melanocytic nevus. Dermatology. 2009;219(2):182-183. to 3 times as often as girls, with an estimated incidence of 1 in 10 000 to 100 000 live births. Its exact cause remains unclear, Uncommon Presentation of Pityriasis Rosea but a prevailing theory is hypoplasia of the subclavian artery After Yellow Fever Inoculation during the critical sixth week of gestation, which leads to a range of musculoskeletal malformations.1 Report of a Case | A man in his 30s was referred for acute onset of Poland syndrome has been associated with hematopoi- pruritic scaly eruptions of the groin, penis, scrotum, and pubic etic malignancies such as leukemia and non-Hodgkin lym- mound. The lesions started to appear 3 weeks prior to presen- phoma, as well as other syndromes, including Möbius syn- tation with an oval erythematous lesion located around the left drome (characterized by congenital bilateral facial paralysis thigh. About 7 days after the appearance of the first lesion, oth- with inability to abduct the eyes) and Klippel-Feil syndrome ers began to appear. The patient reported that 2 weeks before the (characterized by congenital fusion of any 2 of the 7 cervical appearance of the first lesion, he had been inoculated against yel- vertebrae).1 In addition to these known associations, a litera- low fever and had an episode of coryza and hacking cough. ture review revealed a few isolated cases of Poland syndrome The lesions consisted of multiple, coalescent oval plaques with various congenital dermatologic findings, including re- of 0.2 cm to 4 cm in longest diameter (Figure) with atypical cessive X-linked ichthyosis, congenital hemangioma, and café- scales. Other skin areas and mucosal surfaces were unaf- au-lait spots.2-4 To our knowledge, our case is the first re- fected. The findings of general and systemic examinations were ported case of Poland syndrome associated with multiple CMN. normal. Skin scrapings for potassium hydroxide examina- A CMN is a benign, clonal proliferation of melanocytes in the tion, complete blood cell counts, urinalysis, blood glucose as- epidermal, dermal, or subcutaneous tissue that is present at or say, VDRL (Venereal Disease Research Laboratory) test, and hu- shortly after birth, with an estimated prevalence of 0.5% to 31.7%, man immunodeficiency virus antibodies were all normal. The depending on the study. They are usually solitary, but 3% are pruritus and eruptions cleared within 6 weeks following treat- multiple, occasionally arranged in a cluster and rarely in a lin- ment with mometasone furoate cream and oral Levoceti- ear distribution. Indeed, only 2 cases of Blaschkolinear CMN have rizine, 5 mg/d, leaving postinflammatory hyperpigmentation. been reported thus far.5,6 Our patient would represent the third such case, and it occurs in a patient with Poland syndrome. Discussion | Pityriasis Rosea (PR) is a self-limiting papulosqua- The definite risk of developing melanoma in Blaschkolin- mous disorder typically characterized by sudden onset of a ear CMN remains unclear because of its rarity. Our patient had larger scaly plaque (herald patch) followed (about 1-2 weeks no clinical sign of malignant transformation by age 11 years, later) by eruptions of multiple, bilateral, smaller, scaly oval or as in the 2 previously reported cases at age 15 and 28 years. round lesions that follow the Langer lines of cleavage on the However, long-term close clinical monitoring might be war- trunk and proximal parts of extremities. Skin lesions usually ranted, as in giant CMN. last about 6 weeks. Current evidence indicates that PR is a type In summary, we report herein a rare case of Blaschkolin- of viral exanthema and the cause may be linked to human her- ear CMN in a teenager with Poland syndrome. The associa- pes virus (HHV)-6 and HHV-7.1

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