previous tuberculosis infection. The commonest form of cutaneous tuberculosis, and ... infected sebaceous cyst had been made but excision biopsy of a nodule.
Lupus Vulgaris
A Report of 3 Cases
J. Bourke, D. O'Shea, P. Dervan and S. O'Loughlin Department o] Dermatology and Pathology, Mater Misericordiae Hospital, Eceles Street, Dublin 7. Summary
white circular atrophic areas, on her right knee (Fig. la). Diascopy* showed "Apple Jelly Nodules". The lesion was asymptomatic apart from occasional bleeding, physical examination was normal and there was no history of contact with TB. A punch biopsy of the lesion revealed epithelioid granulomas separated by masses of lymphocytes with negative Acid Fast stain. A culture from the biopsy revealed Acid Fast Bacilli (AFB). Routine blood investigations were normal as was MSU. Sputum examinations for AFB were negative. Mantoux (lst Strength) was strongly positive. Chest X-Ray showed calcified granulomas in both lung fields. The skin lesion resolved following a 9 month course of Rifampicin and Isoniazid confirming the histological and microbiological diagnosis of L.V. (Fig. lb). There has been no recurrence over a 6 year follow-up period.
LUPUS Vulgaris
is considered a rarity, its clinical presentation familiar to doctors who practised when tuberculosis was .common. Three patients with this cutaneous form of tuberculosis, who presented at the Dermatology clinic in the past 8 years, are presented. Doctors should be familiar with the clinical features of this condition, as its reappearance may parallel the reported increased incidence of pulmonary tuberculosis in Ireland.
Introduction Lupus Vulgaris (L.V.), a progressive form of cutaneous tuberculosis (TB), occurs in individuals with a moderate to high degree of immunity following previous tuberculosis infection. The commonest form of cutaneous tuberculosis, and once a relatively frequent sight in clinical practice, L.V. has, with the advent of effective anti-tuberculous therapy and subsequent world-wide decline in the incidence of tuberculosis, become a rarity in these islands. We have not found a reported case of L.V. indexed in the Irish Medical Journals over the past 30 years. We report 3 cases referred to the Dermatology Out-patients Department over an 8 year period and discuss their significance.
Case 11 A 66 year old mother of three from Carrick-on-Suir presented with a six year history of an asymptomatic red papulo-squamous facial rash which had started on her nose following trauma from a rusty spike. Inflammation spread to the malar areas with intermittent pustule formation and serous discharge leading to atrophy, scarring and deformity (Fig. 2a). Physical examination was otherwise normal with no history of contact with TB. Punch biopsy of the lesion revealed non-caseating granulomas separated by a lymphoplasmacytic infiltrate with negative staining and culture f o r AFB. Routine blood tests, MSU and Chest X-Ray were normal. Sputa examination for AFB were negative. Mantoux (lst Strength) was strongly positive. The clinical and histological diagnosis of L.V. was confirmed by a dramatic response to a 9 month
Case 1 A 45 year old teaching nun from Navan presented with a scaling 4 x 6 cm red well-demarcated irregular indurated plaque, showing small * Diascopy: Examination of skin lesions or tissue changes through a glass slide pressed against the skin. Cutaneous granulomas manifest as red-brown nodules ("Apple Jelly Nodules"). Address for reprints : Dr. Sean O'Loughlin, Consultant Dermatologist, Mater Misericordiae Hospital, Eccles Street, Dublin 7. Tel. 303685.
Fig. l--Case 1. Scaling irregular plaque on knee (a) before and (b) after treatment.
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Fig. 2--Case 2. Scaling inflammatory plaques on face (a) before and (b) after treatment. course of Rifampicin and Isoniazid (Fig. 2b). There has been no recurrence over a five year follow-up period. Case H I
A 53 year old Arklow woman was referred from the E N T Department with a one year history of an asymptomatic red scaly nodular plaque on her nose. A clinical diagnosis of an infected sebaceous cyst had been made but excision biopsy of a nodule revealed multiple non-caseating granulomas containing AFB which failed to culture out. The patient was otherwise asymptomatic. Physical examination revealed a few mid zone crepitations in both lungs. Chest X-Ray showed marked fibrotic changes in the mid and lower zones of both lung fields, not typical of tuberculosis and unchanged from a chest X-Ray taken four years previously. Routine blood tests and an MSU were normal. Sputa examination for AFB were negative. Mantoux (Ist Strength) was strongly positive. The lesion is responding to a 9 month course of Rifampicin and lsoniazid confirming the diagnosis of L.V.
Discussion Cutaneous tuberculosis has been variously classified over the years by many authors including Dafter and BolgeW. Rook ~ adapts a modified version of the more recent classification of Beyt et at ~ as follows : I. Innoculation Tuberculosis--(exogenous source) Tuberculosis Chancre Verrucosa Cutis *Lupus Vulgafts (some) II. Secondary Tuberculosis (endogenous source) a. Contiguous Spread-Scrofuloderma b. Auto-innoculation-Orificial tuberculosis III. Haematogenous Tuberculosis-Acute Miliary Tuberculosis *Lupus Vulgaris (some). Tuberculosis Gumma IV. Eruptive Tuberculosis (The Tuberculides) I Micropapular-Lichen Scrofulosorum II Papular-Papular/papulonecrotic tuberculides III N o d u l a r ? Nodular Vasculitis (some)
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This classification is useful because of its simplicity. It avoids complex immunological considerations and also the confusion heretofore created by group IV, the Tuberculides. It does have its shortcomings, however, at least in the case cf lupus vulgaris, which we consider fits into each of the four groups outlined. It may arise exogenously by primary innoculation; endogenously by direct extension from underlying tuberculous lymph gland or joint or via lymphatic, spread from mucous membranes of the nose or throat; there is some evidence to suggest that haematogenous spread may be responsib.le for svme cases affecting the nose where the large capacity and unusual architecture of the nasal venules may act as a catchment area with enhanced trapping of bloodborne organisms'. It has also been suggested that organisms disseminated at the height of a previous infection can remain latent in the skin for many years and that local trauma or non-specific inflammation may then stimulate organisms to renewed activity ~. L.V. may even arise in an existing papulonecrotic tuberculide ~. Despite its shortcomings Beyt's classification provides a practical breakdown of cutaneous tuberculosis and is certainly more clear cut than the older classifications of Jadassohn or Bolgert 1. Clinically, L.V. characteristically presents as tiny red brown embedded gelatinous plaques on the head and neck of a patient with a background of tuberculosis which is usually inactive. Diascopy of the lesion reveals a nodular infrastructure - the classical "Apple-Jelly Nodules'". These lesions coalesce into infiltrated dark plaques becoming gyrate or discoid in shape, the ultimate appearance depending greatly on localised inflammatory changes. It is important to note that 11% of patients with L.V. have an active tuberculous focus elsewhere, lymph nodes being the commonest site, and therefore detailed investigations to detect any such focus are mandatory in all cases 6. Rook 2 divides L.V. into 5 clinical grc,ups; plaque, ulcerative, vegetative,
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tumour-like and papulo-nodular forms. The picture varies from a scaling erythematous psoriasiform plaque to a nodular extensively infiltrating tumour destroying mucous membrane and cartilage in its path (lupus vorax); indeed the intensity of local inflammation may virtually obscure the diagnosis. Untreated the course is inexorably progressive resulting in areas of tissue destruction, scarring and recurring inflammation, leading to troublesome contractures and deformity. Histology classically reveals epithelioid granulomas containing Langhans giant cells occurring in sheets of lymphocytes with minimal or absent caseation necrosis. Granulomas are usually situated immediately subepidermally with overlying epidermal atrophy. The picture may vary greatly however, with only scant lymphocytes, epidermal hypertrophy, or fibrosis and telangiectasia (chronic cases) being present. Staining and culture for AFB are rarely positive (6 % of 4,000 cases) 7. This is thought to be due to a combination of organisms of reduced virulence and a moderate to high host immunity resulting in death or incarceration of the bacilli.
clinical suspicion, strongly positive Mantoux, typical histology, rarely the finding of A F B on tissue staining or culture and a satisfactory response to anti-tuberculous therapy of adequate dosage and duration. In atypical cases a successful therapeutic trial with anti-tuberculous drugs may be needed to confirm diagnosis. Because of the wide variation in clinical presentation, the differential diagnosis of L.V. includes many other skin conditions such as psoriasis, rosacea, Bowen's Disease, sarcoidosis, gumma, leprosy and other rarer granulomatous disorders, such as bromoderma. Our patients serve to illustrate a number of important points regarding lupus vulgaris. Its prevalence generally parallels that of acute pulmonary TB ~~ and our cases and two additional cases noted by Dermatologist Colleagues recently in the Irish Republic (personal communication) may reflect the reported increasing of pulmonary TB1L
Chronic untreated lesions result in extreme disfigurement from tissue cartilage destruction. Neoplastic change is rare, usually squamous cell carcinoma (89.3%) and, much less commonly basal cell carcinoma (10.7%) or sarcoma (4.8%) 8 One case of a plasmacytoid lymphoma occurring in L.V. has been reported 9.
Our 3 cases emphasise the preponderence among women of L.V. (F: M, 3 : 1). The nose is involved in one third of patients (cases 2 and 3), the limbs being the next commonest site (case 1)*. Marked inflammation may lead to misdiagnosis (case 3 diagnosed initially as inflammed sebaceous cyst). It is important to be aware that previous local trauma appears to trigger some cases of lupus vulgaris (case 3). We appear to have been fortunate in our yield of AFB - - 2 out of 3 - - as compared to 6% in Horwitz's series of 4,0007. The dramatic response to anti-tuberculosis treatment, in case 2 the ultimate diagnostic indicator, can be seen in the illustrations.
The diagnosis as evident from our cases, depends on a combination of
We report these cases to remind our colleagues of the ever present
Dramatic resolution follows antituberculosis treatment.
problem of TB in its cutaneous presentation as lupus vulgaris.
Acknowledgements To Dr. John Downey, Carrick-on-Suir, Dr. Jack Finn, Navan and Dr. Leitch, Rathdrum who kindly referred the patients; Dr. Patrick Keelan and Dr. Shane O'Neill for advice re management and Professor Mary Leider for Histology of case 3.
References 1. Bolgert, M. La tuberculose cutante. historique clinique et evolution d'therapeutic. Sere. Hop. Paris. 1967: 43, 868-88. 2. Savin, J. A., Wilkinson, D. S. Lupus Vulgaris. In: Rook, A., ed. Textbook of Dermatology, Oxford: Blackwell Scientific Publications. 1968: 809-13. 3. Beyt, B. E., Ortbals, D. W., Santa C r u ~ , ~ J., Kobayashi, G. S., Eisen, A. Z., Medoff, G. Cutaneous Mycobacteriosis: Analysis of 34 cases with a new classification of the Disease. Medicine. 198: 60, 95-109. 4. Kanan, M. W., Ryan, T. J. Endonasal localization of blood borne viable and non-viable particulate matter. Br. J. Derm. 1975: 92, 47578. 5. Uvstedt, J. H., Ostenson, I. W. Cutaneous tuberculosis. Tubercle 1951: 32, 36-40. 6. Morrison, J. G. L., Fourie, E. D. The papulonecrotic tuberculidefrom Arthus reaction to lupus vulgaris. Br. J. Derm. 1974: 91,263-270. 7. Horwitz, O. Lupus vulgaris curls in Denmark. Acta Tuberc. Scand. (Suppl.) 1960: 49, 1-137. 8. Konturkowa, A. Tuberculosis. Przegl. Derrn. 1965: 51,553-555. 9. Harrison, P. V., Marks, J. M. Lupus vulgaris and cutaneous lymphoma~ Clin. and Ex. Derm. 1980: 5, 73-77. 10. Weakley, D. R. Tuberculosis of the skin. In: Demis, D. J., ed. Clinical Dermatology, Philadelphia: Harper and Row. 1984: 16-26. 11. De Buitleir, M., Fitzgerald, M. The changing profile of Tuberculosis in a general teaching hospital--a five year review of 121 cases. Ir. Med. J. 1982: 75, 390-99.
