Atypical Protean Manifestations of Cutaneous Sarcoidosis.

Sir,

Sarcoidosis is a multi-system granulomatous disease of unknown etiology with protean manifestations most commonly affecting lung and lymph nodes. Approximately 25%–35% of cases of sarcoidosis develop cutaneous manifestations.[12] Cutaneous sarcoidosis is a great mimicker due to its diverse clinical manifestations and diagnosis depends on histopathological evaluation. In this article, we describe a patient with polymorphic manifestations of sarcoidosis in whom the diagnosis was confirmed by biopsy.

A 45-year-old man presented to the outpatient department of our institution with alopecia and multiple asymptomatic lesions over the body of 12 months duration. The patient denied any history of systemic symptoms like dyspnoea and chest pain. On examination, there was generalized lymphadenopathy of cervical, axillary and inguinal group of nodes. Systemic examination including ophthalmological evaluation was within normal limits. Cutaneous examination showed scarring alopecia over the vertex of scalp and multiple erythematous plaques of varying sizes involving forehead, periorbital area, nape of neck, trunk, and extensor aspect of legs [Figures 1 and 2]. Diascopy of lesions over face showed apple jelly nodules. A tender plaque was present over the nose with scaling on the ala nasi [Figure 1d]. Lesions over the back appeared psoriasiform with fine scaling and surrounded by a halo [Figure 2a]. Multiple skin colored to erythematous grouped papules were present over the nape of the neck [Figure 2b]. Extensor aspect of knees had erythematous to hyperpigmented atrophic plaques [Figure 2c]. Single well-defined ulcerated lesion with yellow-colored crust was present over the right arm over a preexisting plaque. A subcutaneous non-tender nodule was present over left arm [Figure 2d]. A clinical differential diagnosis of cutaneous sarcoidosis and lupus erythematosus was made.

Figure 1

(a and b) Erythematous plaques with smooth surface present over the forehead, peri-orbital, and infra-orbital region of face. The lesions have classical morphology of sarcoidosis (c) Patch of cicatricial alopecia with dyspigmentation. (d) Erythematous tender plaque over the nose with scaling at ala nasi

Figure 2

(a) Erythematous plaques with surrounding hypopigmentation. Few lesions were scaly. Inset: An ulcerated lesion with yellowish crust (b) Skin colored to erythematous papules coalescing to form plaques over the nape of neck. (c) Atrophic lesions over the extensor aspect of knee (d) Subcutaneous nontender nodule over the left upper arm

Standard hematological and biochemical profiles were normal whereas angiotensin-converting enzyme (ACE) levels were found to be increased (232 IU/L; normal range: 8.3–24.1 IU/L). Mantoux test was negative suggestive of anergy. Bilateral hilar lymphadenopathy was present on chest X Ray [Figure 3a]. Pulmonary function tests and electrocardiogram (ECG) were normal and ultrasound abdomen showed periportal & peripancreatic lymphadenopathy. Fine Needle Aspiration cytology (FNAC) from cervical and axillary lymph nodes showed reactive changes. Skin biopsies were done from truncal plaque, subcutaneous nodule, margin of alopecic patch, and psoriasiform lesion over the leg. Biopsies from all sites revealed the consistent finding of multiple well-formed non-caseating epithelioid cell granulomas with sparse lymphocytic infiltrate in superficial and mid dermis [Figure 3b, c]. Reticulin stain showed granulomas with reticulin fibers.

Figure 3

(a) Chest X-Ray (PA view) showing bilateral hilar lymphadenopathy. (b) Scanner view photomicrograph of skin biopsy showing multiple pandermal granulomas (H and E ×40). (c) Medium power showing noncaseating epithelioid cell granulomas with mild sprinkling of lymphocytes (H and E ×100)

Based on the above findings, a diagnosis of cutaneous sarcoidosis was made, and the patient was started on oral steroids (methylprednisolone) and methotrexate. There was flattening of lesions after 10 weeks of treatment. However, the patient was lost to follow up later.

Sarcoidosis is a granulomatous disease of unknown etiology with characteristic findings of non-caseating epithelioid cell granulomas, variable multinucleated giant cells, perivascular mononuclear cell infiltrates.[1] Cutaneous sarcoidosis is usually an early expression of the disease which requires evaluation for systemic involvement.[2] In 90% of patients pulmonary involvement is the most prominent systemic association. In 39% of patients' musculoskeletal involvement is seen with the most common symptoms of weakness, pain, erythema and tenderness, arthritis, arthralgia, tenosynovitis.[1] Our patient, with bilateral lymphadenopathy and elevated ACE levels, did not have any systemic involvement. Lymphadenopathy is present in 90% of patients with systemic sarcoidosis. Hilar and/or paratracheal adenopathy is the most classical presentation.[1] Elevated ACE levels are the markers of sarcoid activity because of T-cell-stimulated epithelioid cells in sarcoid granuloma that show increased expression of ACE.[13]

Cutaneous lesions of sarcoidosis can be specific and non-specific. Nonspecific lesions show varying morphology and do not exhibit granulomas on biopsy. Among the specific lesions, maculopapules are the commonest followed by plaques. Both maculopapules and plaque [Figures 1] and 2] show diverse colors including red, reddish-brown, translucent violaceous or hyperpigmented. Lupus pernio is the most characteristic cutaneous lesion of sarcoidosis which presents as indurated plaque or papule involving nose [Figure 1d], cheeks, ears, lips, and forehead. It is generally violaceous and is associated with upper respiratory involvement. The latter was absent in our patient. In 20-35% of patients with systemic sarcoidosis, subcutaneous involvement occurs. It may also occur alone without systemic disease.[4] Subcutaneous sarcoidosis appears at onset of disease and is usually the main complaint at diagnosis [Figure 2d].

Sarcoidosis can also involve the scalp in the form of localized lesions or diffuse alopecia. Scarring alopecia [Figure 1c] is rare and affects predominantly women of African descent.[5]

Ulcerative sarcoidosis is seen worldwide in only 5% of patients with cutaneous sarcoidosis.[6] It is twice as common in black versus white individuals. Ulceration is more commonly seen in preexisting scars or cutaneous lesions but can also arise de novo. Ulcerative sarcoidosis can present in two distinct patterns. Violaceous nodules arising in an annular confluent pattern which can eventually ulcerate or necrotic yellow plaques with ulceration as seen in our patient [Figure 2] inset].[6] Psoriasiform sarcoidosis is a rare variant of cutaneous sarcoidosis usually seen in dark-skinned patients [Figure 2a]. The lesions that heal without scarring and are most commonly seen over the lower legs.[7]

Our case highlights an unusual presentation of cutaneous sarcoidosis with multiple morphological variants present in the same patient. Various morphologies have distinct prognosis based on their chronicity but their presence simultaneously in a particular patient is an enigma that needs to be studied further.

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Conflicts of interest

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