A Case of Multifocal Tuberculosis Involving Skin and Bone: An Uncommon Presentation.

Sir,

A 22-year-old female presented to the dermatology outpatient department with lesions on face and back since 5 years of age, which aggravated since 2 weeks. A single red raised lesion was present on the scalp for 5 years, associated with loss of hair. Painful swellings on left thumb, and great toe were present for 3 months, which broke down to form an open wound since 2 weeks. There was history of hoarseness of voice, difficulty in consumption of food due to pain, loss of weight, and loss of appetite. On examination, retroauricular, submandibular, bilateral inguinal, right axillary group of lymph nodes were palpable but nontender. Weight at presentation was 27 kg and body mass index was 11.24 kg/m2. On cutaneous examination, there were multiple erythematous plaques with areas of atrophy, crusting over the scalp with loss of hair, perioral area, nose, forehead, right arm, and upper back. There was associated restriction of mouth opening. Erosion with hemorrhagic crust was present on the lower lip. There were multiple ulcers involving left thumb and left foot, with largest one measuring 4 cm × 5 cm. Deformity and swelling of left wrist and left thumb with restriction of movements was seen. Enlargement of the epiglottis was seen on laryngoscopy. A provisional diagnosis of lupus vulgaris was made. X-ray of left hand and feet showed gross destruction of all joints with cystic changes and osteoporosis [Figure 1a and b]. Purulent discharge from the ulcer was sent for Gram's staining, acid fast bacilli (AFB) staining, and culture. The Gram's stain showed Gram-negative bacilli and few pus cells; culture showed the growth of coagulase negative Staphylococcus, Pseudomonas aeruginosa, and Escherichia coli, AFB was negative. Histopathology of lesional skin and nasal mucosa was done; it had features suggestive of suppurative granuloma but with no definite conclusion [Figure 1c and d]. Chest X-ray was normal. Mantoux test was strongly positive. HIV serology, antinuclear antibody, c-ANCA and p-ANCA were negative. Patient was started on category 1 DOTS as therapeutic trial, and response to treatment was excellent [Figures 2–4].

Figure 1

(a and b) X-ray of left hand and left foot – destructive arthropathy and osteoporosis. (c) Features suggestive of suppurative granuloma, with few Langhans giant cells (H and E stain, ×10). (d) High power view showing epithelioid cells (H and E stain, ×40)

Figure 2

(a) Erythematous plaque present over perioral region, extending to involve the nose and bilateral cheeks; (b) after completion of treatment

Figure 4

(a) Multiple ulcers with largest one measuring 2 cm × 2 cm on left thumb; (b) after completion of treatment

(a) Multiple ulcers with largest ulcer measuring 4 cm × 5 cm on the left great toe; (b) after completion of treatment

Cutaneous tuberculosis is an uncommon type of extrapulmonary tuberculosis. It has a prevalence of 1.5% of all cases of tuberculosis.[1] As a consequence of failure in early diagnosis, the disease can progress to necrosis, destruction of bones, and cartilage, leading to permanent deformity, and this can be disastrous to the patient.[2] Multiple cystic tuberculosis of the bone is a rarer variant of tuberculosis.[3]

Lupus vulgaris is one of the major forms of cutaneous tuberculosis, which usually occurs in a previously sensitized individual with moderate immunity. It can be acquired through both endogenous and exogenous routes.[145] Our patient might have acquired the disease through exogenous route of spread. In India, it commonly presents on the buttocks, thighs, and legs. Face is usually a common area of involvement in European countries. Hence, involvement of nose in India is quite uncommon, so there are high chances of it remaining undiagnosed and leading to irreversible deformities.[5] It is the atypical presentation and unusual site of involvement that often leads to misdiagnosis, thus leading to significant morbidity.[6] Our patient suffered from this disease for the past 17 years, and facial scarring could have been avoided by early diagnosis.

There are many conditions that mimic lupus vulgaris on the nose, which include lupus pernio (sarcoidosis), tuberculoid leprosy, lupoid leishmaniasis, Wegner's granulomatosis, Bowen's disease, deep fungal infection, lymphoma, necrobiotic xanthogranuloma, and psoriasis.[7]

The diagnosis of lupus vulgaris is mainly through combination of clinical features, tissue culture, and skin biopsy. It is a paucibacillary variant of cutaneous tuberculosis, and detection of AFB on culture may not always yield a positive result. Histopathological examination and response to antitubercular treatment are essential for proper diagnosis.[18]

The most frequent site of involvement in skeletal tuberculosis is spine followed by joints. Tuberculous dactylitis and cystic tuberculosis are rare.[6] Jungling reported nine cases of “osteitis tuberculosa multiplex cystoides” in 1920, which occurred in adults and was seen in the hands and feet.[9] Jungling disease or multiple cystic tuberculosis lesions of skeleton is a rare form of skeletal tuberculosis, which used to be reported extensively 30–40 years ago. In recent times, no such severe type of tuberculosis is often reported. The simultaneous occurrence of skin and skeletal tuberculosis is rare, rarer is the occurrence of lupus vulgaris with Jungling disease, which was seen in our patient. It usually has two varieties – child and adult. A number of terminologies have been used for multiple cystic tuberculosis, including disseminated bone disease in adults and Jungling's disease, pseudocystic tuberculosis in children. Adult type mainly involves the axial skeleton and the child type usually affects the metatarsal regions of tubular bones. Mantoux positivity is seen in 60–80% of cases. Though rare, the prognosis of this type of tuberculosis is good, with chemotherapy being the main stay of treatment, especially in the adult form.[310] Due to the increasing number of extrapulmonary cases in recent times, a combination of skin and bone involvement can be expected to occur more frequently in the future.

Both lupus vulgaris and multifocal bone tuberculosis are seen in patients with good immunity.[10] Strong Mantoux positivity in our patient supports this view.

Based on clinical features, positive Mantoux test, and finding of granulomatous dermatitis on histopathology, patient was started on antitubercular drugs as a therapeutic trial. Response to therapy in our patient was excellent. The lesions improved within 1 month and all the lesions healed with residual scarring. There was drastic improvement in weight in our patient from a baseline weight of 27 kg to 42 kg at the end of treatment, with a total weight gain of 15 kg.

Lupus vulgaris can be cured completely. However, the morbidity seen in this disease is mainly attributed to failure in making early diagnosis, leading to disease progression, which can cause necrosis, destruction of underlying cartilage and bones, contributing to permanent scarring, as seen in our patient.[6]

A high degree of suspicion is required to examine and evaluate patients for tuberculosis. In a country like India, where the prevalence of tuberculosis is very high, this disease has to be ruled out whenever indicated. Hence, a therapeutic trial of antitubercular therapy has to be considered when tuberculosis is suspected, even when the conventional methods of diagnosis are inconclusive.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.