Case report Morphea mimicking dermatitis artefacta Deep necrotising morphea mimicking dermatitis artef acta M. Melato, N. Gorji, O. Marangoni and G. Pillitteri SUMMARY The case of a 45-year old woman affected by facial ulcerative dermatitis diagnosed as dermatitis artefacta was reconsidered on the basis of the histological findings, which were consistent with a very unusual type of morphoae. The extraordinary features of this case, which we suggest calling »deep necrotising morphoea«, explain the diagnostic pitfall while highlighting the inadequacy of current classification of morphoea. Introduction In 1954, in a basic paper on scleroclerma, Leinwancl et al. asserted that "In reviewing the histo1y of scle- roclerma one is impressed by the methamorphosis of opinion regarcling the classification ofthis disease" (1). 41 years later, other authors confirmed the persistency of an incomplete and confusing classification of mor- phoea and, while proposing a new one, they observed that the categories they were describing were not necessarily mutually exclusive and that the subtypes often co-occurred in the same patient (2). Therefore no exhaustive classification clearly clistinguishes the diffe- rent existing subtypes of morphoea, especially mor- phoea characterized by unusual features, exists at pre- sent and this fact may determine diagnostic pitfalls. There exist cases characterized by unusual features, which may present diagnostic pitfalls. Case report A 45-year-old woman noted the presence of a small facial dyschromia associated with cleep induration, which in 4 months became complicated by blistering. Because of the progression of the lesion to a facial ulcerative dermatitis, a year later she was examined at a dermatological unit where the lesion was diagnosed as pathomimic ulcer of the face ancl treatecl with local antibiotic therapy. 11 months later, the patient was re- ferrecl to one ofthe Authors (G.P.) complaining of pro- gression ofthe disease complicatecl by facial sensitivity to cole! and warm. The examination revealecl in the right cheek a well demarcatecl, rather linear, subcutaneous induration covered by skin containing a superficial fistula discharging necrotic whitish material. The enlarging lesion showed a delicate violaceous border. Other symptoms and signs, including Raynaud's pheno- Acta Dermatoven APA Vol 9, 2000, No 4 - -----------------JJJ Morphea mimicking dermatitis artefacta menon, were lacking. Laboratory tests disclosed normal or negative CBC with differential count, urinalysis, !iver function tests, rheumatic factor, levels of C3 and C4, and LE cel! tests; antinuclear, anti-DNA, anti-Borrelia- burgdorferi, anti-toxoplasma IgG and IgM antibodies as well as anti-HIV antibodies were negative. ESR was elevated. An adequate biopsy sample taken from the right cheek showed flattened epidermis. Dermis and septa of the subcutaneous fat were composed mainly of wavy collagen fibers , faintly stained with hematoxylin-eosin except at the borders, where there were more fibroblast and newly formed thick capillaries infiltrated by a sprinkling of lymphocytes and plasma cells. Dermal appendages were absent and there was no evidence of mucin (Fig. 1). Collagen bundles were seen deeply into the subcutaneous tissues where they encircled and separated the muscle fibres , causing atropia and necrosis (Fig. 2A & B); vessels and nerves were also involved by the fibrosis and deep arterioles showed hyperplastic walls (Fig. 3A & B) . Homogenization of collagen bundles occurred especially in the deep dermis and subcutis . A necrotic area, poorly circumscribed by macrophages, epithelioid cells, and few eosinophils, was present superficially. Some black, finely granular, foreign material was intermingled with the necrotic tissue (Fig. 4), but no trace of it was found elsewhere. When the woman was asked about the possible source of the foreign material, she hypothesized that it came from the toner cartridges from printers, as she had been working (exposure to solvents) as a cleaner in a bank for five years. She denied self-inflicted injury and use/ abuse of colored chemicals or cosmetic substances. A month later the disease had progressed and two new ulcerative lesions had developed in the left face: the first was oval and located in the zygomatico-orbital region and the second, rather linear, in the naso-orbital region (Fig. 5). In the following twelve months the clinical features did not improve. Discussion Pathomimicry occurs in al fields of medicine. It may aim to obtain a precise material benefit or it may be an expression of psychopathological behavior and, in this second case, it is quite common in intelligent yourig woman. Since it is important for the practitioner to avoid accusing these patient or attempting to get them to admit to pathomirnicry as they are likely to have exaggerated or self-destructive responses, great care must be taken to avoid misdiagnoses. Precisely with this aim, a biopsy was performed in a "difficult" area such as the face of our patient. In the present case, morphoea exhibited a very unusual and misleading clinical feature: the presence 1J6 Figure 1. The epidermis is flattened; dermis and subcutaneous fat are replaced by collagen (H&E; original magnification X2.5). Figure 2. Collagen bundles penetrate deeply into the subcutaneous tissues where they encircle and separate the muscle fibers (A); in B, the arrow indicates where a muscle fiber is constricted by fibrous tissue and undergoes necrosis (AZAN-MALLORY; original magnification A X 2.5 and B X 40). Case report Acta Dermatoven APA Vol 9, 2000, No 4 C ase r epo r t Acta Dermatoven APA Vol 9, 2000, No 4 Morphea mimicking dermatitis artefacta of ulcerative dermatitis with necrosis . This feature has rarely been reported in Literature and always in the extremities, usually at the extreme tip of the finger, close to the nail (1), or on the lower extremities where lymp- hatic obstruction combines with increased hydrostatic pressure (3) . Out of 53 patients with linear scleroderma, cutaneous ulceration of the involved extremity occurred in only 1 case ( 4). Histologically, a precise classification of our case was also difficult. In fact, according to a recent classification (2), the case showed some relationship with morphoea profunda (according to Su and Person (5) as reported by Su and Green in 1986 (6), although in our case inflammation was poor. However, considering the hi- story of blistering reported by the patient and her clinical features as shown in Fig. 5, the case was also related to the bullous subtype, a very rare form since, out of a striking sample of 1071 patients with morphoea and scleroderma only 13 have shown bullous changes (3). Despite their rarity, it is noteworthy that bullae were first described in a patient with morphoea in 1896 (7) . The bullous manifes tation are considered a conse- quence of lymphatic obstruction caused by the sclero- dermatous process (8-9) or of vascular changes such as arteriitis and phlebosclerosis (10). As further causes of bullous manifestations a localized trauma (6, 11-13) or of the eosinophils, which are often present in cutaneous tissue affected by morphoea (3), can be mentioned. Considering that the classification of localized mor- phoea seems to represent a collection of cases with different, although often overlapping, features rather than a predse system based on anatomo-clinical evi- dence, we have preferred to suggest a new definition for our case, i. e. "deep necrotising morphoea" , to high- light its main histological features. We could willfully assign the case one of the mentioned subtypes, though none of these matches in features, but in this case we would not be contributing to advancing the knowledge on morphoea. Figure 3. This deep arteriole is encircled by collagenous fibers and its wall is hyperplastic (A); the nerve showed in B is also surrounded by the fibrotic tissue (H&E; original magnification X 25). Figure 4. Necrotic tissue intermingled with black, finely granular foreign material, poorly circumscribed (H&E; original magnification X 10). Figure 5. Scars on t he face: that in the zygomatico-orbital region is oval and that in the naso-orbital region is rather linear. - - ----------- - - ---1$7 Morphea mimicking dermatitis artefacra The pathogenesis of the uicerative dermatitis with necrosis in the reported case was amply explainecl by the striking collagenous deposition not only in the cler- mis but also in the subcutaneous tissue, which causecl lymphatic obstruction and, at a deeper leve!, arteriolar stenosis complicatecl by ischemic necrosis of the tissues above. Histologically, bullae were not evidenc given progression ancl severity of the clisease, but blistering was observec.l by the pacient at onset anc.l the oval ulce- ration which developed one month after the biopsy looked like an erodecl bulla. Pathogenesis ofthe bullous iesions can be explainecl by ali the mechanisms previo- usly reportec.l in literature. Finally, the involvement of nerves by collagenous cleposition observecl in our case was in agreement with the peripheral neuropathy pre- viously reportecl in morphoea (14) anc.l seemec.l able to exercise a negative neurotrophic effect. Furthermore, although not convincingly relatec.l to the c.lisease in our case, it seems noteworthy that profe- ssional exposure to solvents has been previously consi- c.lerec.l a possible cause of morphoea (15). Conclusion The present classification of morphoea appears to be inac.lequate since it incluc.les a number of insuffi- ciently c.lefinecl subtypes , which fail to account for ali the anatomo-clinical variables, encountered. Such ineffectiveness can leac.l to serious c.liagnostic pitfalls, as it happenec.l in our case where exclusion of dermatitis artefacta requirecl a facial biopsy. Acknowledgement The presence of foreign material exclusively insicle the necrotic tissue, without evidence of cellular reaction, confirmed the hypothesis of external contamination. The authors thank Daria Facciotti for her expert technical assistance. EJ ·1;,FV'O ;;''f'I.Tr''f? C .l . l l .A .:< . l . .4 .l.\. ! . ...i .1. '! \_ _ _. .l.,c l:, Edito rial Note Deep necrotising moiphoea mimic!zing dermatitis artefacta or dermatitis ai11q/'acta mimiching necrotising morphoea? This editorial note is written in the spirit of open-mindedness andj'airness hoth to the authors and to the readers, hecause obviously the editors of a journal have a responsibility concerning the scient[/ic and medica! content qf an article. We disagree with the diagnostic inte1pretation qf the authors hased on the study qf the clinical photographs and the histopathologic specimens, which were fandly provided by the authors. So, in our interpretation this case represents a dermatitis ai1e/'acta somewhat mimic!zing a morphoea. Neve11heless, we did not want to reject this manuscript, hecause the authors were convinced on their inteipretation and we lihe to give them the opportunity to present their concept qf deep necrotising 11101phoea. The readers are encouraged to decide whether they accept the authors' concept qf deep necrotising moiphoea or prtqfer the diagrzosis dermatitis arttq/'acta as we do. Editors 1. Leinwand I, Duryee W, Richter MN. Scleroderma (based ona study of over 150 cases) . Ann lntern Med 1954; 41: 1003-41. 2. Peterson LS, Nelson AM, Su WPD. Classification of morphea (localized scleroderma). Mayo Ciin Proc 1995; 1068-76. 3. Daoud MS, Su WPD, Leiferman KM, Perniciaro C. Bullous morphea: clinical pathologic, and immunologic evaluation of thirteen cases. J Am Acad Dermatol 199-i; 30:937-43. 4. Falanga V, Medsger TAJr, Reichlin M, Rodnan G. Linear scleroderma. Clinical spectrum, prognosis, and laboratory abnormalities. Ann Intern Med 1986; 104: 849-57. 5. Su WDP, Person JR. Morphea profunda. A new concept and a histopathologic study of 23 cases. A.m J Dermatopathol 1981 ; 3: 251-60. 6. Su WPD, Greene SL. Bullous morphea profunda. Am J Dermatopathol 1986; 8: 144-7. 7. Morrow PA. A case of symmetrical morphoea attended with the formation of bullae and extensive ulceration. J Cutan Genito-Urin Dis 1896; 14: 4129-27. Case report 158 Acta Dermatoven APA Vol 9, 2000, No 4 Morphea mimicking dermatitis artefacta Case report AUTHORS' ADDRESSES 160 8. Templation HJ. Localized scleroderma with buliae. H Dermatol Syphilol 1941; 43: 360-5. 9. Synkovski DR, Lobitz WC Jr, Provost TI. Bulious scleroderma. Arch Dermatol 1981; 117: 135-7. 10. Pautrier LM. Sclerodermie a evolution rapide, en plaques multiples. Importance