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CUTANEOUS SARCOIDOSIS by GORDON B 274 Postgrad Med J: first published as 10.1136/pgmj.34.391.274 on 1 May 1958. Downloaded from , II CUTANEOUS SARCOIDOSIS By GORDON B. MITCHELL-HEGGS, M.D., F.R.C.P. and MICHAEL FEIWEL, M.B., Ch.B., M.R.C.P. Department of Dermatology, St. Mary's Hospital, W.2 Sarcoidosis of the skin is often a striking picture for systemic features, a skin biopsy 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, lupus 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 papules 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 fatigue malaise, dyspnoea cough, anywhere body, http://pmj.bmj.com/ weight loss and fever, 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 liver 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 1 .. f. gt ......................2 :: :'5f0:-~ ........ copyright. 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 (lupus erythematosus) 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 lichen planus, an acute ulcerating type and a miliary form. Involvement :- :: :: }........ of the scalp in sarcoidosis may lead to partial or complete alopecia. Erythema 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. Erythema nodosum 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 subcutaneous tissue 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.
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