Nodular Morphea

Case Report Dermatology 2009;218:63–66 Received: July 13, 2008 DOI: 10.1159/000173976 Accepted: July 23, 2008 Published online: November 13, 2008 Nodular Morphea a b c F. Kauer J.C. Simon M. Sticherling a b Department of Dermatology and Venerology, Vivantes Klinikum Neukölln, Berlin , Department of Dermatology, c Venerology and Allergology, University of Leipzig, Leipzig , and Department of Dermatology, Venerology and Allergology, University of Erlangen, Erlangen , Germany Key Words can range in size from 2 mm to 4–5 cm, flamed skin that is already involved in an -Scleroderma ؒ Keloid ؒ Hypertrophic scar ؒ usually appear spontaneously and tend to active fibrotic process inherent to the dis Morphea involve the trunk and upper extremities. ease in those patients who are genetically A linear presentation has also been de- predisposed to keloid development, or at scribed. The literature on this topic is con- sites of the skin that show a high predilec- Abstract fusing because the terms ‘nodular sclero- tion for keloid formation, such as the trunk Scleroderma may present as being strictly derma’ and ‘keloidal scleroderma’ are used [6, 7] . limited to the skin, as in morphea, or within interchangeably even though there is a a multiorgan disease, as in systemic sclero- great degree of variability in the histologi- sis. Accordingly, cutaneous manifestations cal findings of these nodules [4] . In con- C a s e R e p o r t vary clinically. In nodular or keloidal sclero- trast, other authors stress that the cutane- derma, patients develop lesions that are ous manifestations may vary clinically, but Medical History clinically indistinguishable from a keloid; all share the same histopathological pat- A 16-year-old girl presented with mul- however, the histopathological findings are tern of both morphea/scleroderma and ke- tiple progressive morpheic skin lesions more variable. We describe a 16-year-old girl loid [5] . limited to the abdomen. These lesions had with morpheic lesions for 3–4 years and ad- Nodular or keloidal scleroderma may developed over the course of 3–4 years ditional development of keloidal nodules represent a keloidal response of the in- without any tendency to heal. Six months within these lesions. The histological examination revealed a hypertrophic scar besides morphea. Copyright © 2008 S. Karger AG, Basel Introduction Several clinical presentations of both systemic and localized scleroderma are described. The nodular or keloidal variant is one of the rarest forms. It was first de- Color version available online scribed by the English physician Thomas Addison in the mid 1800 as ‘untrue keloid’. Later on, Paul Gerson Unna included an article about this variant of scleroderma in his textbook on the histology of skin diseases [1–3] . Nodular scleroderma can occur in association with progressive systemic scleroderma or with localized morphea. It most- Fig. 1. Clinical presentation of the morpheic and nodular lesions. ly affects young and middle-aged women. The biopsy was taken from the keloidal margin of the right upper The firm elevated nontender lesions, which paraumbilical lesion. © 2008 S. Karger AG, Basel Dr. med. Friederike Kauer 1018–8665/09/2181–0063$26.00/0 Department of Dermatology and Venerology, Vivantes Klinikum Neukölln Fax +41 61 306 12 34 Rudower Strasse 48 E-Mail [email protected] Accessible online at: DE–12351 Berlin (Germany) www.karger.com www.karger.com/drm Tel. +49 130 143 601, Fax +49 130 144 995, E-Mail [email protected] Color version available online Color version available online Fig. 2. Morpheic plaque with nodular erythematous lesions on the Fig. 3. This arciform-bordered erythematous nodule with its border. slightly ‘forme lilacée’ is reminiscent of the clinical variant of su- perficial scleroderma Pasini-Pierini. Color version available online Color version available online Fig. 4. Low magnification of the histological findings from a Fig. 5. Higher magnification of the hypertrophic scar tissue: par- biopsy of a nodule: a slightly elevated hypertrophic scar with allel collagen bundles with numerous fibroblasts and perpendicu- an increased number of fibroblasts and collagen bundles. Scale larly oriented capillaries with a sparse inflammatory infiltrate. bar = 200,000 ␮ m. Scale bar = 500 ␮ m. after first manifestation of these lesions, sis, syphilis, antinuclear antibodies, anti- found limited to the abdomen, in addition additional pink firm nodules occurred centromere, anti-ENA (anti-extractable to firm pink (skin-colored) indolent nod- within the morpheic manifestations. There nuclear antigen), anti-dsDNA, antihistone, ules. These nodules were arranged in a ra- was no association with an acute infection. anti-MPO (myeloperoxidase) and anti- dial and circular pattern (see fig. 1–3 ). The patient denied any manipulation or PR3 (anti-proteinase 3) antibodies were all trauma at those sites, and there was no negative or within the normal range. H i s t o l o g i c a l F i n d i n g s family or personal history of keloid forma- Neither allergies nor internal diseases Two skin biopsies were taken from the tion. Previous treatments comprised local were known and the past medical history abdominal skin within a morpheic and a ointments for scars and topical corticoste- was uneventful. The family history was keloidal lesion (see fig. 4–5). roids without any effect. Laboratory evalu- negative for major internal diseases. In the morpheic lesion, the epidermis ations demonstrated a slightly elevated revealed basket-like orthokeratosis with blood sedimentation rate (14/37); however, Skin Examination continuous acanthosis. In the dermis, a all other inflammatory parameters were Six sharply demarcated, light brown perivascular infiltrate of inflammatory negative. Antibodies indicating borrelio- pigmented, oval flat and soft plaques were cells, mostly lymphocytes and some plas- 64 Dermatology 2009;218:63–66 Kauer /Simon /Sticherling Table 1. Algorithm for terminology: (1) systemic involvement (nodular/keloidal scleroderma); (2) no systemic involvement (nodular/keloidal morphea) Pro Contra – clear difference between systemic and nonsystemic – histological findings too varied [7] involvement [6] – overlap of symptoms – treatment after exact classification – etiopathogenic mechanism still unclear – better possibility for follow-up/further investigations if common classification is used ma cells, was found. The bundles of colla- have a characteristic histological appear- In our patient, no systemic involve- gen were partially thickened, hypereosin- ance with broad, homogenous, brightly ment could be found, and the histopatho- ophilic and in parallel orientation. These eosinophilic collagen bundles in a haphaz- logical findings of the plaque-like lesion findings were interpreted as morphea. ard pattern, and a distinct elevation above were consistent with morphea. As in addi- The keloidal lesions presented as a the surrounding skin surface. In contrast, tion the nodular lesions did not show any nodular dermal tumor and overlying epi- hypertrophic scars are only slightly elevat- characteristics of a keloid but a hypertro- dermis with basket-like orthokeratosis ed and the collagen bundles are character- phic scar, we concluded that ‘nodular mor- and irregular acanthosis. In the dermis, istically oriented parallel to the skin sur- phea’ should be diagnosed. The fact that thickened and curly bundles of collagen face, as are the accompanying fibroblasts. we would not have established this diagno- oriented parallel to the skin surface with Capillaries run perpendicularly to the skin sis in the absence of any morpheic lesions many small vessels and discrete lympho- surface [8] . In the elastica-Giemsa stain- shows the importance of a clear classifica- cytic infiltrates were found. These find- ing, elastic fibers are diminished. tion (see table 1 ). This can be difficult in ings represented a hypertrophic scar. As in our case the histopathological the case of overlapping symptoms. There findings represent a hypertrophic scar, the might be a continuous spectrum, but to Therapy and Course of Disease clinical findings have to be interpreted as enlighten this issue we need further inves- The patient was treated intravenously ‘nodular’ type rather than ‘keloidal’. In the tigations and a follow-up of the patients with penicillin G 10 Mega daily for 10 literature, there is no consistent use of the when the symptoms have started. days. terms ‘nodular’ and ‘keloidal’ scleroder- Apart from precisely defining the dis- An artificial cause or self-manipulation ma. Some authors define nodular/keloidal ease clinically and histologically, underly- was excluded by psychological examina- scleroderma as a fibrosing reaction in pa- ing etiopathogenic mechanisms still re- tions, which also described the psychologi- tients with systemic or localized scleroder- main unclear. As demonstrated by immu- cal coping with the disease as appropriate. ma with highly variable histological find- nohistochemical and in situ hybridization For topical treatment of the morpheic ings. In one report, both hypertrophic and examination of the extracellular matrix and nodular lesions, crème-PUVA therapy keloid scarring were found simultaneously protein tenascin and connective tissue was initiated and continued up to a cumu- [9] . Because of the unclear nature of nodu- growth factor, the regulation of the con- lative dose of 2.32 J/cm 2 with slight im- lar/keloidal scleroderma, many authors nective tissue metabolism varies among provement,

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