CASE LETTER

Atrophodermalike Guttate Morphea

Nicholas A. Ross, MD; Jason B. Lee, MD; Matthew S. Keller, MD

associated arthropod assault. She was treated by an out- PRACTICE POINTS side dermatologist without result for presumed tinea ver- • Atrophodermalike guttate morphea is a potentially sicolor. A follow-up superficial shave biopsy cited subtle underreported or undescribed entity consisting of a psoriasiform dermatitis. Topical steroids did not improve combination of clinicopathologic features. the lesions. Her medical history also was remarkable for a • Widespread hypopigmented macules on the reportedly unprovoked complete rotator cuff tear. trunk and extremities marked by thinned collagen, Physical examination revealed 0.5- to 2.0-cm, ill- fibroplasia, and altered fragmented elastin in the defined, perifollicular and nonfollicular, slightly scaly papillary dermis and upper reticular dermis are the macules and patchescopy on the trunk, arms, and legs. There key features. was no follicular plugging (Figure 1A). The hands, feet, • Atrophoderma, morphea, and lichen sclerosus face, and mucosal surfaces were spared. She had no et atrophicus should be ruled out during clinical workup. family history of similar lesions. Although atrophic in appearance, a single lesion on the left thigh was palpably depressednot (Figure 1B). Serology demonstrated a normal complete blood cell count and comprehensive meta- To the Editor: bolic panel, and negative Lyme titers. Light therapy and Morphea, atrophoderma, guttate lichen sclerosus et atro- topical steroids failed to improve the lesions; calcipotriene phicus (LS&A), anetoderma, and their subtypesDo are cream 0.005% made the lesions erythematous and pruritic. inflammatory processes ultimately leading to dermal A biopsy from a flank lesion demonstrated a normal remodeling. We report a case of a scaly, hypopigmented, epithelium without thinning, a normal basal melanocyte macular rash that clinically appeared as an entity along population, and minimally effaced rete ridges. Thin col- the morphea-atrophoderma spectrum and demonstrated lagen bundles were noted in the upper reticular and unique histopathologic changes in both collagen and papillary dermis with associated fibroplasia (Figure 2). elastin confined to the upper reticular and papillary der- Verhoeff-van Gieson stain revealed decreased and frag- mis. This case is a potentially rare variant representing a mented elastin filaments in the same dermal distribution combination of clinical and microscopicCUTIS findings. as the changed collagen (Figure 3). There was no evi- A 29-year-old woman presented for an increasing dence of primary inflammatory disease. The dermis was number of white spots distributed on the trunk, arms, thinned. Periodic acid–Schiff stain confirmed the absence and legs. She denied local and systemic symptoms. of hyphae and spores. The patient reported that she was stung by 100 wasps The relevant findings in our patient including the fol- 23 years prior. Following the assault, her grandmother lowing: (1) onset of hypopigmented macules and patches placed chewed tobacco leaves atop the painful erythema- following resolution of a toxic insult; (2) initially stable tous wheals and flares. Upon resolution, hypopigmented number of lesions that progressed in number but not size; macules and patches remained in their place. The patient (3) thinned collagen associated with fibroplasia in the denied associated symptoms or new lesions; she did not upper reticular and papillary dermis; (4) decreased num- seek care at that time. ber and fragmentation of elastin filaments confined to the In her early 20s, the patient noted new, similarly dis- same region; (5) no congenital lesions or similar lesions in tributed hypopigmented macules and patches without family members; and (6) a complete rotator cuff tear with

From the Department of Dermatology and Cutaneous Biology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania. The authors report no conflict of interest. Correspondence: Matthew S. Keller, MD, Department of Dermatology and Cutaneous Biology, 833 Chestnut St, Ste 740, Philadelphia, PA 19107 ([email protected]).

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FIGURE 1. Multiple slightly scaly, hypopig- mented macules coalescing into patches on the flank (A) as well as hypopigmented macules and a minimally depressed patch on the left thigh (B). A B

copy FIGURE 2. Normal epidermis with minimally effaced rete ridges and thinned collagen in the upper reticular and papillary dermis, not seen in the lower not dermis, without overall thinning of the dermis (A)(H&E, original magnification ×40). Normal collagen bundles in the lower Do reticular dermis (B)(H&E, original magnification ×200). A B

no findings of a systemic connective-tissue disorder such elastolysis (patient 1 and patient 2, respectively).1 Our as Ehlers-Danlos syndrome. patient’s lesions were hypopigmented and atrophic in We performed a literatureCUTIS search of PubMed arti- appearance but were slightly scaly and also involved the cles indexed for MEDLINE using combinations of the extremities. Distinct from these patient reports, histopa- terms atrophic, hypopigmented, white, spot disease, confetti- thology from our case demonstrated thin packed collagen like, guttate, macules, atrophoderma, morphea, anetoderma, bundles and decreased fragmented elastin filaments con- elastin, and collagen to identify potentially similar reports fined to the upper reticular and papillary dermis. of guttate hypopigmented macules demonstrating Plaque morphea is the most common type of changes of the collagen and elastin in the papillary and localized scleroderma.2 The subtype APP demonstrates upper reticular dermis. Some variants, namely atropho- round to ovoid, gray-brown depressions with cliff-drop derma of Pasini and Pierini (APP), guttate morphea, and borders. They may appear flesh colored or hypopig- superficial morphea, demonstrate similar clinical and mented.3,4 These sclerodermoid lesions lack the viola- histopathologic findings. ceous border classic to morphea. Sclerosis and induration Findings similar to our case were documented in also are typically absent.5 Clinically, our patient’s macules case reports of 2 women (aged 34 and 42 years)1 pre- resembled this entity. Histopathologically, APP shows senting with asymptomatic, atrophic, well-demarcated, normal epithelium with an increased basal layer pig- shiny, hypopigmented macules over the trunk and upper mentation; preserved adnexal structures; and mid to extremities, which demonstrated a thinned epidermis lower dermal collagen edema, clumping, and homog- with coarse hyalinized collagen bundles in the mid enization.3,4 Elastic fibers classically are unchanged, with and lower dermis. There was upper and diffuse dermal exceptions.6-11 Changes in the collagen and elastin of our

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FIGURE 3. Normal epidermis (A) (Verhoeff-van Gieson, original mag- nification ×40). Normal elastin net- work in the lower reticular dermis; note the normal size of elastin fibers (B)(Verhoeff-van Gieson, original A B magnification ×200).

patient were unlike those reported in APP, which occur in however, were characteristic findings of LS&A, including the mid to lower dermis. upper dermal homogenization, near-total effacement Guttate morphea demonstrates small, pale, min- of rete ridges, orthokeratosis, and vacuolar degeneration imally indurated, coin-shaped lesions on the trunk. at the dermoepidermal junction. As such, this entity is Histopathology reveals less sclerosis and more edema, less compatible. resembling LS&A.12 The earliest descriptions of this entity Atrophodermacopy elastolyticum discretum has clinical describe 3 stages: ivory/chalk white, scaly, and atrophic. features of atrophoderma with elastolytic histopathologic Follicular plugging (absent in this patient) and fine scale findings.1 Anetoderma presents with outpouchings of can exist at any stage.13,14 Flattened rete ridges mark atrophic skin with a surrounding ring of normal tissue. an otherwise preserved epidermis; hyalinized collagen Histopathologically, this entity shows normal collagen typically is superficial and demonstrates less sclerosis withnot elastolysis; there also is a decrease in desmosine, an yet increased edema.12-14 Fewer elastic fibers typically are elastin cross-linker.1,3 Neither the clinical nor histopatho- present compared to normal skin. Changes seen in this logic findings in this patient matched these 2 entities. entity are more superficial, as with our patient, than clas- The reported chronologic association of these lesions sic scleroderma. However, classic edema was not foundDo in with an arthropod assault raised suspicion to their associ- our patient’s biopsy specimen. ation with toxic insult or postinflammatory changes. One Superficial morphea, occurring predominantly in study reported mechanical trauma, including insect bites, females, presents with hyperpigmented or hypopig- as a possible inciting factor of morphea.11 These data, mented patches having minimal to no induration. The gathered from patient surveys, reported trauma associ- lesions typically are asymptomatic. Histopathologically, ated to lesion development.1,17 A review of the literature collagen deposition and inflammation are confined regarding atrophoderma, morphea, and LS&A failed to to the superficial dermis without homogenization asso- identify pathogenic changes seen in this patient following ciated with LS&A, findingsCUTIS that were consistent with initial trauma. Moreover, although it is difficult to prove this patient’s biopsy.15,16 However, similar to other causality in the formation of the original hypopigmented morpheaform variants, elastic fibers are unchanged.15 spots, the development of identical spots in a similar dis- Verhoeff-van Gieson stain of the biopsy (Figure 3) tribution without further trauma suggests against these showed the decreased and fragmented elastin network etiologies to fully explain her lesions. Nonetheless, cir- in the upper reticular and papillary dermis, making this cumstance makes it difficult to prove whether the original entity less compatible. arthropod insult spurred a smoldering reactive process Guttate LS&A may present with interfollicular, blu- that caused the newer lesions. ish white macules or papules coalescing into patches or Hereditary connective-tissue disorders also were con- plaques. Lesions evolve to reveal atrophic thin skin with sidered in the differential diagnosis. Because of the follicular plugging. Histology demonstrates a thinned patient’s history of an unprovoked complete rotator cuff epidermis with orthohypokeratosis marked by flattened tear, Ehlers-Danlos syndrome was considered; however, rete ridges. The dermis reveals short hyalinized collagen the remainder of her examination was normal, making a fibrils with a loss of elastic fibers in the papillary and upper syndromic systemic disorder a less likely etiology. reticular dermis, giving a homogenized appearance. Early Because of the distinct clinical and histopathologic disease is marked by an inflammatory infiltrate.17 Most findings, this case may represent a rare and previously of these findings are consistent with our patient’s pathol- unreported variant of morphea. Clinically, these hypopig- ogy, which was confined to the upper dermis. Lacking, mented macules and patches exist somewhere along

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the morphea-atrophoderma spectrum. Histopathologic 6. Pullara TJ, Lober CW, Fenske NA. Idiopathic atrophoderma of Pasini findings do not conform to prior reports. The name atro- and Pierini. Int J Dermatol. 1984;23:643-645. 7. Jablonska S, Szczepanski A. Atrophoderma Pasini-Pierini: is it an entity? phodermalike guttate morphea may be an appropriate Dermatologica. 1962;125:226-242. appellation. It is possible this presentation represents 8. Ang G, Hyde PM, Lee JB. Unilateral congenital linear atrophoderma of a variant of what dermatologists have referred to as the leg. Pediatr Dermatol. 2005;22:350-354. white spot disease.18 We hope that this case may bring 9. Miteva L, Kadurina M. Unilateral idiopathic atrophoderma of Pasini and others to discussion, allowing for the identification of Pierini. Int J Dermatol. 2006;45:1391-1393. 10. Kee CE, Brothers WS, New W. Idiopathic atrophoderma of Pasini a more precise entity and etiology so that patients may and Pierini with coexistent morphea. a case report. Arch Dermatol. receive more directed therapy. 1960;82:100-103. 11. Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: REFERENCES clinical and epidemiological features in 750 children. an international 1. Aksoy B, Ustün H, Gulbahce R, et al. Confetti-like macular atrophy: a study. Rheumatology. 2006;45:614-620. new entity? J Dermatol. 2009;36:592-597. 12. Winkelmann RK. Localized cutaneous scleroderma. Semin Dermatol. 2. Uitto J, Santa Cruz DJ, Bauer EA, et al. Morphea and lichen sclerosus 1985;4:90-103. et atrophicus. clinical and histopathologic studies in patients with com- 13. Dore SE. Two cases of morphoea guttata. Proc R Soc Med. 1918;11:26-28. bined features. J Am Acad Dermatol. 1980;3:271-279. 14. Dore SE. Guttate morphoea. Proc R Soc Med. 1919;12:3-5. 3. Buechner SA, Rufli T. Atrophoderma of Pasini and Pierini. clinical 15. McNiff JM, Glusac EJ, Lazova RZ, et al. Morphea limited to the superfi- and histopathologic findings and antibodies to Borrelia burgdorferi in cial reticular dermis: an underrecognized histologic phenomenon. Am J thirty-four patients. J Am Acad Dermatol. 1994;30:441-446. Dermatopathol. 1999;21:315-319. 4. Saleh Z, Abbas O, Dahdah MJ, et al. Atrophoderma of Pasini and 16. Jacobson L, Palazij R, Jaworsky C. Superficial morphea. J Am Acad Pierini: a clinical and histopathological study. J Cutan Pathol. Dermatol. 2003;49:323-325. 2008;35:1108-1114. 17. Bolognia J, Jorizzo JL, Rapini RP, eds. Dermatology. 2nd ed. London, 5. Canizares O, Sachs PM, Jaimovich L, et al. Idiopathic atrophoderma England: Mosby Elsevier; 2007. of Pasini and Pierini. Arch Dermatol. 1958;77:42-58; 18. Bunch JL. White-spot disease (morphoea guttata). Proc R Soc Med. discussion 58-60. 1919;12:24-27. copy

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