|ONLINE ONLY - CASE REPORT
|Year : 2022 | Volume
| Issue : 1 | Page : 55
A rare cause of interstitial lung disease with skin lesion
Smera Ramakrishnan, Spandana P Hegde, Manjunath Shenoy Mala, Nazir Attar
Department of Dermatology and General Medicine, Yenepoya Medical College, Mangalore, Karnataka, India
|Date of Submission||26-Jun-2020|
|Date of Decision||05-Jan-2021|
|Date of Acceptance||03-Feb-2021|
|Date of Web Publication||25-Feb-2022|
Manjunath Shenoy Mala
Department of Dermatology, Yenepoya Medical College, Deralakatte, Mangalore, Karnataka
Source of Support: None, Conflict of Interest: None
We report a case of an interstitial lung disease of unknown etiology in a middle-aged man who later presented with skin lesions on the upper back. Histopathological examination showed noncaseating granulomas suggesting a possibility of cutaneous sarcoidosis. With further workup that included histopathology, the diagnosis of sarcoidosis was established. Sarcoidosis is a multisystem granulomatous disease of unknown etiology primarily affecting the lungs and intrathoracic lymph nodes but can affect virtually any organ. In our case, appearance of cutaneous lesion and further workup lead to the diagnosis of sarcoidosis.
Keywords: Cutaneous, interstitial lung disease, noncaseating granuloma
|How to cite this article:|
Ramakrishnan S, Hegde SP, Mala MS, Attar N. A rare cause of interstitial lung disease with skin lesion. Clin Dermatol Rev 2022;6:55
| Introduction|| |
The term interstitial lung disease (ILD) encompasses pulmonary manifestations of a large number of conditions such as connective tissue diseases, drugs, infections, and sarcoidosis. Diagnosis is challenging and often unresolved due to multitude of etiologies with overlapping clinical, radiological, and pathological presentations. Sarcoidosis is a rare cause of ILD. It is a multisystem disease of unknown etiology characterized by noncaseating epithelioid cell granulomas in different organs. Cutaneous lesions can occur in 20%–35% of patients with systemic sarcoidosis. The disease usually begins at around 40 years of age, and nearly two-thirds of the cases are females. Sarcoidosis is reported uncommonly from India, and coexistent cutaneous and systemic sarcoidosis is infrequent. In addition, cutaneous sarcoidosis is a great imitator like syphilis, but in our case, the presence of cutaneous lesion prompted the diagnosis of the recalcitrant internal disease.
| Case Report|| |
A 41-year-old male presented with insidious onset of cough with expectoration of 2-year duration. He also had generalized fatigue and weight loss. Respiratory system examination revealed bilateral coarse inspiratory crepitations. He had undergone evaluations by physicians and was treated as ILD of unknown etiology. He had received antibiotics, inhaled bronchodilators and corticosteroids, and short courses of systemic steroids. There was symptomatic improvement, while on treatment, however, there was a progressive deterioration of the symptoms. The patient did not have any other comorbidities on clinical evaluation.
During the course of stay in the hospital, dermatology reference was given as the patient complained of few minimally itchy lesions over his upper back. He noticed them a few months following the onset of respiratory symptoms, but the lesions were not causing major discomfort, hence he did not complain. It became prominent in the recent few weeks and brought the attention of the patient and the treating physician. Examination revealed multiple erythematous scaly confluent papules and plaques over the upper back [Figure 1]. Mucosa, hair, and nail examinations were normal.
|Figure 1: Upper back showing multiple erythematous scaly confluent papules and plaques|
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Blood counts, erythrocyte sedimentation rate, and biochemistry were normal. Mantoux test, sputum for acid-fast bacilli (AFB), and HIV serology were negative. Chest radiograph showed bilateral hilar and reticulonodular lung field opacities. Raised anti-cyclic citrullinated peptide (163Ru/ml) was also found. Antinuclear antibody test by indirect immunofluorescence was negative. A skin biopsy from the lesion over the right upper back showed numerous dermal confluent granulomas with epithelioid cells and giant cells of Langhans type along with paucity of lymphocytes [Figure 2]. Reticulin staining was positive and AFB staining was negative. This prompted the diagnosis of sarcoidosis.
|Figure 2: Histopathology examination (H and E, ×10) showing numerous dermal confluent granulomas with epithelioid cells and many giant cells of Langhans type with paucity of lymphocytes|
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In high suspicion of pulmonary sarcoidosis, a complete workup included serum angiotensin-converting enzyme (ACE) levels, pulmonary function test (PFT), and high-resolution computed tomography (HRCT). There were elevated ACE levels (150 U/L; reference range: 8–53U/L). PFT showed features suggestive of ILD. HRCT of the thorax showed centrilobular nodules, mild interstitial thickening, and areas of ground-glass appearance suggestive of usual interstitial pneumonitis type of ILD [Figure 3]. Multiple enlarged mediastinal lymph nodes in the pretracheal, hilar, and subcarinal region were also noted [Figure 4]. These radiological findings suggested that the patient has Stage 2 pulmonary sarcoidosis. Urine routine, liver function test, electrocardiogram, and ultrasound abdomen and pelvis were done and found to be normal.
|Figure 3: High-resolution computed tomography of the thorax showing mild interstitial thickening and areas of ground-glass appearance|
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|Figure 4: Multiple enlarged mediastinal lymph nodes noted in the pretracheal, bilateral hilar, and subcarinal region|
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With these clinical and laboratory findings, a final diagnosis of pulmonary and cutaneous sarcoidosis was made. It was the histopathology report that ultimately clinched and led us to the diagnosis. He was then treated with daily oral prednisolone 40 mg with which he experienced improvement of pulmonary symptoms. Cutaneous lesions completely subsided at the end of 10 days [Figure 5]. His steroid dose was gradually tapered and oral azathioprine was added as a steroid-sparing agent.
|Figure 5: Resolution of cutaneous lesions with oral prednisolone at the end of 10 days|
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| Discussion|| |
Sarcoidosis is a granulomatous disease with multisystem involvement that presents with cutaneous manifestation which can be broadly classified into sarcoid lesions and other lesions [Table 1]. Specific lesions are more often seen with chronic and recalcitrant course of the disease while nonspecific lesions are usually associated with acute but benign disease. Various cutaneous presentations are maculopapular, papular, plaque-type, annular, or nodular lesions. Erythema nodosum, angiolupoid, lupus pernio, subcutaneous nodules, and scar sarcoidosis are other cutaneous manifestations.
Cutaneous sarcoidosis is a great mimicker and distinguished by a massive list of differentials that include cutaneous tuberculosis, leprosy, psoriasis, syphilis, mycosis fungoides, and many other conditions. Histopathologically, sarcoidosis has to be distinguished from lupus vulgaris and leprosy as they all have epithelioid cell granulomas. While the granulomas in lupus vulgaris are caseous and present in the upper dermis, those in leprosy are mainly around dermal nerve twigs and admixed with abundant lymphocytic infiltration. Sarcoidal granulomas are discrete, uniformly distributed in the dermis, and surrounded by sparse lymphocyte cuffing (“naked tubercles”), with fine reticulin fibers in and around the tubercles. Serum ACE is derived from epithelioid cells of the granulomas and reflects the granuloma load in the patient. Serum ACE levels are neither diagnostic nor predictors of systemic involvement. It is elevated in about 60% of patients and helps in monitoring the clinical course of the disease.
The presence of pulmonary lesion, elevated ACE levels, a negative Mantoux test, and histopathology helped in the diagnosis in our patient. There are various treatment options for sarcoidosis, but corticosteroids remain the mainstay of treatment. Cutaneous sarcoidosis may be treated with topical or intralesional steroids and topical tacrolimus. For those with lupus pernio, bone cysts, and pulmonary involvement, treatment may be required for years and may need to be supplemented with steroid-sparing agents such as methotrexate, azathioprine, and mycophenolate mofetil. Our patient responded well to systemic steroids, and both his pulmonary and cutaneous diseases improved significantly.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]