Melanoma Developing from an Intradermal Nevus: Report on Two Patients.

Dear Editor, Approximately 25-33% of cutaneous melanomas arise from nevi (1). Shitara et al. suggested that junctional and compound nevi are more likely give rise to melanoma than intradermal nevi, but this has not been definitively confirmed (2). Based on these results and our own clinical observation on rare malignant transformation in intradermal nevi, we present two patients with melanoma developing from an intradermal nevus. The first patient, a 63-year-old woman, presented with a suspicious lesion in 2017 on the upper back in the form of a dark brown macula juxtapositioned next to the dermal nevus (Figure 1, a). Dermoscopy of a flat part showed a dark-brown reticular, slightly structureless pattern (Figure 1, b). The patient was therefore referred to surgical excision. Histopathology of the elevated part showed aggregates of intradermal nevus cells of normal morphological characteristics. Atypical and irregularly sized melanocytes were observed in the flat part, infiltrating the entire depth of the epidermis and the upper parts of the papillary dermis. The diagnosis of malignant melanoma developing from a dermal nevus was established (Breslow 0.4 mm, pT1A) (Figure 1, c). The second patient, a 71-year-old man, presented in 2018 with a pendular non pigmented intradermal nevus on middle part of the back. The left-hand lateral side of the intradermal nevus showed a brown to dark-brown spot which measured 12 mm (Figure 2, a). A central blue white veil, atypical pigment network, and dots and globules of various sizes and shapes were observed on dermoscopy (Figure 2, b). The base of the nevus showed an asymmetric pigmentation. Because the lesion was highly suspicious of melanoma, an urgent excision was indicated. The histopathology of the elevated part (dermal nevus) showed a regular maturation of the nest of nevus cells in the dermis. The histopathology of the dark-brown macule showed proliferation of atypical melanocytes with well-marked nucleoli throughout the epidermis with the infiltration of the suprabasal epidermal layers and papillary dermis. The lesion was classified as melanoma with a partial regression (Breslow 1.3 mm, pT2A), arising in association with an acquired intradermal nevus (Figure 2, c). Case reports with melanoma developed from a small congenital or acquired dermal nevus are extremely rare in the literature. In all published cases, histopathology revealed a melanoma component situated below or laterally, next to the merging dermal nevus (3) and in one case next to and above the dermal component (4), which is very similar to our cases. In both of our cases, melanoma presented an epidermal component with atypical, large melanocytes next to or above the typical and small intradermal melanocytes of the Unna nevus. Despite the fact that the reported statistical occurrence of malignant transformation of every individual nevus is very low in the elderly population (>60 years of age), 1 in 33,000 (5), we believe our two presented cases show a striking similarity in the melanoma manifesting in the vicinity of a previously existing lesion, indicating nevus-associated melanoma (NAM). This letter presents an interesting finding of two cases, with a form of melanoma (NAM) that is statistically very rare in older patients but occurred twice within the span of a year within the same town and was diagnosed in the same hospital. Intradermal nevi are most commonly considered to be benign skin lesions. However, previous research and our two cases shows that intradermal nevi are not immune to malignant alteration. Based on these results, we suggest a detailed clinical and dermoscopic evaluation of each skin lesion, including intradermal nevi. Flat melanocytic parts in the vicinity of intradermal nevi should always raise suspicion and warrant excision with histopathological evaluation of the lesion so as to allow timely response to any malignant alteration.

Pemphigus Vegetans of Hallopeau: A Case Report.

Dear Editor, Pemphigus vegetans (PV) of Hallopeau is a rare and indolent variant of pemphigus clinically characterized by vegetating lesions preceded by pustules mainly in flexural areas (1,2). This helps us to differentiate it from PV of Neumann, which is a more extensive and refractory disease, more alike to a pemphigus vulgaris outbreak with blisters which turn into vegetating plaques (3). We report the clinical presentation, course, and therapeutic response in a patient diagnosed with PV of Hallopeau from its early stage during a 3-year follow up. A 62-year-old man, non-smoker, presented at our clinic in July 2018 with hemorrhagic-serous crusts and fissures on the vermilion of the lower lip (Figure 1, a) and two merged circinate, sharply demarcated plaques on the right side of the groin (Figure 1, b). Plaque margins were elevated, with hypertrophic granulation tissue studded with pustules. Mucosal and cutaneous lesions persisted 6 and 4 weeks, respectively. The rest of the mucosa and skin were unaffected; the general state was good. The patient's family history for skin diseases was negative. The medical history included hypertension, atherosclerosis and hypercholesterolemia, hiatus hernia, and recent surgery (3 months prior) of an aortic abdominal aneurysm with reconstruction and synthetic graft placement. He was taking antihypertensives (fixed combination of 3 drugs, among them the ACE-inhibitor perindopril) with well-regulated blood pressure, statins, a pump-proton inhibitor, and acetylsalicylic acid. Differential blood count revealed eosinophilia. Histopathology finding showed acanthosis, suprabasal clefting with a suprabasilar bulla and acantholysis, prominent eosinophilic intraepidermal spongiosis, and heavy dermal infiltration of eosinophils and lymphocytes (Figure 2, a and b). The diagnosis of pemphigus was confirmed by direct immunofluorescence (DIF), which detected C3 deposits on the surface of keratinocytes throughout the epidermis of perilesional skin. Circulating pemphigus antibodies were detected by indirect IF. Only Dsg 3 antibodies were detected using an ELISA assay (233.23 RU/mL). After establishing the diagnosis of PV of Hallopeau, treatment with prednisolone 0.75 mg/kg/day orally in combination with adjuvant immunosuppression (azathioprine 100 mg daily) was started. Appropriate topical therapy with local steroids and antiseptic was applied. The steroid dose was titrated and gradually tapered down to the minimum required to control the disease - 10 mg. One-year remission was achieved. Azathioprine was withdrawn in October 2019 and since then the patient experienced a flare-up twice. The control of pemphigus flare-ups was achieved by a low dose of steroids (30 mg prednisolone orally). It remains debatable whether surgical trauma and radiology procedures such as angiographies (4) well as ACE-inhibitor drugs (5) triggered or aggravated the pemphigus. Early recognition and correct diagnosis of this rare type of pemphigus allows us to treat and control the disease successfully with lower doses of steroids, reducing complications to the minimum.

Dermoscopy of Zosteriform and Swirling Pattern Type 1 Segmental Darier Disease.

Dear Editor, Segmental Darier disease (DD) is a rare disease with around 40 described English literature cases. It is hypothesized that one of the causes of the disease is a post-zygotic somatic mutation for the calcium ATPase pump, only present in lesional skin. There are two types of segmental DD: type 1, where lesions follow Blaschko's lines unilaterally, and type 2, characterized by focal areas of increased severity in patients with generalized DD (1). Type 1 segmental DD is not easily diagnosed due to the lack of positive family history, the late onset of the disease in the third or fourth decade of life, and lack of DD-associated features. The differential diagnosis of type 1 segmental DD includes acquired papular dermatoses distributed in linear or zosteriform fashion, such as lichen planus, psoriasis, lichen striatus, or linear porokeratosis (2). We report two cases of segmental DD, of which the first case was a 43-year-old woman who presented with pruritic skin changes five years in duration and a history of seasonal aggravation. On examination, light brownish to reddish keratotic small papules were observed on the left abdomen and inframammary area, arranged in a swirling pattern (Figure 1, a). Dermoscopy showed polygonal or roundish yellowish/brown areas surrounded with whitish structureless areas (Figure 1, b). The histopathological correlations for dermoscopic brownish polygonal or round areas are hyperkeratosis, parakeratosis, and dyskeratotic keratinocytes, which were present in the biopsy specimen (Figure 1, c). The patient was prescribed 0.1% tretinoin gel, which led to marked improvement (Figure 1, d). The second case was a 62-year-old woman who presented with a flare of small red-brown papules, eroded papules, and some yellowish crusts arranged in a zosteriform pattern on the right side of the upper abdomen (Figure 2, a). Dermoscopy showed polygonal, roundish, yellowish areas surrounded with whitish and reddish structureless areas (Figure 2, b). Histopathology mainly revealed compact orthokeratosis and small foci of parakeratosis, marked granular layer with dyskeratotic keratinocytes, and foci of suprabasal acantholysis consistent with the diagnosis of DD (Figure 2, d, d). The patient was prescribed topical steroid cream and 0.1% adapalene cream, which also led to improvement. In both of our cases, a final diagnosis of type 1 segmental DD was established based on clinico-histopathologic correlation, since acantholytic dyskeratotic epidermal nevus could not have been ruled out only based on the histopathology report as it is clinically and histologically indistinguishable from segmental DD. However, the late age of onset and aggravation resulting from external factors such as heat, sunlight, and sweat supported the diagnosis of segmental DD. Although the final diagnosis of type 1 segmental DD is typically established based on clinico-histopathological correlation, we find dermoscopy particularly useful in aiding the diagnosis by eliminating differential diagnoses and being aware of their well-known dermoscopic patterns.

Dermatoscopy of nodular/plaque-type primary cutaneous T- and B-cell lymphomas: A retrospective comparative study with pseudolymphomas and tumoral/inflammatory mimickers by the International Dermoscopy Society.

BACKGROUND: Limited data on dermatoscopy of nodular/plaque-type T-/B-cell primary cutaneous lymphomas (PCLs) is available. OBJECTIVE: To describe dermatoscopic features of nodular/plaque-type PCLs, comparing them with those of clinical mimickers (pseudolymphomas, tumors, and inflammatory lesions) and investigating possible differences according to histologic subtypes. METHODS: Participants were invited to join this retrospective, multicenter case-control study by submitting histologically/immunohistochemically confirmed instances of nodular/plaque-type PCLs and controls. Standardized assessments of the dermatoscopic images and comparative analyses were performed. RESULTS: A total of 261 lesions were included (121 PCLs and 140 controls). Orange structureless areas were the strongest PCL dermatoscopic predictor on multivariate analysis compared with tumors and noninfiltrative inflammatory dermatoses. On the other hand, a positive association was found between PCLs and either unfocused linear vessels with branches or focal white structureless areas compared with infiltrative inflammatory dermatoses, whereas white lines were predictive of PCLs over pseudolymphomas. Differences in the vascular pattern were also seen between B- and T-cell PCLs and among B-cell PCL subtypes. LIMITATIONS: Retrospective design and the lack of a dermatoscopic-pathologic correlation analysis. CONCLUSION: Nodular/plaque-type PCLs display dermatoscopic clues, which may partially vary according to histologic subtype and whose diagnostic relevance depends on the considered clinical differential diagnoses.

Hyperpigmented Mycosis Fungoides - A Rare Entity.

Dear Editor, A 67-year-old man of Kosovar-Albanian ethnic origin (skin phenotype IV) presented to our dermatology clinic with generalized hyperpigmented patches and plaques all over the body, so-called melanoerythroderma (Figure 1). The lesions, which first appeared nearly six years ago, developed gradually; they were diagnosed as mycosis fungoides (MF), and were subsequently treated only with topical corticosteroids. We performed further examinations upon admission to our department. Relevant laboratory parameters - blood cell count, LDH, urinalysis, and serum chemistry - were within normal limits. Endocrinological examination excluded Addison disease, and the patient was not receiving any drugs that could cause skin hyperpigmentation. Chest-abdomen-pelvis computed tomography (CT) scan and sternal puncture were normal. Flow cytometric immunophenotyping revealed less than 5% aberrant T-cells. Histopathology and immunohistochemistry of skin specimens revealed lichenoid infiltration of small- to medium-sized atypical T-lymphocytes within the upper dermis, epidermotropic lymphocytes with several Pautrier's microabscesses (Darier's nests), pigment incontinence, abundant melanophages in the papillary dermis (Figure 2, a, b), and the T-cell CD4+CD7-CD8+ phenotype (Figure 2, c, d). Based on the clinical picture, histopathology, and immunohistochemistry the diagnosis of hyperpigmented mycosis fungoides (MF) stage IIIA (T4N0M0B0) was established. Skin-oriented therapy (retinoids-PUVA) resulted in slight improvement. Hyperpigmented MF is a rare, uncommon, clinical variant of MF, with a predilection for dark-skinned people (1). Only a few cases of hyperpigmented MF have been reported so far, and our case being one of them (2-5). Hyperpigmented patches or/and plaques dominate the clinical picture. Hyperpigmented MF is characterized by a predominantly CD8+ epidermotropic T-cell phenotype, although different phenotypes have been reported (CD4+ or CD4-CD8-) (2). Histopathologically, interface changes, pigment incontinence and melanophages are usually found in addition to the classical findings of early MF (1). The exact mechanism of hyperpigmentation is not well understood. Hyperpigmented MF had an indolent course in most reported cases, and skin-directed therapy is therefore the treatment of choice. Although MF and its hyperpigmented variant is a lymphoma of low-grade malignancy, large-cell transformation (CD30+) of hyperpigmented MF can occur (1). These rare cases of special clinical MF variants are extremely valuable and can help us investigate and understand the pathophysiology of the disease. Treatment and close follow-up is mandatory in the hyperpigmented variant of MF.

Long-term Follow-up of a Case of Lymphomatoid Papulosis with a Benign Course.

Dear Editor,We present the case of a 40-year old male patient with lymphomatoid papulosis of a waxing and waning course on whom three biopsies were performed during a 14-year period with no change in histopathological or immunophenotypical characteristics. Lymphomatoid papulosis (LP) is a chronic, recurrent, self-healing papulonodular skin eruption with the histopathologic features of a cutaneous T-cell lymphoma but an often benign and indolent clinical course (1). It is designated as a primary, cutaneous, CD30+ lymphoproliferative disorder. The histopathologic features of LP are variable, with five main types (A-E) and several other variants (2). In most cases, LP presents with a generalized eruption of reddish-brown papules or nodules usually smaller than one cm on the trunk and limbs. Rarely, large, rapidly growing nodules may be the first manifestation of the disease (3). Patients with LP have an excellent prognosis even though they are at increased risk of developing secondary cutaneous or nodal lymphomas such as mycosis fungoides, primary cutaneous anaplastic large cell lymphoma (PC-ALCL), or Hodgkin lymphoma (4). LP-associated lymphomas develop in between 10% and, as recently reported, 52% percent of patients and may occur before, concurrent with, or after the onset of LP (4,5). Our patient was diagnosed with "conventional" type An LP 14 years earlier based on the clinicopathologic correlation. The diagnosis was confirmed a year later after excision of a rapid growing ulcerated nodule on the forearm measuring 17 mm in diameter, which was clinically suspected to be anaplastic large cell lymphoma. During these 14 years, there were only a few worrisome recurrences of the disease, which resolved spontaneously or were successfully controlled with local steroids. During a recent exacerbation, when the third biopsy was performed, the patient presented with a large number of generalized reddish-brown pruritic papules and nodules on the trunk, extremities, neck, and face, predominantly up to one cm, some among which were necrotic and excoriated (Figure 1). There were three sites of clustered papules on the trunk, groin, and neck that resembled large, infiltrated plaques larger than two cm, at a glance mimicking cutaneous lymphoma (Figure 1, b, c). There were also older residual hyper- and hypopigmentations on the skin with prominent scarring. Excisional skin biopsy of one larger papule from the abdominal plaque was performed and was not morphologically or immunophenotypically different from the previous ones (Figure 2). Immunohistochemistry showed expression of CD 30 and the phenotypic markers of T-helper lymphocytes (CD 3+/-, CD4+) by neoplastic cells (Figure 2, c, d). Associated systemic malignant lymphoma was excluded based on examination findings, normal laboratory tests, the absence of palpably enlarged lymph nodes, hepatosplenomegaly, and systemic symptoms followed with MSCT. Serology for HIV and EBV was performed and was positive for EBV EBNA, VCA IgG, and IgM, which could be associated with the exacerbation of LP. Topical corticosteroids and phototherapy were administered when needed in this 14-year period, and methotrexate in a lower dose was prescribed during the extensive generalized eruptions. All of the applied therapeutic modalities led to a partial response. LP is a self-limiting disease for which many patients do not require specific treatment. Therapy should be directed at controlling symptoms in generalized eruptions or minimizing the frequency of recurrences, but none of the available treatment options disrupt the natural history of LP or reduce the risk of developing an associated lymphoma (6). Low-dose methotrexate is the initial therapy of choice in patients with the extensive or symptomatic disease or disease involving cosmetically sensitive areas like the face or hands, which were the affected areas in our patient (6,7). There are no markers that can help predict the course of the disease in a given patient, although some indicators have been suggested (8,9). Because of this lack of markers that can help predict the course of the disease and occurrence of malignant lymphoma, patients should remain in monitoring for the rest of their life.

Folliculotropic Mycosis Fungoides Associated with Autoimmune Hepatitis.

Dear Editor, the association between lymphomas and autoimmune manifestations, as well as the prevalence of the cases of coexistent lymphomas and autoimmune conditions, has not been completely established (1-3). Since cutaneous T-cell lymphoma (CTCL) cases are rare, any hypothesis can only be based on case reports or small case series. We present the case of a male patient with folliculotropic mycosis fungoides (FMF) and synchronous autoimmune hepatitis (AIH) with extremely high levels of cancer antigen 19-9 (CA 19-9). The patient was under the supervision of a multidisciplinary team consisting of dermatologists, hepatologists, and hematologists. The patient died 15 months after the diagnoses of FMF and AIH were established and 3.5 years after the first skin changes. Based on our knowledge and search of medical databases, this is the first case of AIH in a patient with CTCL, i.e. with MF. A 53-year-old male patient was admitted to our Dermatology Clinic in September 2014 after being briefly treated with acitretin. During hospitalization, he was diagnosed with FMF, autoimmune hepatitis, and newly developed diabetes mellitus. At the time of hospital admission, about 70 percent of the surface of the skin was affected, infiltrated with numerous cysts on the face, neck, and upper thorax. The patient also presented with alopecia affecting most of the scalp, loss of eyebrows and eyelashes (Figure 1), and complained of intensive itching. The clinical presentation suggested the diagnosis of FMF, which was later confirmed based on histological (Figure 2) and immunohistochemical (Figure 3) findings. The histochemical staining method PAS-Alcian did not reveal mucin deposits. Immunohistochemical findings revealed tumor cells to show aberrant T-immunophenotypes - CD3+, CD2+, CD5-, CD7-, CD4+, CD8-, CD30-. Due to elevated serum conjugated bilirubin and extremely high levels of hepatocellular and cholestatic liver enzymes, the patient was transferred to the Gastroenterology Department. Diagnosis of AIH was established based on the liver biopsy (highly active autoimmune hepatitis) and the exclusion of viral etiology, drug-induced hepatotoxicity, and inherited metabolic disorders of the liver. CA 19-9 level was extremely high (4475.0; RR <37.0 µg/L). In March 2015, CA 19-9 decreased to 365.3. In April 2015, erythroderma and small isolated tumors on the trunk and extremities developed. The patient was treated with RE-PUVA and radio-therapy. In June 2015, due to systemic symptoms, the patient was started on PUVA with IFNα. In November 2015, erythroderma persisted together with larger and ulcerated tumors. The patient was treated at the Hematological Department with two cycles of cyclophosphamide, vincristine, doxorubicin, and methylprednisolone. From March 2015, the patient was continuously treated with ursodeoxycholic acid, prednisolone, azathioprine, analog insulin, and allopurinol. MSCT revealed lymphoma infiltrates in the liver, spleen, and peritoneum (gross tumors). The immunophenotypic analysis of the cells in ascites revealed atypical lymphocytes with convoluted nuclei - LCA+, CD3+, CD20-. The patient died in December 2015 due to sepsis with febrile neutropenia. Before death, he suffered from candidiasis and toxic liver damage due to fluconazole. FMF is an aggressive MF variant with infiltration of lymph nodes, visceral involvement at an earlier stage, and decreased life expectancy (4). Autoimmune hepatitis (AIH) is still an unclear progressive liver disease of unknown etiology which features hypergammaglobulinemia, detectable autoantibodies, and interface hepatitis (5). Being exposed to xenobiotic (acitretin) with consequent liver damage could lead to the formation of self-antigens to which the patient's immune system might have sensitized, and the autoimmune attack continued (6). Slightly elevated CA 19-9 levels in autoimmune hepatitis were reported by other authors (7-9). It should be noted that the liver involvement with atypical lymphocytes can be diffuse without any detectable nodules on a CT scan (4). Soluble liver antigen and liver-pancreas antibodies, together with CA 19-9, need to be implemented as routine diagnostic tools to rationalize the usage of tumor markers in day-to-day practice as well in diagnosis of AIH (10).

Mycobacterium marinum Infection of the Hand in an Immunocompromised Aquarium Hobbyist.

M. marinum, a nontuberculous mycobacterium, is a rare human pathogen widely distributed in the aquatic environment. In the previous century, epidemics took place due to inadequately chlorinated swimming pool water. Nowadays the majority of infections are acquired through contact of previously damaged skin with contaminated fish tank water. We present a case of M. marinum infection of the hand in an aquarium hobbyist which stayed unrecognized for 2 years. After confirming the correct diagnosis, the patient was successfully treated with a regiment containing clarithromycin and rifampicin. The aim of this paper is to raise the awareness of the possibility of M. marinum infection when encountered with non-healing nodular/verrucous/ulcerative lesions of the extremities.

Dermoscopy of Nodular Melanoma: Review of the Literature and Report of 3 Cases.

Nodular melanoma is the most aggressive subtype of melanoma, with rapid growth rate and metastatic potential. It is usually diagnosed at a locally advanced stage (Breslow thickness <2 mm) and is therefore associated with a poor prognosis. Nodular melanoma often does not fit the classic clinical ABCD criteria, but rather the EFG rule or 3 Cs criteria. Missing the diagnosis of nodular melanoma is a dermatologist's worst nightmare, especially since nodular melanomas can have a non-alarming clinical appearance and imitate a wide range of benign lesions. All evolving nodular lesions, despite their size, symmetry, and color, which cannot be confidently diagnosed as benign, should be excised in order to rule out nodular melanoma. Almost all melanoma-specific dermoscopic criteria are described in context of superficial spreading melanoma. Thus, physicians are not familiar and aware enough of dermoscopic features for early detection of nodular melanomas. Herein we present 3 cases of nodular melanomas from our Department and give a review of the current literature.

Advanced Pyoderma Gangrenosum Previously Treated as Squamous Cell Carcinoma.

Pyoderma gangrenosum is a rare, neutrophilic ulcerative skin disease of unknown etiology often associated with an underlying systemic disease. We present a case of a pyoderma gangrenosum that was initially misdiagnosed and treated as squamous cell carcinoma in another hospital. Multiple surgical treatments triggered postoperative exacerbations and further rapid progression of the lesions. History of pathergy, clinical findings, and histopathological features examined at our Department indicated pyoderma gangrenosum. The diagnosis was confirmed by excluding other diseases that could cause similar-appearing cutaneous lesions. No associated underlying disease was determined. After the diagnosis was confirmed, corticosteroid therapy was initiated until complete remission of ulcerations.

Eruptive Syringoma in the Elderly: A Case Report.

Syringomas are benign tumors of adnexal origin, and eruptive syringoma is an extremely rare subtype. In this paper, we present a case of a unusual occurrence of eruptive syringoma in 66-year old woman that includes clinical and pathohistological findings and the review of the clinical picture, diagnosis, and treatment options.

Radiotherapy of primary cutaneous follicle center lymphoma: case report and review of literature.

Primary cutaneous follicle center lymphoma is an indolent primary cutaneous B-cell lymphoma originating from the follicle center cells, composed of a combination of centrocytes (small and large cleaved cells) and centroblasts (large noncleaved cells) with a follicular, follicular/diffuse, or diffuse growth pattern. Lesions are mostly located on the head, neck and trunk. A case is presented of a 56-year-old male patient with primary cutaneous follicle center lymphoma, with lesions involving the skin of the back, shoulders, presternal area and right forearm. As the patient presented a disseminated cutaneous form of the disease that involved several anatomical regions, complete work-up was followed by superficial fractionated radiotherapy of eight fields in VI expositions, with total irradiation dose of 1400 cGy upon the following fields: right and left pectoral region, left and right shoulders, right suprascapular region, and proximal third of the right forearm. Total irradiation dose applied upon each field for the lesions located on the left and right side of the back was 1500 cGy. This therapy resulted in significant reduction of visible tumor. The patient was regularly followed up on outpatient basis for 12 months of radiotherapy, being free from local recurrence and systemic spread of the disease.

Palisaded neutrophilic and granulomatous dermatitis in association with subcutaneous nodular and systemic sarcoidosis.

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare entity that has been clearly defined neither clinically nor histopathologically. PNGD has been associated with some immune-mediated disorders such as rheumatoid arthritis, systemic lupus erythematosus, systemic vasculitis, Behçet's disease, as well as with lymphoproliferative conditions, bacterial endocarditis, sarcoidosis, and various drugs. We present a 44-year-old Caucasian woman with roundish erythematous-livid plaque and erythematous papules on the left calf that were present for three months. Histopathology of plaque lesion showed palisading neutrophilic and granulomatous dermatitis. Subsequently, she developed a firm and tender nodule on the right calf. Histopathology of the nodule showed typical naked sarcoid granulomas in the dermis and subcutis. Additionally, the patient developed non-tender subcutaneous nodules on the cheeks, submandibular region and left breast with normal overlying skin, which were histopathologically diagnosed as sarcoid granuloma. Red eyes and lower visual acuity on the right eye were diagnosed as anterior uveitis. Therefore, systemic sarcoidosis was established. This is a case of PNGD described in an adult patient with sarcoidosis with cutaneous, breast, eye and lung involvement established by clinical, radiographic, laboratory, and histopathologic criteria.