|Year : 2015 | Volume
| Issue : 7 | Page : 40-42
Spina ventosa with lupus vulgaris and lymphadenopathy: Multifocal tuberculosis
Sanjeev Sharma1, Shikha Sood1, Mudita Gupta2
1 Department of Radiodiagnosis, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
2 Department of Dermatology, Deen Dayal Upadhaya Zonal Hospital, Shimla, Himachal Pradesh, India
|Date of Web Publication||4-Dec-2015|
Department of Dermatology, Deen Dayal Upadhaya Zonal Hospital, Shimla, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Tuberculous dactylitis is a rare yet well-recognized disease of small bones of the hands and feet. It occurs in young children below five years of age. Tubercular dactylitis with lupus vulgaris and lymphadenopathy was suspected clinically and radiologically in an 8-year-old girl who had multiple soft tissue swelling of hands and feet with ulceration, encrustations, and an atrophic scar with lytic expansile lesions of the small bones of the hands and feet. Tubercular lymph node involvement was confirmed histopathologically.
Keywords: Lupus vulgaris, lymphadenopathy, tuberculous dactylitis
|How to cite this article:|
Sharma S, Sood S, Gupta M. Spina ventosa with lupus vulgaris and lymphadenopathy: Multifocal tuberculosis. Indian Dermatol Online J 2015;6, Suppl S1:40-2
|How to cite this URL:|
Sharma S, Sood S, Gupta M. Spina ventosa with lupus vulgaris and lymphadenopathy: Multifocal tuberculosis. Indian Dermatol Online J [serial online] 2015 [cited 2020 Jul 11];6, Suppl S1:40-2. Available from: http://www.idoj.in/text.asp?2015/6/7/40/171042
| Introduction|| |
Tubercular dactylitis (TD) is a tubercular infection of the short tubular bones of the hands and feet. Cystic expansion leads to formation of spina ventosa (spina—spine-like projections; ventosa—puffed, full of air). This form of osteoarthritic tuberculosis is extremely rare. Lupus vulgaris is one of the common forms of cutaneous tuberculosis. There are a few reports of association of cutaneous tuberculosis with skeletal tuberculosis.,,, Lupus vulgaris has been very rarely reported along with TD.,
| Case Report|| |
A 8-year-old girl presented in skin department with a history of gradually progressive painless swelling of both hands and feet for the past 2 years. There was history of ulcerations on dorsum of hand and foot for the past 1 year. On examination, there was swelling over the dorsal aspect of second metacarpal and proximal phalangeal area of the right hand [Figure 1]a, fourth metacarpophalangeal area of the left hand, and dorsum of both feet. Swellings were nontender with no increased local temperature. Dorsum of the left hand showed three plaque lesions with central ulceration and margins showing hyperpigmentation surrounded by areas of atrophic scars [Figure 1]c. There were no puckered scars. These lesions were painless with a granulated floor, not fixed to underlying bone. Similar lesions were also seen on the dorsum of feet. Over the dorsum of right hand, there was an atrophic plaque lesion, with no preceding history of ulceration or discharge [Figure 1]a. There was no history of trauma, fever, weight loss, anorexia, or malaise. There was no family history of tuberculosis. No muscle atrophy was seen. Her general physical examination revealed left axillary lymphadenopathy of size 1.5 cm diameter. Rest of her general and systemic examination was unremarkable. BCG scar was present. The patient had received multiple courses of antibiotics with no relief.
|Figure 1: (a) Showing atrophic plaques on right hand. (b) Showing ulcerative lupus vulgaris lesions on left hand with swelling|
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Radiological examination of both hands (anteroposterior view) showed expansile lytic lesions in 1st and 5th metacarpals of the right hand and 1st and 2nd metacarpals of the left hand with erosive destruction of proximal phalanx and involvement of the metacarpo-phalangeal joint of the 4th digit of the left hand with multiple soft tissue swellings in both hands [Figure 2]. Bone density was maintained and there was no periosteal reaction. Anteroposterior view of both feet showed similar expansile lytic lesions of the 5th metatarsal joint of the right foot and 2nd metatarsal of the left foot with normal bone density and without periosteal reaction [Figure 3]. Lytic lesions were also seen in left calcaneum. Chest, skull, and spinal radiographs and abdominal sonography were normal. The erythrocyte sedimentation rate was 35 mm/1st hour, hemoglobin was 12 g/dl. Peripheral blood smear and tests for syphilis were normal. Mantoux test was positive, 17 × 17 mm. Zeihl–Neelsen staining from ulcer base showed no acid fast bacilli. Biopsy from the left axillary lymph node showed granulomatous inflammation without necrosis [Figure 4]. Biopsy from ulcer was consistent with lupus vulgaris. The diagnosis of tuberculous dactylitis with lupus vulgaris and tuberculous lymphadenopathy was made. The patient was put on antitubercular drugs (isoniazid, rifampicin, ethambutol, and pyrazinamide in initial phase and rifampicin and isoniazid in maintenance phase) and is now on follow up.
|Figure 2: Radiographs of bilateral hands anteroposterior view showing expansile lytic lesions in multiple metacarpals with erosion destruction of proximal phalynx and involvement of 4th metacarpophalangeal joint of the right hand with multiple soft tissue swellings in both hands. Bone density is maintained and there is no periosteal reaction seen|
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|Figure 3: Radiographs of both feet anteroposterior view showing expansile lytic lesions in multiple metatarsal bones with normal bone density and without periosteal reaction|
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|Figure 4: Histopathology from lymph node showing granulomatous inflammation|
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| Discussion|| |
Extrapulmonary tuberculosis is relatively more common in children than in adults. Skeletal tuberculosis is seen in 1%–3% of extrapulmonary tuberculosis in children. Tuberculous dactylitis is seen in children younger than 6 years and its incidence among children with tuberculosis was reported to be 0.65%–6.9%. Cutaneous tuberculosis has an incidence of 1% of extrapulmonary tuberculosis. Lupus vulgaris (LV) is the commonest form of cutaneous tuberculosis in adults, whereas scrofuloderma is the commonest form in children. LV is a multifactorial disease, which can be caused by contiguous lymphatic or hematogenous spread. The lesions have varied presentation in the form of plaque, ulcerative, mutilation, vegetating, or tumor form. Atrophic scarring may be seen even without prior ulceration as in our patient. It is a progressive and chronic form of tuberculosis. Skeletal tuberculosis in children involves peripheral bones symmetrically and lesions are nonsclerotic, whereas in adults they are axial and sclerotic. Tubercular involvement of short bones of hand and feet bones, known as tubercular dactylitis is extremely rare. Cystic tuberculosis is seen in children with good resistance and acquired immunity. Skeletal tuberculosis is generally secondary to hematogenous spread, most commonly from lungs (75% cases). As was observed in our case, no primary tuberculosis has been seen in the few other reported cases., Axillary lymph node involvement with multifocal tuberculosis may occur. Patients may have accompanying low-grade fever, anorexia, weight loss, and muscle atrophy around the joint. Hands are more frequently involved than feet. Proximal phalanx of middle and index fingers is the commonest involved site. Cystic tuberculosis is generally seen below the age of five years as these bones offer a fertile field for hematogenous bacterial implants.
In children, short tubular bones have a very good blood supply through a large nutrient artery entering almost at the middle of the bone. Tuberculous granulation tissue expands the relatively soft cortex as it is resorbed or infarcted by underlying process. Expansion of the bone with cystic changes is called spinaventosa. There is little reactive proliferation. Skeletal infection takes 1–3 years to manifest clinically. The lesions are paucibacillary, so there is a delay in diagnosis. TD may involve the overlying skin leading to cold abscess, ulceration, and sinus formation. Because of abscess formation overlying a swelling they are normally misdiagnosed as pyogenic infections. The differential diagnosis includes histiocytosis; lymphoreticular malignancy; syphilitic, pyogenic, or mycotic osteomyelitis; or neuroblastoma. Single lesion may be confused with sickle cell anemia, syphilitic lesions, leukemia, hyperparathyroidism, or fibrous dysplasia. In syphilis, the bone is thickened by periosteal reaction. In tubercular infection, there is diffuse osteopenia associated, which distinguishes it from pyogenic infection, as well as absence of sequestration. Also pyogenic osteomyelitis tends to be acutely painful, swollen, hot, and is associated with fever. Tubercular osteomyelitis is slightly painful, pyrexia is minimal. In sarcoidosis, well-demarcated cystic lesions are found in the phalanges of the fingers, there is no bony expansion and periosteal new bone formation. Punctate calcification is characteristic of enchondromatosis.
Both lupus vulgaris and TD can be caused by hematogenous spread and both are seen in patients with good resistance and acquired immunity., Both type of lesions are paucibacillary so Zeihl–Neelsen staining is usually negative. In both the conditions, Mantoux is strongly positive. Both lesions may arise without any primary lesion and may be multifocal. In spite of these common features, both these entities have been rarely reported concomitantly.
Diagnosis is usually done after excision biopsy. Mycobacterial culture is the gold standard for diagnosis. ATT is the mainstay of treatment of both conditions. Surgery is limited in curetting the cavities in promoting early healing in cystic tuberculosis.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]