Rapidly Progressive and Debilitating Epithelioid Hemangioendothelioma: An Atypical Presentation of a Rare Malignant Cutaneous Vascular Neoplasm.

ABSTRACT: Epithelioid hemangioendothelioma (EHE) is a rare vascular malignant tumor that comprises less than 1% of all vascular tumors. Cutaneous involvement in EHE can occur either by spreading from underlying bone or rarely could be limited to the skin and mostly presents as solitary well-circumscribed mass to an ill-defined infiltrative lesion. We present a case of rapidly progressive and debilitating EHE presenting multiple vascular papules and nodules. Histopathology showed an ill-circumscribed nodular proliferation of epithelioid and spindled cells in the dermis that extended into the subcutaneous tissue. The tumor cells had moderate eosinophilic cytoplasm, vesicular chromatin, and prominent nucleoli. In addition, they showed evidence of lumen formation and intracytoplasmic vacuoles. Brisk mitosis was noted. On immunohistochemistry, the cells were strongly positive for CD31, CD34, and ERG (ETS [erythroblast transformation-specific]-related gene). MIB-1 labeling index was more than 75% in the highest proliferating areas. A high degree of clinical suspicion and immunopathological examination is recommended for early diagnosis of this rare condition before it becomes function or life-threatening.

Tofacitinib in Pediatric Alopecia Areata Totalis and Alopecia Universalis: A Retrospective Analysis From India.

Alopecia areata totalis and universalis are disabling conditions and therapeutically challenging as they are refractory to conventional options. Tofacitinib is a Janus-kinase (JAK) inhibitor utilized to treat alopecia areata (AA) as an off-label drug. In India, FDA-approved JAK inhibitors such as baricitinib and ritlecitinib are not available. There are only a few case reports on tofacitinib in AA in the Indian population. We present the data of 9 pediatric cases of clinically and histologically proven alopecia areata totalis (AT) and alopecia universalis (AU), for whom oral tofacitinib was given after baseline investigations. The following parameters were analysed: Photographic image and severity of alopecia tool (SALT) score at baseline, 3 months and 6 months, and Children Dermatology Life Quality Index (cDLQI) at baseline and 6 months. The mean ± standard deviation (M ± SD) of the SALT score and cDLQI(M ± SD) at baseline were 95 ± 5 and 17 ± 2. At weeks 4 and weeks 12, the SALT (M ± SD) score was 92.7 ± 6.1 and 34.35 ± 11.16, respectively. At weeks 24, the SALT (M ± SD) score and cDLQI (M ± SD) were 3.33 ± 5 and 6 ± 2. The final reduction in SALT score from the baseline was 100% in 6/9 cases (66.67%), 75% to 99% in 3/9 (22.23%), and 50 to 75% in 1/9 (11.12%). We also observed minimal adverse effects (one child developed herpes zoster) with tofacitinib. Our study demonstrates that oral tofacitinib represents a viable modality in managing difficult-to-treat pediatric AA, such as AT and AU, with a good safety profile.

A rare diagnosis of pulmonary actinomycosis by bronchoalveolar lavage cytology.

The role of a bronchoalveolar lavage sample cell block in the diagnosis of pulmonary actinomycosis.

Lethal Neurotoxicity in Lambda-Cyhalothrin Poisoning: A Rare Case Report.

ABSTRACT: Agricultural poisons (insecticides and pesticides) are the most common types of poison implicated in the morbidity and mortality associated with acute poisoning. Suicidal ingestion is more frequent than accidental or homicidal poisonings. Pyrethroids are considered relatively safer than other insecticides. Lambda-cyhalothrin (LCH) belongs to the fourth-generation, type II synthetic pyrethroid. To the best of our knowledge, fatalities after LCH exposure have not yet been reported in the literature. Here, we describe a case of LCH poisoning in a 54-year-old male farmer after an accidental pipe burst in a sprayer while spraying in the field. The patient died 10 days after poisoning due to severe neurotoxicity resulting in bilateral parieto-occipital and brainstem infarcts. The histopathological features of the brain associated with LCH poisoning have been discussed in this report.

Cytology of angiofibroma of soft tissue of the inguinal region.

Angiofibroma of the soft tissue is a recently described benign fibroblastic/myofibroblastic tumour. This report describes the cytology of an angiofibroma of soft tissue occurring in a 30-year-old lady which showed bland spindle cells, occasional polygonal cells with nuclear grooving, prominent vessels, frayed stroma around the blood vessels, and scattered lymphocyte-rich inflammatory cells in the background.

T-Regulatory Cells in Erythema Nodosum Leprosum: An Immunohistochemical and Image Morphometric Study.

ABSTRACT: Erythema nodosum leprosum (ENL) occurs as an immune-inflammatory complication of multibacillary leprosy (MBL), precipitated by an interaction between the host, bacilli, and the environment. This complication often causes significant morbidity due to systemic involvement and needs to be treated aggressively. T-regulatory cells (T-regs) are the immunomodulatory subset of T cells that are hypothesized to play a role in ENL. We have performed immunohistochemistry for FoxP3 (T-reg), CD3 (pan-T), CD4 (helper T), and CD8 (cytotoxic T) on 50 biopsy-proven cases of ENL along with 84 biopsy-proven cases of paucibacillary leprosy (PBL) (n = 49) and MBL (n = 35). Image morphometry was applied to objectively assess the relative preponderance of these subsets of T cells. The area fraction of T-regs showed a trend of reduction from PBL to MBL to ENL (P = 0.068), whereas the FoxP3:CD3 (T-reg: pan-T) ratio showed a significant reduction across these groups (P = 0.023). However, there was no significant difference of T-regs or FoxP3:CD3 ratio between MBL and ENL. The T-regs showed a significant positive correlation (P = 0.007) with the cytotoxic T cells in the skin biopsy. The presence of dermal eosinophils in ENL showed a trend association with the FoxP3:CD3 ratio (P = 0.05). Various histopathological parameters including epidermal spongiosis, dermal stromal edema, dermal ill-formed granuloma, and the presence of bacilli within the endothelium and vascular smooth muscle correlated with various T-cell subsets. Our study, one of the largest on this topic, objectively assessed the role of T-regs in the spectrum of leprosy. Nevertheless, the precipitation of ENL from MBL is probably not associated with the T-reg subset alone.

Image Morphometric Analysis of B Cells and Plasma Cells in Erythema Nodosum Leprosum With Clinicopathological Correlation.

ABSTRACT: Erythema nodosum leprosum (ENL) occurs as an immunological complication of multibacillary leprosy (MBL). The pathogenesis of ENL is long considered to be a T-cell-mediated process. The role of B cells and plasma cells in ENL is not well described in the literature. Therefore, we investigated the B-cell and plasma cell infiltrates in the skin biopsies of biopsy-proven cases of ENL by immunohistochemistry and image morphometry and compared the result with paucibacillary leprosy and MBL. Moreover, we sought a correlation of the B-cell and plasma cell infiltrates with different clinical, hematological, histopathological, and bacteriological parameters as well as the T-cell subsets in the skin biopsies. Our study highlighted a significant reduction in the number of B cells from paucibacillary leprosy to MBL to ENL, although there was no significant variation in the plasma cell infiltrate. The plasma cell infiltrate correlated with absolute neutrophilia in the blood and the presence of eosinophils in the ENL lesions. Both B cells and plasma cells positively correlated with CD4-positive T-helper cells and the CD8-positive cytotoxic T cells. Besides, the B cells also correlated positively with the CD3-positive pan T cells in the biopsy and negatively correlated with the T-regulatory:T-cell ratio. Our results suggested the role of B cells and plasma cells even at the tissue level in the pathobiogenesis of ENL.

Dermoscopic features of acute cutaneous lupus erythematosus: A retrospective analysis from a tertiary care centre of East India.

BACKGROUND: The cutaneous features of acute cutaneous lupus erythematosus (ACLE) can be confused with other dermatoses. The dermoscopic features of ACLE have rarely been reported. AIMS: We aimed to study the dermoscopic features of ACLE, malar rash, and generalized ACLE. MATERIALS AND METHODS: The dermoscopic features of all biopsy and direct immunofluorescence confirmed ACLE cases were analysed retrospectively between May 2019 and May 2020. RESULTS: A total of 21 patients with skin phototypes III, IV, or V were included in the analysis. A pinkish-white to reddish-white homogenous area (94.1%), brown dots, globules and peppering (76.4%), and keratotic follicular plugging (64.7%) were the common dermoscopic features observed in the case of generalized ACLE. Similarly, in the case of malar rash, a pinkish-white to reddish-white homogenous area (77.8%) was the most common dermoscopic feature, followed by keratotic follicular plugging (66.7%), scales (44.5%), and brown dots, globules, and peppering (44.5%). In both variants, vascular structures were dominated by linear vessels with or without branching. CONCLUSIONS: A multicomponent pattern comprising white scales, homogenous pinkish-white to the reddish-white area, brown dots/globules/peppering, keratotic follicular plugging, and linear vessels with or without branching were the common dermoscopic features observed in our study and may indicate the diagnosis of both localized and generalized ACLE. In addition, a paucity of dotted vessels may serve as an additional clue.

Actinic keratoses and squamous cell carcinoma limited to photo-exposed skin in vitiligo vulgaris in pigmented skin type.

Vitiligo skin has a lesser number of photoprotective melanocytes-theoretically, there is a higher risk of development of non-melanoma skin cancers in such patients. But most studies in Caucasian patients have shown decreased incidence of non-melanoma skin cancers in patients with vitiligo. In Indian patients, there is a paucity of literature on such adverse events. We report a case of actinic keratoses, cutaneous horn with dysplasia and squamous cell carcinoma developing exclusively over photo-exposed vitiligo lesions in an Indian woman in her 60s (housewife, Fitzpatrick skin type V and average daily photo-exposure time 2-4 hours) of long-standing vitiligo vulgaris without any history of phototherapy. The photoprotected lesional skin was completely normal with no clinically appreciable enlarged regional lymph nodes. Shave and elliptical excision of the suspicious lesions were done, and histopathology showed various degrees of malignant transformation in various lesions. The patient was started on topical imiquimod for the lesions of actinic keratoses and was referred for staging and wide excision of squamous cell carcinoma lesion. We report this case for its rarity and to emphasise the fact that there is a need for counselling for lifestyle modification in patients with vitiligo as the use of sunscreens is often not practised by Indian patients due to financial constraints and physical measures such as using full sleeves, high-collared dresses and scarves should be encouraged.