Skin as a marker of internal disease: a case of sarcoidosis.

A 52-year-old female presented to our out patient department with asymptomatic, hypopigmented lesions on the neck and back since 2 months. There was a history of taking antitubercular treatment for suspected pulmonary tuberculosis 2 years back. On blood investigations, the serum angiotensin converting enzyme levels were increased and the skin biopsy revealed a naked granuloma in the dermis. A diagnosis of systemic sarcoidosis was made and the patient was started on oral corticosteroids and Methotrexate, with clinical improvement.

Introduction

Multisystem diseases of undetermined etiology and exibiting a wide spectrum of clinical features can be diagnosed with the help of proper and thorough clinical examination of the involved or suspected-to-be-involved systems. Here, we are presenting the case of a middle-aged, apparently healthy female with skin rash that was not associated with any symptoms. We would like to elaborate how examination of the pulmonary system with the help of investigations led us to the final diagnosis of systemic sarcoidosis.

Case Report

A 52-year-old female patient came with the chief complaints of multiple asymptomatic, hypopigmented, raised lesions over the neck and upper back since 2 months. There was a history of gradual increase in the number of lesions. On further enquiry, she revealed that she had taken 6 months of antitubercular treatment 2 years ago. There was no history of hypopigmented, anesthetic patches on the body, epistaxis, pedal edema or any other systemic complaints.

Cutaneous examination revealed multiple, well-defined, hypopigmented and erythematous papules ranging from 1 mm to 1 cm in size, present over the nape of the neck and upper back, with few papules coalescing to form plaques [Figure 1]. Skin biopsy stained with hematoxyline and eosin showed normal epidermis, while the papillary dermis showed a well-defined granuloma composed of epithelioid cells [Figure 2] with absence of a lymphoctic rim in the periphery. In the center of the granuloma, few Langhan's giant cells were noted [Figure 3]. These features were suggestive of cutaneous sarcoidosis.

Figure 1

Clinical photograph

Figure 2

Hematoxylin and eosin under low power

Figure 3

Naked granuloma under high power

The patient was further investigated to look for any systemic involvement. All the blood investigations were within normal limits except for raised serum angiotensin converting enzyme levels (72U/L) [Table 1]. The chest, on an anterioposterior view, revealed bilateral hilar lymphadenopathy whereas the computed tomography-guided lung tissue biopsy showed necrotizing granuloma [Figure 4].

Table 1

Blood and urine investigations

Figure 4

Chest X-ray

In view of this, a final diagnosis of systemic sarcoidosis was made and the patient was started on topical midpotency steroids and was asked to follow-up every 2 weeks. However, after 2 months of treatment, the patient did not respond well and also complained of similar lesions on the face. Then, she was started on oral corticosteroids in the form of tab Prednisolone 40 mg once daily. After no response to this therapy in 8 weeks, the patient was started on oral methotrexate 7.5 mg once a week. The patient responded well to the therapy after 6 weeks and is on monthly follow-up since the last 6 months.

Discussion

Sarcoidosis is one of the greatest mimickers in dermatology because of its wide spectrum of presentation, from innocent lesions to florid manifestations. Sarcoidosis is a multisystem disease of undetermined etiology, characterized by the formation of granulomas in different organs of the body.[1] The disease is more common in females than in males of the age group of 30–40 years. In 1889, Besnier reported the first case of sarcoidosis.[12] In 1899, Boeck reported a case of multiple benign cutaneous sarcoid.[3] In 1957, Rajam reported the first case of sarcoidosis in India.[12] The organs involved include the skin, eyes, lymph nodes, respiratory system, cardiovascular and nervous systems, skeletal system and genitourinary system. About 20–35% of the patients with systemic sarcoidosis have skin lesions. In about 20% of the cases, skin lesions appear before systemic disease. In 50%, there is a simultaneous appearance. In 30%, the skin lesions appear 10 years after the systemic disease has occurred.[34] The lesion of sarcoidosis is generally of a papular,[5] nodular or plaque type and also may be annular, ulcerative,[6] lupus pernio or erythema nodosum.[7] Treatment is with topical corticosteroid and cryotherapy and for hypopigmented and erythrodermic sarcoidosis, psoralens with ultraviolet A can be used. In systemic sarcoidosis, oral steroids in the dose of 30–40 mg can be given daily, and then tapered gradually[8] over 6 months. Methotrexate,[9] Chlorambucil,[10] Allopurinol[11] and Thalidomide are few of the other drugs proven to be useful.

This case was earlier diagnosed as pulmonary tuberculosis and given 6 months of antitubercular treatment empirically, which delayed the correct diagnosis of systemic sarcoidosis. However, in this case, the tiny papules on the skin guided our way to identify the pulmonary system involvement. Therefore, we would like to once again emphasize on cutaneous examination in any case of systemic disease as “Skin is the marker of internal diseases” still holds true.