Letters to the Editor

Histopathology of the lesions of all our cases showed thickened, sclerotic collagen bundles in the deep reticular dermis and mucopolysaccharide in the dermis by Alcian blue staining. After excluding autoimmune diseases and scleredema,5 the diagnose of SSS was made. The first case also had keratosis pilaris, and the third case had herpes zoster. Our series of cases highlight some unusual phenotypes of SSS. First, skin induration and hypertrichosis developed simultaneously on the outer side of bilateral thighs in case 1. Second, hernial sac-like changes concomitant with herpes zoster that occurred in case 3 were previously reported only in a Chinese patient.6 Finally, case 4 had slightly lower levels of IgA and IgG than normal, which has not been previously reported in the literature. Our series of cases not only highlight the heterogeneous nature of SSS but provide some clues of a possible association between SSS and immunologic problems. X. Deng, F. Chen, Z. Song, H. Yan, Y. You, B. Zhong, X. Yang, F. Hao* Department of Dermatology, Southwest Hospital, Third Military Medical University, Chongqing, China *Correspondence: F. Hao. E-mail: [email protected]



hypopigmented area. Two years prior to the appearance of the mass, a similar but flatter skin lesion appeared in the same area and persisted for 8 months. Medical advice was not sought for that lesion. Herbal remedies were applied to the lesion for several months causing the lesion to heal, leaving a hypopigmented area. Systemic review and past medical/surgical histories were unremarkable. Skin examination revealed a fleshy to brownish firm plaque measuring 4 9 4 cm with central ulceration on her right thigh at the periphery of an old postinflammatory hypopigmented patch (Fig. 1). No lymphadenopathy or organomegaly. A skin punch biopsy (4 mm) showed a poorly circumscribed, deeply infiltrative, asymmetric tumour composed of squamoid or basaloid nests, infiltrating cords and ductular structures set in desmoplastic stroma (Fig. 2). The immunohistochemical analysis showed positivity for CEA, CK7, EGFR and S100. The BerEP4 and PHLDA1stains were negative. The diagnosis of microcystic adnexal carcinoma was made. Complete blood count with differentials, ESR, liver enzymes, urea, creatinine, electrolytes, CT scan of the chest, abdomen and pelvis were all normal. A surgeon carried out complete surgical excision of the lesion with wider margins. Radiotherapy was considered but patient refused. The patient did not pursue further follow-up. Microcystic adnexal carcinoma (MAC) is a very rare cancer of the skin that demonstrates follicular and eccrine differentiation.

1 Jablonska S, Blaszczyk M. Stiff skin syndrome is highly heterogeneous, and congenital fascial dystrophy is its distinct subset. Pediatr Dermatol 2004; 21: 508–510. 2 Liu T, McCalmont TH, Frieden IJ, Williams ML, Connolly MK, Gilliam AE. The stiff skin syndrome: case series, differential diagnosis of the stiff skin phenotype, and review of the literature. Arch Dermatol 2008; 144: 1351–1359. 3 Morrell DS, Challgren E, Nijhawan A et al. Two cases for diagnosis: asymmetric childhood scleredema or stiff skin syndrome? Pediatr Dermatol 2003; 20: 350–355. 4 Gilaberte Y, Saenz-de-Santamaria MC, Garcia-Latasa FJ, Gonzalez-Mediero I, Zambrano A. Stiff skin syndrome: a case report and review of the literature. Dermatology 1995; 190: 148–151. 5 Azevedo VF, Serafini SZ, Werner B, Muller CS, Franchini CF, Morais RL. Stiff skin syndrome versus scleroderma: a report of two cases. Clin Rheumatol 2009; 28: 1107–1111. 6 Xu J, Ma DL, Fang K. A case of stiff skin syndrome with local hernial sac-like changes. J Clin Dermato. 2010; 39: 343–344. DOI: 10.1111/jdv.12657

Microcystic adnexal carcinoma: unusual presentation Editor A 60-year-old otherwise healthy female presented with 1 year history of an asymptomatic slowly progressing skin mass on her right thigh that appeared at the periphery of an old

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Figure 1 Fleshy firm plaque with central ulceration at the periphery of an old postinflammatory hypopigmented scar on the right thigh.

© 2014 European Academy of Dermatology and Venereology

Letters to the Editor






Figure 2 H&E (magnification 9100) showing islands and strands of squamoid and basaloid epithelium with variable ductal differentiation (a), multiple nests and cords of cells in dense desmoplastic stroma (9100) (b). Immunohistochemical stain showing positive CK7 (95) (c) and positive CEA (95) (d).

It was first described by Goldstein et al. in 1982.1 MAC is primarily a locally aggressive tumour. Metastasis to the lymph nodes or other sites remains uncommon.2 Most lesions manifest in the 5th–7th decade of life. The classic clinical presentation of MAC is an indolent, indurated, skin-coloured, solitary nodule developing on the face for 3–5 years.3,4 Our case is unusual in the following: location on lower extremity, size is larger than 2 cm in diameter and duration of the lesion is less than 3 years. Like our case, MAC shows a slight female predominance.4 A case of a MAC on the lower extremity, similar to ours, was reported in a 60-year-old male. It was measuring less than 2 cm in size and of 30 years duration.4 Other pathological findings of MAC like small keratin-filled cysts in the upper dermis, and perineural invasion are lacking in our patient.5–7 Morpheaform basal cell carcinoma (MBCC) and desmoplastic trichoepithelioma (DTE) have similar histopathological features of MAC. Immunohistochemical stains can clearly differentiate between them. Positive CK7 and CEA and negative BerEP4 and PHLDA1 support the diagnosis of MAC over BCC and DTE.5,7 Positive BerEP4 is seen in MBCC and DTE. Positive PHLDA1 is seen in DTE and MAC.8 The appearance of MAC on top of an old postinflammatory hypopigmented scar in our patient creates uncertainty as to whether it is a MAC that remitted by herbal therapy and then recurred again, or it is a development of MAC on top of an old scar of unknown primary lesion, or a precancerous lesion that appeared to heal after application of an herbal remedy. There has been a unique case report of occurrence of MAC within a teratoma.4 Surgery that results in complete removal of the tumour is the treatment of choice for locally invasive MAC.3 The surgical

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techniques most commonly utilized for MAC include Mohs surgery and conventional surgical excision with significantly wider margins.3 Recurrence after conventional surgical excision is very high with a rate as high as 60%. Although radiotherapy was reported to induce MAC, it can be used as a treatment for MAC as a monotherapy especially for facial MAC when cosmetic outcome is considered. It can also be used as adjuvant therapy after surgical excision in patients with high risk of local recurrence.9 Given that this tumour demonstrates aggressive local invasion and that Mohs micrographic surgery was not used for the treatment of our patient, radiotherapy as adjuvant therapy would be a good choice but our patient refused to begin various types of treatment. A. Jfri,1 K. Al Hawsawi,2,* S. Marghalani,3 A. Alkhuzaie4 1

Dermatology Department, King Saud bin Abdulaziz University for Health Sciences, Jeddah, 2Dermatology Department, King Abdul Aziz Hospital, Makkah, 3Dermatology Department, King Abdulaziz Medical City for National Guard, Jeddah, 4General Surgery Department, King Abdul Aziz Hospital, Makkah, Saudi Arabia *Correspondence: K. Al Hawsawi. E-mail: [email protected]

References 1 Goldstein DJ, Barr RJ, Santa Cruz DJ. Microcystic adnexal carcinoma: a distinct clinicopathologic entity. Cancer 1982; 50: 566–572. 2 Pugh TJ, Lee NY, Pacheco T, Raben D. Microcystic adnexal carcinoma of the face treated with radiation therapy: a case report and review of the literature. Head Neck 2012; 34: 1045–1050. 3 Yu JB, Blitzblau RC, Patel SC, Decker RH, Wilson LD. Surveillance, Epidemiology, and End Results (SEER) database analysis of microcystic adnexal carcinoma (sclerosing sweat duct carcinoma) of the skin. Am J Clin Oncol 2010; 33: 125–127.

© 2014 European Academy of Dermatology and Venereology

Letters to the Editor

4 Hansen T, Kingsley M, Mallatt BD, Krishnan R. Extrafacial microcystic adnexal carcinoma: case report and review of the literature. Dermatol Surg 2009; 35: 1835–1839. 5 Wick MR, Cooper PH, Swanson PE, Kaye VN, Sun TT. Microcystic adnexal carcinoma. An immunohistochemical comparison with other cutaneous appendage tumors. Arch Dermatol 1990; 126: 189–194. 6 Cooper PH, Mills SE. Microcystic adnexal carcinoma. J Am Acad Dermatol 1984; 10(5 Pt 2): 908–914. 7 Diamantis SA, Marks VJ. Mohs micrographic surgery in the treatment of microcystic adnexal carcinoma. Dermatol Clin 2011; 29: 185–190. 8 Sellheyer K, Nelson P, Kutzner H, Patel RM. The immunohistochemical differential diagnosis of microcystic adnexal carcinoma, desmoplastictrichoepithelioma and morpheaform basal cell carcinoma using BerEP4 and stem cell markers. J Cutan Pathol 2013; 40: 363–370. 9 Schwarze HP, Loche F, Lamant L, Kuchta J, Bazex J. Microcystic adnexal carcinoma induced by multiple radiation therapy. Int J Dermatol 2000; 39: 369–372. DOI: 10.1111/jdv.12659

Dante and dermatological diseases: a literary description of scabies in the Middle Ages Editor, The year 2015 marks the 750th anniversary from the birth of the Italian poet Dante Alighieri (1265–1321), universally considered as one of the most important medieval writers. The ‘Divine Comedy’, his masterpiece written between 1308 and 1321, is an epic poem divided into three parts – ‘Inferno’ (Hell), ‘Purgatorio’ (Purgatory) and ‘Paradiso’ (Heaven) – and represents an imaginative and allegorical vision of the after-life. It is less known that, even if Dante never practiced medicine, he studied its basic principles; the Italian poet also had to enrol in the guild of physicians and apothecaries (‘ars medicorum et spetiariorum’) to take part in the public and political life of his city, Florence.1 Thus, it should come as no surprise that descriptions of some medical problems (e.g. epileptic seizures, eye problems, musculoskeletal disorders, hepatic and cardiovascular diseases) may be found in Dante’s literary works.1,2 These descriptions may provide with us information on state of knowledge of some pathologies during Middle Ages, including dermatological diseases. In particular, during his imaginative journey in the eighth circle of Hell (also named as ‘Malebolge’), Dante sees various sorts of falsifiers (alchemists, counterfeiters, perjurers and impostors). As these sinners were considered as a ‘disease’ in the medieval society, in Hell they are condemned to be plagued by horrible and disfiguring diseases, including scabies. The Italian poet describes the affliction and torment of the damned souls, suffering from this itching infection: ‘I saw two sitting leaned against each other, / as leans in heating platter against platter, / from head to foot bespotted o’er with

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scabs; [. . .] / everyone was plying fast the bite / of nails upon himself, for the great rage / of itching which no other succour had. / and the nails downward with them dragged the scab (scabbia), / in fashion as a knife the scales of bream / or any other fish that has them largest’ (Inferno, XXIX, 73–84).3 So, the bodies of these sinners are shown as place atop one another, whereas they continue to crawl on the ground across one another scratching their scabs. Even if it appears to be poetically emphasized, the itch that torments the souls of falsifiers in Dante testifies that scabies and its symptoms were well known by medieval medicine, several centuries before the classical descriptions by Guido Giudi (ca. 1500–1559), Gerolamo Mercuriale (1530–1606) and Giovanni Cosimo Bonomo (1663– 1696).4,5 Its itchiness was considered so irritating and bothering that scabies was even included by the Italian poet among hellish punishments. Furthermore, the reference to scabies in a sopopular literary work may also suggest its high prevalence in medieval period, when low hygienic conditions allowed its spread.5 Finally, it is necessary to recall that some Dante’s commentators as Nardi (1944) postulated that the diseases could be identified as a kind of leprosy, called ‘serpentine’ and first described by Bartholomeus Anglicus (ca. 1203–1272). He suggested that the choice of this disease as the punishment for alchemists was derived from their way of considering some metals as ‘diseases of gold’ and lead, in particular, as the ‘leprosy of gold’.6 Nevertheless, the use of vernacular word ‘scabbia’ in Dante’s poem confirms that this term – that derives from the Latin verb ‘scabere’ (to scratch, to scrape) – was commonly employed in medieval language to indicate a skin infection. In conclusion, even if we do not have historical evidence on diffusion of scabies and other skin disorders in Middle Ages, modern historian of dermatology should not underestimate the value of information that could be achieved by nonmedical texts,7,8 including the work of the Italian poet Dante. M.A. Riva,* S. Anchise, A. Rotasperti, G. Deho History of Medicine, University of Milano Bicocca, Monza, Italy *Correspondence: M.A. Riva. E-mail: [email protected]

References 1 Riva MA, Arpa C, Gioco M. Dante and asthenopia: a modern visual problem described during the Middle Ages. Eye (Lond) 2014; 28: 498. 2 Mattioli M. Dante e la medicina. Edizioni Scientifiche Italiane, Napoli, Italy, 1965: 164–165. 3 Alighieri D. The Inferno. Translated and with notes by Henry Wadsworth Longfellow. Houghton Mifflin, New York, 1886. 4 Montesu MA, Cottoni F. G.C. Bonomo and D. Cestoni. Discoverers of the parasitic origin of scabies. Am J Dermatopathol 1991; 13: 425–427. 5 Bellini A. Storia della dermatologia e della venereo-sifilologia in Italia. A. Cordani, Milan, Italy, 1934. 6 Durling RN, ed. The Divine Comedy of Dante Alighieri. Volume 1. Inferno. Oxford University Press, New York-Oxford, 1996. 7 Riva MA, Sironi VA, Fano D, Cesana G. Workers’ health conditions in the Greco-Roman world: the contribution of non-medical sources. Arch Environ Occup Health 2011; 66: 54–55.

© 2014 European Academy of Dermatology and Venereology

Microcystic adnexal carcinoma: unusual presentation.

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