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.

Hobnail subtype of papillary carcinoma of the thyroid-a rare case with uncommon features.

The cytological features of the hobnail variant of papillary thyroid carcinoma may be subtle. It is important to recognize this variant because it may influence the corresponding surgical treatment and follow-up due to its aggressive nature. The hobnail subtype of papillary thyroid carcinoma is a rare entity with aggressive features. It presents extrathyroidal extension or lymph nodal metastasis in a high percentage of the cases.

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.

Neonatal autopsy-is it relevant in today's era?

Autopsy of infants can provide vital information about the cause of death and contributes to the detection of diagnostic errors, especially in a low- or middle-income country. To observe the clinicopathological agreement in neonatal deaths in neonatal intensive care units (NICU) and comment on the additional information retrieved by autopsy. A retrospective observational study was conducted in the NICU from January 2020 to December 2022. Neonatal deaths were analyzed, and clinical details and autopsy findings were collected. Both clinical and pathological diagnoses were classified according to the Goldman classification. Twenty-two newborn infants were enrolled. The mean gestational age was 33.5 (±4.38) weeks, and the median birth weight was 1510 (1005-2100) g. There was complete concordance between clinical and pathological diagnosis in 11 (50%) cases. Major diagnostic errors occurred in 41% of cases. Respiratory system disorders (lung infections, airway anomalies) accounted for six (54%) cases of missed diagnosis. Our study showed that the diagnosis was revised after autopsy in about one-third of cases, and newer findings were identified in one-fifth of cases.

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.

Expression of CD56 is Not Limited to Biliary Atresia and Correlates with the Degree of Fibrosis in Pediatric Cholestatic Diseases.

BACKGROUNDS AND AIMS: CD56 immunostain is used as an adjunct to aid in the preoperative diagnosis of biliary atresia (BA) by liver biopsy. We aimed to study the expression of CD56 in different pediatric cholestatic diseases thereby evaluating its utility in the diagnosis of BA. METHODS: We performed immunohistochemistry for CD56 on 35 cases of pediatric cholestatic diseases and five age-matched controls. CD56 expression was assessed by a multiplication score (percentage positivity x intensity) in the biliary epithelium. RESULTS: The multiplication score between BA and choledochal cyst was not significantly different. High scores were also encountered in other cholestatic disorders. The score showed a significant negative association with serum albumin and a significant positive correlation with the serum ALT level. Very significant positive correlation between the score and portal fibrosis was obtained. CONCLUSION: CD56 expression is an infidel marker for the histological diagnosis of BA and rather provides a clue to the disease status in pediatric cholestatic diseases.

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.

The Presence of Pericholedochal Hyaline Cartilage in Biliary Atresia: A Report and A Review.

Background: The presence of cartilage in extra hepatic biliary tree is an unusual finding. An isolated presence of the cartilage is possibly heterotopic or occurs as a metaplastic response to the inflammatory insult.Material and methods: We had examined the liver biopsy and the resected specimen of a biliary atresia (BA) after Kasai procedure.Results: There was hyaline cartilage around the common hepatic and common bile duct in a 3-months-old male infant with distal obstructive cholangiopathy on liver biopsy and had positive serum IgM for cytomegalovirus (CMV). Similar findings could not be documented in the pericholedochal tissue of any of the 25 other pediatric cases operated for BA or choledochal cyst and three neonatal autopsies performed for liver-related deaths.Conclusion: Peri-bile duct cartilage is a unique finding and could represent an unusual form of heterotopia or connective tissue metaplasia.