A Rare Presentation of Sarcoidosis: A Case of Acute Onset Bilateral Pulmonary Infiltrates and Skin Lesions

Abstract

Sarcoidosis is a multisystem granulomatous disease of uncertain etiology that most often involves the lungs and skin, though nearly any organ may be affected. Pulmonary disease typically manifests as bilateral hilar lymphadenopathy with diffuse interstitial infiltrates, whereas cutaneous involvement is reported in about 25%–35% of cases, presenting as erythema nodosum, plaques, nodules, or lupus pernio. Acute onset of extensive pulmonary infiltrates with cutaneous lesions other than erythema nodosum is uncommon and frequently misdirects clinicians toward infectious or malignant causes, particularly in tuberculosis-endemic regions. We describe a 35-year-old previously healthy male who presented with sudden dyspnea, persistent cough, and erythematous plaques and nodules on the extremities. Chest imaging revealed diffuse bilateral infiltrates with mediastinal lymphadenopathy, and laboratory findings showed elevated serum angiotensin-converting enzyme levels. Pulmonary function testing demonstrated a restrictive pattern with impaired diffusion capacity, while bronchoalveolar lavage revealed lymphocytosis with a CD4/CD8 ratio above 3.5. Skin biopsy confirmed noncaseating granulomas, establishing the diagnosis of sarcoidosis with pulmonary and cutaneous involvement. The patient was started on oral corticosteroid therapy with prednisone 60 mg daily, gradually tapered according to clinical response. Within 2 weeks, there was marked improvement in respiratory symptoms and regression of skin lesions, leaving only residual pigmentation. Follow-up lung function and imaging demonstrated near-complete resolution, and steroids were successfully discontinued after 6 months without recurrence. This case highlights the importance of recognizing sarcoidosis in acute pulmonary–cutaneous presentations, where timely diagnosis and corticosteroid therapy can lead to excellent outcomes.

INTRODUCTION

Sarcoidosis is a multisystem granulomatous disorder of uncertain origin that predominantly affects young and middle-aged adults across the globe.[1] The worldwide burden of the disease is uneven, with higher prevalence rates reported in certain geographic and ethnic populations, such as Scandinavians and African Americans, while in many Asian countries, including India, it is often underdiagnosed or mistaken for other granulomatous conditions like tuberculosis.[2] Global estimates suggest prevalence ranges between 10 and 40 per 100,000 individuals, but the actual figures are likely higher due to variable diagnostic capabilities and reporting practices. Despite its geographic diversity, pulmonary disease remains the hallmark of sarcoidosis, typically manifesting as bilateral hilar lymphadenopathy and diffuse interstitial infiltrates. These features are often the earliest signs that raise clinical suspicion. In addition to lung involvement, the skin is affected in a significant subset of patients, roughly one-quarter to one-third, with lesions presenting as erythema nodosum, maculopapular rashes, plaques, nodules, or the chronic and disfiguring lupus pernio.[3] Lupus pernio, characterized by violaceous thickened plaques predominantly affecting the face, carries a strong association with persistent pulmonary disease. Because the skin is easily accessible for biopsy, dermatological findings often provide a pathway to earlier diagnosis when systemic manifestations remain ambiguous.[4]

The natural history of sarcoidosis generally follows a subacute or indolent course, but acute variants have been documented. One such acute form is Löfgren syndrome, which is classically described by the presence of erythema nodosum, bilateral hilar lymphadenopathy, periarticular inflammation, and fever.[5] However, not all presentations conform to this recognizable pattern. Cases in which acute pulmonary infiltrates occur simultaneously with cutaneous lesions other than erythema nodosum are rare and often create diagnostic dilemmas.[6] These atypical presentations may resemble infectious conditions such as tuberculosis or fungal pneumonia, malignant infiltration, or other inflammatory disorders. The overlap of clinical and radiological features can misdirect initial investigation and delay proper diagnosis, particularly in regions where alternative granulomatous diseases are more prevalent.[7]

The case under discussion illustrates such an uncommon scenario in which a patient presented with abrupt bilateral pulmonary infiltrates together with striking skin lesions not typical of Löfgren syndrome. The unusual clinical picture initially raised a strong suspicion for infectious or neoplastic pathology.[8] A thorough diagnostic approach that included chest imaging, histopathological examination of skin lesions, and a high index of clinical suspicion was essential to reach the correct diagnosis of sarcoidosis.[9] Figures 1-3 have been cited following the description of the histopathology findings and noncaseating granulomas ultimately confirmed the disease and guided appropriate management. This emphasizes the importance of considering dermatological manifestations as integral to systemic evaluation, since they often provide a less invasive and more accessible means of establishing diagnosis.[10]

Figure 1.

H and E-stained section showing a multinucleated giant cell with a central asteroid body radiating in a star-like fashion

Figure 3.

Histopathology section highlighting an asteroid body (arrow) within a giant cell, characteristic of sarcoidosis

Figure 2.

Multinucleated giant cell with a distinct eosinophilic stellate asteroid body, surrounded by inflammatory cells and foamy macrophages

This unusual presentation underscores several key lessons for clinicians. Sarcoidosis should be considered in the differential diagnosis of acute pulmonary syndromes worldwide, even when the onset is sudden and uncharacteristic.[11] Skin manifestations, being visible and amenable to biopsy, can expedite the diagnostic process and prevent mismanagement.[12] Histopathological confirmation remains indispensable, especially in regions with a high prevalence of other granulomatous diseases.[13] By documenting such a rare overlap between acute pulmonary and cutaneous sarcoidosis, this case reaffirms the protean nature of the condition and highlights the need for broad diagnostic vision. Recognition of atypical presentations contributes not only to timely and accurate patient care but also to a deeper global understanding of sarcoidosis and its variable clinical spectrum.[7]

CASE REPORT

A 35-year-old male with no significant been previously healthy, with no chronic cough, asthma, or prior exposure to tuberculosis, and denied occupational or environmental risk factors. He was a nonsmoker with only occasional alcohol use. Two days before admission, he developed progressive breathlessness, persistent cough, and erythematous skin eruptions over his arms and legs. The acute onset of pulmonary and cutaneous symptoms in an otherwise healthy individual prompted evaluation for infectious, inflammatory, and granulomatous diseases.

On examination, the patient was tachypneic with bilateral inspiratory crackles and multiple erythematous plaques and nodules over the extremities.

Chest radiography [Figure 4] revealed diffuse bilateral infiltrates. The initial chest X-ray also demonstrated cardiomegaly, likely secondary to myocardial edema and inflammatory involvement associated with systemic sarcoidosis . High-resolution computed tomography of the chest showed extensive ground-glass opacities with mediastinal and hilar lymphadenopathy, suggestive of an interstitial granulomatous process. Abdominal ultrasound did not show organomegaly or retroperitoneal lymphadenopathy. Laboratory evaluation demonstrated elevated serum angiotensin-converting enzyme levels, while other hematological and biochemical parameters remained within normal limits. Pulmonary function testing revealed a restrictive pattern with impaired diffusion capacity.

Figure 4.

Chest X-ray (PA view) showing bilateral hilar lymphadenopathy with perihilar infiltrates, consistent with Stage II sarcoidosis

Fundoscopic examination was performed to evaluate for ocular sarcoidosis, including features such as candle-wax drippings, choroidal granulomas, retinal vasculitis, or optic disc edema. No abnormalities were detected. There was no evidence of uveitis, retinal vasculitis, choroidal granulomas, or optic disc edema, and the fundus appeared normal.

Bronchoalveolar lavage showed lymphocytic predominance with a CD4/CD8 ratio >3.5. Histopathological examination of a skin biopsy confirmed noncaseating epithelioid cell granulomas, establishing the diagnosis of sarcoidosis with pulmonary and cutaneous involvement [Figures 1-3].

The patient was started on oral corticosteroid therapy with prednisone at 60 mg daily, tapered gradually according to clinical response. Supportive measures included bronchodilators to relieve breathlessness and gastric prophylaxis to counter steroid-induced side effects. Dermatological lesions were also managed conservatively, with the expectation that systemic therapy would induce resolution. Close monitoring with repeat pulmonary function tests, serum ACE levels, and follow-up imaging guided the course of treatment, and the patient was educated about adherence, potential adverse effects, and infection prevention while on long-term steroids.

Marked improvement was noted within 2 weeks of therapy, with a reduction in cough and dyspnea as well as regression of skin lesions, which left only mild pigmentation. Repeat lung function tests demonstrated improved parameters, and follow-up imaging revealed near-complete resolution of infiltrates and nodal enlargement [Figure 5]. The follow-up chest X-ray no longer demonstrated cardiomegaly, suggesting resolution of myocardial edema and inflammation following corticosteroid therapy. Dilated bronchi were noted posterior to the cardiac shadow; however, the patient had no clinical symptoms suggestive of bronchiectasis. Corticosteroids were successfully tapered and discontinued over 6 months, with no recurrence during subsequent visits. This case emphasizes the importance of considering sarcoidosis in acute presentations with pulmonary and skin involvement and highlights the favorable outcomes achievable with timely corticosteroid therapy.

Figure 5.

Chest X-ray (PA view) after 6 months of corticosteroid therapy, showing near-complete resolution of infiltrates and hilar lymphadenopathy

DISCUSSION

Our finding of a 35-year-old male with acute respiratory complaints, progressive breathlessness, persistent cough, and erythematous skin eruptions [Figure 6] closely aligns with previous literature. Foley et al. described concurrent pulmonary disease with worsening respiratory symptoms and prominent cutaneous lesions such as lupus pernio, where HRCT [Figure 7] and bronchoscopy confirmed active pulmonary involvement, emphasizing the value of skin manifestations as an accessible diagnostic window and their responsiveness to systemic therapy. Similarly, Seol et al. reported cutaneous sarcoidosis with pulmonary nodules and hilar adenopathy, where histology revealed noncaseating granulomas, underscoring the diagnostic significance of simultaneous skin-lung involvement. Their study highlighted the role of biopsy and imaging in confirming systemic disease and guiding corticosteroid-based management. Together, these studies support our case, demonstrating the importance of recognizing acute pulmonary–cutaneous presentations as potential indicators of systemic granulomatous pathology.[14,15]

Figure 6.

Multiple hyperpigmented and scaly patches with post-inflammatory pigmentation over the legs; skin biopsy revealed noncaseating granulomas

Figure 7.

High-resolution computed tomography of the chest showed extensive ground-glass opacities with mediastinal and hilar lymphadenopathy

Our finding of sarcoidosis with pulmonary and cutaneous involvement, supported by imaging, elevated serum ACE, restrictive pulmonary function, BAL lymphocytosis with a CD4/CD8 ratio >3.5, and skin biopsy showing noncaseating granulomas, closely correlates with recent studies. Foley et al. reported a patient with classic lung and skin manifestations, radiographic and spirometric abnormalities, and disease refractory to oral corticosteroids and hydroxychloroquine, eventually requiring TNF-inhibitor therapy for clinical and functional improvement, highlighting the spectrum of therapeutic response. Zannese et al. emphasized the prognostic relevance of BAL profiles, demonstrating that patients with a CD4/CD8 ratio <3.5 were prone to severe respiratory impairment, multisystem involvement, and higher immunosuppressive needs, while those with a CD4/CD8 ratio ≥3.5, as in our case, often had more cutaneous disease and a milder overall course. Together, these studies reinforce the diagnostic and prognostic value of integrating histology, imaging, BAL analysis, and clinical features in sarcoidosis management.[14,16]

Our findings show that the patient responded to oral corticosteroid therapy initiated with prednisone 60 mg daily, tapered gradually with supportive measures and structured monitoring through pulmonary function tests, serum ACE levels, and imaging. This approach aligns with Dhooria et al., who in a randomized trial demonstrated that higher doses, such as 40 mg, were not superior to 20 mg for lung outcomes or HRQoL, supporting the use of the lowest effective dose with close follow-up for relapse. Dhooria further outlined a practical protocol recommending prednisolone ~20 mg/day in organ-involved disease, with a short higher starting dose followed by careful tapering, structured monitoring, and escalation to steroid-sparing agents if toxicity or poor response occurs. Similarly, Di Marco Berardino et al. reinforced systemic corticosteroids as the mainstay for pulmonary and cutaneous sarcoidosis but highlighted the broad adverse effect spectrum, emphasizing tailored dosing, structured monitoring, tapering, and timely consideration of alternative immunosuppressants.[17,18,19]

Our finding of rapid improvement within 2 weeks of corticosteroid therapy, with resolution of respiratory symptoms and regression of skin lesions leaving only mild pigmentation, correlates closely with prior reports. Seol et al. described a case of cutaneous sarcoidosis with pulmonary involvement following SARS-CoV-2 mRNA vaccination, where systemic plus topical corticosteroids achieved marked improvement in skin lesions and hilar lymphadenopathy by 3 months, with successful tapering and discontinuation without relapse, underscoring the benefit of timely therapy with structured monitoring. Similarly, Gupta et al. reported a patient who started on prednisolone 60 mg/day who showed remarkable improvement within 2 weeks, complete resolution by 2 months, and sustained remission after tapering steroids over 6 months without recurrence. These findings reinforce our observation that acute sarcoidosis with pulmonary and cutaneous manifestations responds favorably to early corticosteroid therapy with carefully monitored tapering.[15,20]

CONCLUSION

The present case highlights the rare coexistence of acute pulmonary infiltrates and cutaneous sarcoidosis in a previously healthy young male, emphasizing the protean nature of the disease. Timely recognition of dermatological manifestations, supported by imaging, bronchoalveolar analysis, and histopathological confirmation, enabled accurate diagnosis and effective management. Oral corticosteroid therapy led to rapid and sustained clinical, functional, and radiological improvement, with complete resolution of lesions and no recurrence on follow-up. This case underscores the importance of maintaining a broad differential diagnosis in acute presentations, as early intervention with structured monitoring can ensure favorable outcomes in sarcoidosis.

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.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.