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Context: Sarcoidosis is a systemic disorder characterized histologically by noncaseating granulomas. There is paucity of Indian data on cutaneous sarcoidosis. Aims: To describe the clinical, histopathological findings, and extracutaneous involvement in cutaneous sarcoidosis. Materials and Methods: A retrospective study was done in patients of cutaneous sarcoidosis who had attended the dermatology clinic of a tertiary health care center in India from May 2009 to April 2015. The clinical details, histopathological findings, treatment, and response were reviewed. Results: There were 38 patients with cutaneous sarcoidosis. Mean age was 48 ± 13 years; 58% were female. Median duration of disease was 11 months (IQR 4–48 months). More than one morphology was seen in 28.9%, commonest being plaques (65.7%), and papules (50%). Erythema nodosum was rare. More than one site was involved in 55.3%, most commonly trunk (52.6%). Six patients had isolated cutaneous sarcoidosis. Commonest extracutaneous organs involved were lung (73.7%) and lymph nodes (68.4%). Histopathologically, classical naked sarcoidal granulomas were found in only 55.3%. Angiotensin converting enzyme (ACE) levels were elevated in 74.3% (26/35) with significant association with extracutaneous disease. Treatment included topical and/or systemic corticosteroids, hydroxychloroquine, and tacrolimus. Statistics: Pearson's Chi-square test was done to analyze associations between the skin lesions, ACE levels, and systemic involvement; P < 0.05 was considered significant. Conclusions: Cutaneous manifestations of sarcoidosis are varied, commonest being erythematous plaques. Even though most patients had systemic involvement, we found no significant association of the type and number of skin lesions with extracutaneous involvement or prognosis. Elevated ACE levels were significantly associated with systemic involvement.

Background: Paraneoplastic autoimmune multiorgan syndrome (PAMS), first described as paraneoplastic pemphigus (PNP) is a heterogeneous autoimmune syndrome with a diverse spectrum of clinical and immunopathological features associated with an internal neoplasm. Materials and Methods: The details of the patients diagnosed with PAMS/PNP from an Indian tertiary center between January 2010 to December 2019 were retrieved from the hospital database. The clinical manifestations, histopathological features, immunofluorescence findings, and other relevant clinical details were obtained. Results: There were eight patients (4 males, 4 females) with PAMS, age ranging from 8 to 46 years (mean 31 years), of whom two were 8-year-old children. The mucocutaneous manifestations were polymorphic and all had recalcitrant oral mucosal involvement. The most common mucosal presentation was pemphigus-like (5/8), and the cutaneous presentation was lichen planus-like (5/8). Castleman's disease (5/8) was the commonest neoplasm followed by thymoma (2/8). Interface dermatitis was seen in all biopsies and three different patterns of direct immunofluorescence were seen, which were intercellular “fish-net” fluorescence in the epidermis (2/8), granular/linear deposition along the basement membrane (4/8) and a combination of both patterns (1/8). Indirect immunofluorescence done on rat bladder in 3 patients showed intercellular “fish-net” fluorescence. Desmoglein levels were not elevated in any of our patients. The follow-up period ranged from 1 to 112.5 months (mean, 23.6 months) with a mortality rate of 12.5%. Conclusion: In our study, Castleman's disease was the most common associated malignancy, and the mucocutaneous and histopathological findings were heterogeneous. Timely diagnosis and early intervention improved the outcome in our patients.

Context: Lymphomatoid granulomatosis (LYG) is a rare B-lymphoproliferative disorder characterised by an angiocentric and angiodestructive pattern along with Epstein - Barr virus (EBV) association. It is one of the diagnostic challenges in lymphoma pathology. Deregulation of EBV immune surveillance is one of the narrated hypotheses in the literature. Extrapulmonary manifestations are rare with LYG. Morphological grading is done based on the number of EBV-positive B cells, which is useful to strategize treatment protocol. Aims: We report here a series of nine cases of LYG to discuss the clinical, histological, and immunohistochemistry findings. Settings and Design: This is the first case series from India in published literature. Subjects and Methods: We reviewed cases of LYG diagnosed at our center for the past 11 years (2006-2016). A total of nine cases were included in this study. Histomorphology was studied in conjunction with immunohistochemistry and clinical details. Cases without classical morphology and negative for EBV immunostain were excluded from the study. Results: There were nine patients in our study (7 males and 2 female; M:F ratio 3.5:1). The age of these patients ranged from 4 years to 57 years (mean age: 30 years). The most common site involved was the lung (4, 44%), followed by the skin (2, 22%), central nervous system (2, 22%) and lymph node (1, 11%). One patient had primary immunodeficiency. Another patient had undergone renal transplant 11 years before the development of the lesion. Angiocentricity and angioinvasion were appreciated in all nine cases (9/9) with necrosis in four cases (44%) and ill-defined histiocytic aggregates in three cases (33%). The histological features were as follows: Grade 1(4 cases, 44%), Grade 2(2 cases, 22%), and Grade 3(3 cases, 33%). Conclusion: LYG is a rare EBV driven angiodestructive disease with predominantly lung involvement as well as isolated extrapulmonary sites as seen in our study. It is often progressive and ultimately fatal in the absence of appropriate treatment. Grading of the lesion helps to initiate the appropriate treatment of choice.

Acquired cutis laxa (ACL) is a rare connective tissue disorder characterized by pendulous and coarsely wrinkled skin. There have been few cases of its association to monoclonal immunoglobulin deposition disease (MIDD), which constitutes the light chain (LCDD), heavy chain (HCDD), and light and heavy chain (LHCDD) deposition disease. MIDD predominantly involves the kidney. Skin is the next common organ to be affected by HCDD, which presents as ACL. We report the case of a 40-year-old male who presented with ACL associated with LHCDD. The clinical features of ACL in the present case appeared prior to the development of clinical features related to LHCDD.