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.

Linear indurated plaque over the neck.

Isolated cutaneous swelling can have varied etiologies. The clinical diagnosis is usually difficult, and a correct diagnosis always requires a pathological examination. Hereby, we report a case of linear keloidal morphea on the neck of an 18-year-old male who presented with an asymptomatic, firm lesion for 6 months. Histopathological examination was consistent with morphea. This case highlights the uncommon form of morphea in an unusual location, which can be misdiagnosed for numerous neoplastic conditions and for which simple histopathological evaluation can clinch the diagnosis.

Trichoscopic Features of Scalp Discoid Lupus Erythematosus versus Lichen Planopilaris: A Systematic Review.

Introduction: Lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) are primary scarring alopecias that pose diagnostic challenges clinically, where trichoscopy features may provide benefit in delineating these two cicatricial alopecia, and also helps in assessing the evolution and therapeutic response. To date, there are few reviews on dermoscopic findings in differentiating these two alopecias. Methods: A systematic literature review was conducted using the PubMed and Google Scholar databases. The search terms included for scalp DLE were 'lupus' OR 'discoid lupus' OR "scalp lupus" and for scalp LPP were "lichen planopilaris" OR "scalp follicular lichen planus" OR "lichen planus follicularis" and were combined with "dermoscopy" OR "dermatoscopy" OR "videodermoscopy" OR "video dermatoscopy" OR "trichoscopy". The differences in the prevalence of dermoscopic features in scalp DLE and LPP were calculated using the Chi-square test. Results: Of 52 articles, 36 (17 LPP, 19 DLE) were eligible for quantitative analysis. We found predominant peripilar tubular casts and perifollicular erythema with the presence of arborizing vessels in the vicinity of these changes, indicating early LPP. In contrast, follicular red dots, speckled brown pigmentation, and hair diameter variability indicated active DLE. Shiny white areas were common in both the groups in late stages. The target pattern of distribution of blue-grey dots, milky red areas, and irregular white fibrotic dots were seen in LPP, and pink-white background, follicular plugs, perifollicular and interfollicular scale, rosettes, chrysalides, and red spider on yellow dots were detected in DLE. Features such as yellow dots and blue-grey structureless areas were nonspecific and did not have a major role in differentiating DLE from LPP. Conclusion: This article provides a comprehensive review of the literature and delineates the trichoscopic differences and peculiarities of scalp DLE and LPP, including the correlation of dermoscopic features with histopathological findings.

Giant cell collagenoma: Report of a rare tumor and review of literature.

ABSTRACT: Giant cell collagenoma (GCC) is a rare benign dermal fibrous tumor. Although it has many clinicopathological differential diagnoses, it is often confused with cutaneous sclerotic fibroma/storiform collagenoma (SF/SC) and dermatofibroma. The following characteristic features point to GCC's diagnosis over the latter: the presence of peculiar multinucleated giant cells, and vimentin positivity of both single and multinucleated giant cells on immunohistochemistry. Most of the reported cases have mentioned that GCC is a variant of SF/SC. We report a rare case of GCC presenting as a slow-growing solitary firm nodule over the right ankle. To the best of our knowledge, only less than ten cases have been reported, including the index case. We have also reviewed the clinicopathological features of those cases and discussed the approach to the diagnosis.

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.

Xeroderma pigmentosum group G with pellagroid rash: A rare presentation.

Xeroderma pigmentosum (XP), a heterogeneous genodermatoses, has a variable clinical spectrum ranging from mild freckling and photosensitivity to severe skeletal and neurological abnormalities and cutaneous malignancies. Herein, we present the case of a 4-year-old boy with XP group G who presented with a pellagroid rash.