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274 Postgrad Med J: first published as 10.1136/pgmj.34.391.274 on 1 May 1958. Downloaded from

, II CUTANEOUS By GORDON B. MITCHELL-HEGGS, M.D., F.R.C.P. and MICHAEL FEIWEL, M.B., Ch.B., M.R.C.P. Department of , St. Mary's Hospital, W.2

Sarcoidosis of the is often a striking picture for systemic features, a skin is again an easy and led to its recognition as a disease entity. For means of establishing the diagnosis. the patient, its importance lies in disfigurement In either case, the clinician is helped if he carries more than in disability. For the clinician, it may in his mind's eye the varying aspects of cutaneous provide a ready means of diagnosis towards which sarcoidosis. At the same time, conditions re- one glance may give a clue. In addition, the skin sembling sarcoidosis of the skin must be differ- has played an important role in the study of entiated. This is not easy because the eye needs aetiology. The reactions to injected tuberculin, practice and neither description nor photograph the response to B.C.G. inoculation, and to Kveim can adequately convey the subtleties of the make- antigen are some of the ways in which the skin has up of a skin lesion on which a diagnosis rests. been tested in sarcoidosis. Clinical Manifestations Sarcoidosis The picture of the skin is a varied one and classi- The aetiology is not definitely established. The fication based on the early descriptions is into four disorder involves the reticulo-endothelial system types: Boeck's sarcoid, subcutaneous sarcoid ofcopyright. and many organs, including the lungs, the lymph Darier-Roussy, pernio of Besnier and nodes, the bones, the heart, the uveal tract, the erythrodermic sarcoid. Pautrier's monograph skin and so on may be affected, although it is (1940) gives a full account. generally confined to a few situations. Now Boeck's sarcoid: This is the most common recognized more commonly, the onset is mainly type. Sharply defined or nodules may between the tenth and fiftieth year. Sarcoidosis. occur symmetrically, over face, shoulders and may be heralded by prodromal symptoms of extensor surfaces of arms mainly, but may be and and anorexia, found on the even on the mucous , dyspnoea cough, anywhere body, http://pmj.bmj.com/ and , any one of which may be the membranes. Ulceration is practically unknown. dominant feature of the illness. Sometimes a (i) Papules. They usually erupt rather sud- serious symptom marks the onset. The course of denly, and are well-defined, firm, pinkish-red or the disease is then erratic, many relapses and re- reddish-brown. They are smooth or scaly, missions may occur, and it is difficult and some- sparsely distributed or in clusters, and slightly times impossible to judge its natural course and infiltrated. Their evolution may extend over to assess its response to treatment. several years with remissions and relapses, but The process of sarcoidosis is essentially benign, finally the lesions flatten, leaving faint pigmented on October 1, 2021 by guest. Protected but may nevertheless bring about death by inter- or erythematous areas. ference with the function of the heart, the brain, (ii) Nodules. These are fewer in number but the or kidney, or through some other larger, and red, reddish-brown, or mauve in complication. colour. They are smooth, firm, infiltrated and moveable over the underlying structures. Dia- Cutaneous Aspects of Sarcoidosis scopy shows the resemblance of the small yellow- Diagnosis grey foci that make up the lesions, to the apple Skin manifestations will help in two ways. The jelly nodules of lupus vulgaris. Early nodules clinician, finding a disease picture suggestive of look bright red, then as they grow larger, they be- sarcoidosis, will search for dermatological features come less vivid, and when fully evolved, they may and biopsy one to give the characteristic histology. remain static for years. Then, as the centre slowly Even where none will be found at the time, they flattens, the colour darkens leaving finally pig- may appear later. Or the patient may present skin mented areas crossed by telangiectases. Whitish lesions making sarcoidosis likely. After searching scarring with slight infiltration may also result. May I958 MITCHELL-HEGGS and FEIWEL: Cutaneous Sarcoidosis 275 Postgrad Med J: first published as 10.1136/pgmj.34.391.274 on 1 May 1958. Downloaded from

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FIG. I.-Boeck's sarcoid. Reddish-brown hue; infiltrated, clearly defined, smooth lesion, without telantiectasia.

(iii) Plaques. These show a tendency to central swellings result both from bone changes and http://pmj.bmj.com/ clearing, so that annular and circinate figures cutaneous infiltration. The skin of lupus pernio form, with telihgiectatic, raised edges. is thick and indurated, with a network of telan- Subcutaneous sarcoid, Darier and Roussy: Round giectases. Mutilating varieties have been re- or oval nodules are deeply seated, and may be skin ported. coloured, blue or purplish. They vary in size, Angio-lupoid, Brocq-Pautrier: A well-defined, usually from i cm. to 4cm. in diameter. They indurated, solitary plaque or nodule, occurring on the trunk and the (here re- on or around the nose, and develop slowly legs affecting mainly on October 1, 2021 by guest. Protected sembling acrocyanosis) without subjective symp- middle-aged women, and round, cherry-sized and toms, and rarely exceed twenty in number. This purplish in colour, with yellowish patches and type is not common and may occur with Boeck's obvious telangiectases. The yellow colour pre- Earcoid. dominates under 'diascopy. Evolution is very Lupus Pernio, Besnier. The smooth, shiny, slow, with little tendency to spontaneous re- purple nodules oflupus pernio can be distinguished gression. from chilblain lupus () by the Erythrodermic Sarcoid. An uncommon form absence of pain and their persistence during the (reviewed by Wigley and Musso, 1951). Some- summer. In lupus pernio, systemic sarcoidosis is times associated with other types of cutaneous frequently concurrent. The eruption is sym- sarcoidosis, erythrodermic areas may be single, but metrical, occurring mainly on the ears, cheeks, are more often multiple. Found anywhere in- forehead, nose, dorsa of the hands, fingers and cluding the scalp, they are of differing shades of toes. The nose, most commonly affected, may red and on diascopy show no yellow miliary lupoid have a disfiguring bulbous look. Fingers and foci but diffuse yellow staining. Of various loes may be the site of osteitis cystica, so that shapes and sizes, they may enclose normal skin 276 POSTGRADUATE MEDICAL JOURNAL May I958 Postgrad Med J: first published as 10.1136/pgmj.34.391.274 on 1 May 1958. Downloaded from and have clear-cut or ill-defined edges. Infiltra- tion is absent, slight or moderate. Scales, if present, are fine and transparent or coarse and adherent but readily removed. Often of rapid onset, the eruption lasts a few months to a few years or longer (even permanently), with remis- ...... :...... sions and relapses at times. When it subsides it leaves no trace, or else slight pigmentation. Rare forms. These include a lichenoid type with a resemblance to , an acute ulcerating type and a miliary form. Involvement :- :: :: }...... of the scalp in sarcoidosis may lead to partial or complete alopecia. Nodosum. One of the most interesting recent advances in our knowledge of sarcoidosis i~;·4 is that nodosum be one of its erythema may early ....^ - . . ~ X'' ·. manifestations. It emphasizes that sarcoidosis ^ may, along with other processes, produce a non- ..:..:. ,. <..:~.. specific picture. When erythema nodosum is part of a syndrome and a negative Mantoux re- action and bilateral symmetrical hilar adenopathy accompany, sarcoidosis becomes the likely cause. is clinically distinctive. It appears to be an allergic reaction and antigens are of virus, bacterial, fungal or drug origin. (The unknown cause of sarcoidosis, possibly having its source of in the to hilar entry lungs produce gland copyright. enlargement, would be one of these antigens.) Clinical Features. The swellings may be ac- companied by fever and joint pains which settle as the subside. The raised nodules arise FIG. 2.-Sudden eruption of fine papular sarcoid, swellings covering almost the whole skin surface within 14 mostly on the front of the shins, but also on the days. Iritis and radiological lung changes present. arms, trunk or face. They vary from about i cm. (By courtesy of Miss M. Savory, photograph by Miss to about 5 cm. or more in diameter, are pink or red E. Mason, St. James' Hospital). at first, then become more livid showing the colour changes of a bruise and leaving a little of a granuloma. When this is examined histo- http://pmj.bmj.com/ residual staining. They are smooth, soft, roundish logically, the picture of sarcoidosis may be seen. and tender on pressure. Additional crops can However, with the polarising microscope, doubly occur but the trouble is over in a few weeks or a refractile silica particles can be found within the month or two. There is no breaking down. It is sarcoid-like tissue. Two possibilities exist. Silica probable that the initial change is vascular, for may excite a tuberculoid- or sarcoid-like granuloma in the histology the larger veins in the upper part after a variable period, or the patient may have of the show endothelial pro- sarcoidosis localizing in a scar. In practice, both liferation and the vessel wall infiltrated and sur- occur. The granuloma may arise only where on October 1, 2021 by guest. Protected rounded by the inflammatory cells. Later foci silica lies in the skin, but if the lesions are found of epithelioid cells appear with giant cells, usually elsewhere, sarcoidosis becomes likely. Tattoos of the foreign body type inside or outside these likewise may become raised and infiltrated. foci, and there is a resemblance to . Again there are two possibilities. The patient Scars, Pseudotuberculoma Silicoticum, Tattoos. may have developed an allergy to the mercury, The occurence of sarcoidosis in abnormal skin is chromium or other constituent of the pigment, not unusual and may be early. Infiltration of possibly by further contact. Alternatively, the scars by sarcoid tissue may only produce a change tattoo has become the site of sarcoidosis (or of colour. They become reddish-brown or livid, , , tuberculosis or even lupus with no palpable infiltration, or raised and even erythematosus). keloidal. A biopsy at this stage is typical of sarcoidosis. Scars containing particles of silica, Histology engrained at the time of , may, many years While macroscopically pleomorphic, the micro- later, become raised and firm with the formation scopic appearance in the skin is remarkably uni- MITCHELL-HEGGS and FEIWEL: Postgrad Med J: first published as 10.1136/pgmj.34.391.274 on 1 May 1958. Downloaded from Ilay I958 Cutaneous Sarcoidosis 277

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FIG. 4.-Nodular sarcoid in clusters. (Photographs I, 3 and 4 by Dr. P. Cardezc, St. Mary's Hospital) form, as in other organs. A granuloma of tuber- in the dermis in Boeck's sarcoid, and in the sub- culoid structure is composed mainly of circum- cutaneous tissue in Darier-Roussy sarcoid. Lupus scribed islands of epithelioid cells, and is situated pernio shows dilated capillaries in the upper part 278 POSTGRADUATE MEDICAL JOURNAL May 1958 Postgrad Med J: first published as 10.1136/pgmj.34.391.274 on 1 May 1958. Downloaded from

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F ss - o s .. .; ...... ii~i~:iii'iiiiii ::::R e::- -; '-l--- FIG. 6.-Lupus pernio, purple sausage-shaped fingers. -t:£C ..:. .::: .::.....:::: ...... *...! l;; (Photographs 5 and 6 by courtesy of Dr. J. Ingram.) establish the diagnosis. The lepromin test is FIG. 5.-Irregular, discrete plaques of sarcoid over ususally positive in tuberculoid leprosy. Zirconium trunk. granuloma. Silicon granuloma has already been of the dermis, and in erythrodermic sarcoid, the discussed in this article and that caused by foci of epithelioid cells are small and histiocytes berylliosis in this symposium (p. ). A sarcoid- and lymphocytes surround superficial vessels. like eruption in the axilla following the use of details the in the skin as anti-perspirant sticks containing zirconium has Lever (I954) histology been described (Rubin et al., I956; Saunders, follows. In active lesions of sarcoidosis, the 1957; Shelley, I957). islands of epithelioid cells contain few Langhans Clinical Differences: giant cells or none at all. A slight and occasionally Similarities, Histological copyright. moderate admixture of lymphocytes is present, Syphilis. A nodular syphilide is serpiginous as a particularly at the margins of the epithelioid cell rule. Gummatous ulceration and scarring tend to islands. Caseation necrosis is almost always occur. On section, the granulation tissue shows absent. Rarely, some necrosis is found at the many plasma cells, vascular changes are conspicu- centre of the islands. A reticular network sur- ous and endarteritis is found. agminata rounds almost every epithelioid cell in most areas, (Lupus miliaris disseminatum faciei).- Small ele- but in others the fibres are concentrated at the vated papules on the face, especially the eyelids, periphery. In the healing phase, increasing show central caseation surrounded by tubercles fibrosis, usually starting at the periphery of the under the microscope. Lupus erythematosus. http://pmj.bmj.com/ islands, obliterates the epithelioid cells and the Found on the nose, cheeks, ears, backs of fingers lymphocytes. Reticular fibres transform into and the hands, one variety of lupus erythematosus collagen fibres. A moderate number of giant (chilblain lupus of Hutchinson) may resemble cells, more of the foreign body type than Langhans lupus pernio. The changes in the , cells, may occasionally contain so-called Schau- however, of follicular plugging and scaling, are mann inclusion bodies and asteroid inclusion reflected in the histology. . but neither is for sarcoidosis. Small, firm, skin- or red-coloured papules or bodies, specific nodules arranged in rings are found and resemble on October 1, 2021 by guest. Protected Differential Diagnosis a papular sarcoid. The characteristic histology The diagnosis of cutaneous sarcoidosis can be shows an area of degenerated collagen surrounded confirmed by systemic involvement, by an absent by a ring of histiocytes, fibroblasts and lympho- or weak tuberculin reaction, possibly by a positive cytes. Erythema elevatum diutinum, with red to Kveim test, as well as through its typical histology. purple nodules, especially about the joints on the Many conditions remain to be differentiated. dorsa of the hands, the wrists and the elbows, may Those with Clinical and Histological Similarities: be a variant of granuloma annulare. The reticuloses Lupus vulgaris. A patch of lupus, commonest on (). Red or purple papules or nodules the face, often starts in childhood and extends are not infrequent, whether the disorder is gradually. Tuberculoid leprosy. Annular lesions leukaemic or not. Microscopically, a dense cel- resemble annular sarcoid. Finding thickened, lular infiltrate of the predominating cell type fills palpable nerves, areas of anaesthesia, and, in the the dermis and extends deeply. Lymphocytoma section, neural elements involved by tuberculoid cutis, the facial eosinophilic granuloma, Kaposi's infiltration and an occasional lepra bacillus help to idiopathic haemorrhagic sarcoma, dis- May 1958 MITCHELL-HEGGS and FEIWEL: Cutaneous Sarcoidosis 279 Postgrad Med J: first published as 10.1136/pgmj.34.391.274 on 1 May 1958. Downloaded from seminatum, and urticaria pigmentosa. Conditions, related to the reticuloses which might occasionally give rise to difficulty. (dia- beticorum vel non-diabeticorum), and granuloma disciformis chronica et progressiva. Sharply de- marcated glazed patches, depressed and yellowish HB"ir.: ii:"·." ...8: .i :Z ·:""::l::·i. J·'·:'·;:r· in the centre and violaceous at the ·· "'' periphery, i. found on the are like an annular ··*·· *··· usually legs, :1 :.: ,·· .,-,.··.:·,·:·.?. R P&.i-r,.ii ·.I:JI.,I sarcoid. On section, necrobiotic areas of collagen i·;ili;':' t:gl·: .8·.i:.'·.:a are seen. ·r·i surrounded inflammatory cells .i.l* a ·.·.. by cJ: Clinical Differences, Histological Similarities: .:···-: : i.:: ';'' Cutaneous leishmaniasis. Although the :' f:i"" relapsing ··.i·.: miscroscope shows a tuberculoid picture and no .;i:h·.'·. Leishman-Donovan bodies, the history and the ':I localization on exposed parts, usually the face, should establish the diagnosis. with chronic lymphoedema (rosacea acuminatum), rosa- ceous tuberculide (Lewandowsky), and cheilitis i'· and the Melkersson-Rosental triad "iii.i- ..':r granulomatosa :·I...'*i ·:. facial palsy, thick lips, and lingua :·:;·.;···: --1.·- (relapsing "::. plicata). It is only the histology that suggests ':P sarcoid. Other Causes of Erythema Nodosum. Tuber- FIG. 7.-Annular sarcoid over left temple, confirmed by culosis, streptococcal , drugs such as histology. (Photograph by Mr. R. Lunnon. By sulphathiazole, as well as sarcoidosis, have been courtesy of Dr. R. M. B. MacKenna and Dr. F. R. mentioned (this symposium, p. 24z). With in- Bettley, Dean of the Institute ofDermatology.) creasing travel of all peoples, further causes of erythema nodosum require mention. Lympho- plaques are deeply situated and ulceration is com- copyright. granuloma inguinale. A virus disease which begins mon. More on the backs of the calves and lower as a small in the genital or perineal region third of the legs than on the upper part of the and is followed by enlargement, sometimes painful, shins, they occur usually in young women who of the regional lymphatic glands. Fever and have fat, cold legs and who may have had active erythema nodosum may accompany. Leprosy. tuberculosis in the past, especially glandular Erythema nodosum is one form of lepra reaction. tuberculosis. (Whitfield's It occurs during treatment by sulphones when type), nodular . This is a non-tuberculous are and at the same more chronic than nodosum. lepra bacilli multiplying time, condition, erythema http://pmj.bmj.com/ are being rapidly destroyed by the drug. Deep Females in an older age group than those with fungus . In coccidiomycosis, cutaneous Bazin's disease have painful nodules, usually on manifestations are usually late (coccidial granu- the legs. The cause is not known, and it is un- loma). Erythema nodosum may occur in an early common. Erythema perino and erythrocyanosis. stage of the disease (San Joaquin Valley disease). Young females with chilblain circulation may suffer Superficial fungus infections. When these are from nodular vascular inflammatory changes at pustular, generalized allergic reactions are not the backs of the calves, which may persist in the uncommon (trichophytids). Rarely a trichophytid warmer weather. This form of chilblain, some- on October 1, 2021 by guest. Protected is an erythema nodosum, especially in cases of times occurring in the absence of toe and finger pustular ringworm of the scalp (kerion). involvement, may also affect the cold leg of past Conditions Resembling Erythema Nodosum. If poliomyelitis. Chronic meningococcal septicaemia. correctly diagnosed, they will exclude sarcoidosis. In the epidemic of cerebrospinal fever during , toxic erythema. The eruption World War II, cases of chronic septicaemia were is more widespread and more profuse, and noted. There was fever, headache, pains in the mucous membranes may be involved. Macules, joints and muscles, and an eruption of tender papules or bullae make up the picture rather than nodules, though perhaps more multiforn and nodules. Variations in colour (iris or target profuse than in erythema nodosum, sometimes lesions) may be seen. Viruses, bacteria, fungi and with purpura. Sporadic cases continue. Thrombo- drugs may produce it, but not tuberculosis or phlebitis. Fever and malaise accompany the sarcoidosis, and it may also occur without obvious painful nodules which are felt rather than seen cause (and can be recurrent). Erythema induratunr' along the course of the veins. Distribution tends, (Bazin's disease). Chronic purple nodules and therefore, to be correspondingly linear. Nodules in 280 POSTGRADUATE MEDICAL JOURNAL May I958 Postgrad Med J: first published as 10.1136/pgmj.34.391.274 on 1 May 1958. Downloaded from

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.:.~ *.·: FIG. 8.-Section I (x 25). The cutis exhibits numerous in- nodules separated by connective tissues. The copyright. dividual nodules are composed of closely-set epithelioid cells and only a few round cells. The surrounding collagen looks normal. FIG. 9.-Section 2 (x 90). Detail of Section i. (Sections i and 2 by courtesy of Dr. H. Haber, Institute of lupuis erythematosus and polyarteritis nodosa. Sub- Dermatology.) acute systemic lupus erythematosus may show a Later the lesions fibrose and the skin becomes nodular eruption on the legs owing to inflammatory or the mass and involvement ofthe vasculature. Without depressed, may liquefy discharge. superficial http://pmj.bmj.com/ a typical and with a negative L.E. cell phenom- Treatment enon, the diagnosis may be difficult, but the A decision on the use of steroids will depend on prolonged course and the polysymptomatic nature the overall picture of sarcoidosis. The degree to will arouse suspicion. In polyarteritis nodosa, which oral cortisone helps in healing the cutaneous the nodules are usually deeply situated and with manifestations is difficult to assess. If lesions are overlying skin of normal colour. Macules, papules, few and disfiguring, local injections of hydro- ulcers or livido racemosa ac- cortisone can be tried, but results are still un- petechiae, may certain. Calciferol in company the nodules. Nodular allergide of heavy dosage was given on October 1, 2021 by guest. Protected Gougerot (Maladie trisymptomatique). This has following its success in lupus vulgaris, but toxic featured in the dermatological literature recently. sequelae were frequent. As some cases of There are purple nodules on the legs, but also sarcoidosis have hypercalcaemia which may pro- macules, papules, purpura and ulcers. The duce renal failure, calciferol would not seem a condition is chronic and some cases end fatally. suitable drug in the treatment of sarcoidosis. It may well be one presentation of the better Happily, one can look forward to spontaneous recognized anaphylactoid purpura, polyarteritis. resolution in many cases. nodosa or lupus erythematosus. In some cases, REFERENCES have been found. PALTRIER, L. M. (1940), Maladie de Besnier-Boeck-Schaumann, cryoglobulins Relapsing febrile Masson et Cie., Paris. nodular (Weber- WN'IGLEY, J. E. MI., and MUSSO, L. A. (1951), Brit. J. Derrm., nonsuppurative 63, 398. Christian disease). Nodules and plaques appear LEVER, XV. F. (I954), Histopathology of the Skin,' 2nd edition, in in the subcutaneous fat without a J. B. Lippincott & Co., Philadelphia, London, Montreal, p. 188. crops parti- RUBIN, L., et al. (1956), J.A.M.A., I62, 953. cular for the lower limbs. Nodules SAUNDERS, T. S. (1957), A.M.A. Arch. Derni., 76, 619. predilection SHELLEY, XV. B. (I957, in press), Transactions of the St. John's are hard, and overlying skin is normal but red. Hosp. Derm. Soc.'