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.

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.

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.

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.

Dermoscopy of Cutaneous Neoplasms in Skin of Color - A Systematic review by the International Dermoscopy Society "Imaging in Skin of Color" Task Force.

Over the last few decades, dermoscopy has been showed to facilitate the non-invasive diagnosis of both benign and malignant skin tumors, yet literature data mainly comes from studies on light photo-types. However, there is growing evidence that skin neoplasms may benefit from dermoscopic assessment even for skin of color. This systematic literature review evaluated published data in dark-skinned patients (dermoscopic features, used setting, pathological correlation, and level of evidence of studies), also providing a standardized and homogeneous terminology for reported dermoscopic findings. A total of 20 articles describing 46 different tumors (four melanocytic neoplasms, eight keratinocytic tumors, 15 adnexal cutaneous neoplasms, seven vascular tumors, four connective tissue tumors, and eight cystic neoplasms/others) for a total of 1724 instances were included in the analysis. Most of them showed a level of evidence of V (12 single case reports and six case series), with only two studies featuring a level of evidence of IV (case-control analysis). Additionally, this review also underlined that some neoplasms and phototypes are underrepresented in published analyses as they included only small samples and mainly certain tones of "dark skin" spectrum (especially phototype IV). Therefore, further studies considering such limitations are required for a better characterization.

Dermoscopy of Inflammatory Dermatoses (Inflammoscopy) in Skin of Color - A Systematic Review by the International Dermoscopy Society "Imaging in Skin of Color" Task Force.

Dermoscopic patterns of inflammatory dermatoses (inflammoscopy) have been extensively studied in the recent years, though data on patients with darker phototypes (IV-VI) are sparse. The aims of this systematic review were to summarize the current state of knowledge on inflammoscopy applied to skin of color and provide a standardized nomenclature of reported findings. Besides dermoscopic features, type of setting and magnification, number of cases, and histopathological correlation were analyzed. Eighty-five papers addressing 78 different dermatoses (25 papulosquamous dermatoses, 19 hyperpigmented dermatoses, eight hypopigmented dermatoses, four granulomatous dermatoses, two sclerotic dermatoses, five facial inflammatory dermatoses, and 15 miscellaneous conditions) for a total of 2073 instances were retrieved. Only one study showed a level of evidence of III (cross-sectional study), whereas 10 and 74 displayed a level of evidence of IV (case-control studies) and V (case-series and case-reports), respectively. Moreover, our analysis also highlighted that most of papers focalized on a limited number of dermatoses, with several conditions having only single dermoscopic descriptions. Additionally, few studies compared findings among phototypes belonging to the "skin of color" spectrum. Further studies designed according to a systematic approach and considering the above-mentioned issues are therefore needed.

International Dermoscopy Society (IDS) Criteria for Skin Tumors: Validation for Skin of Color Through a Delphi Expert Consensus by the "Imaging in Skin of Color" IDS Task Force.

INTRODUCTION: A structured set of eight basic dermoscopic parameters (lines, clods, dots, circles, pseudopods, structureless, else, and vessels) including a total of 77 variables with corresponding descriptive and metaphoric vocabulary has been released for evaluation of skin tumors by the International Dermoscopy Society (IDS). OBJECTIVES: To validate the aforementioned criteria for the use in darker phototypes (phototypes IV-VI) via an expert consensus. METHODS: The two-round "Delphi method" was adopted, with an iterative process including two rounds of email questionnaires. Potential panelists were asked to take part in the procedure via email on the basis of their expertise in the dermoscopy of skin tumors in dark phototypes. RESULTS: A total of 17 participants were involved. All the original variables of the eight basic parameters reached agreement during the first round, except for "pink small clods" ("milky red globules") and "structureless pink zone" ("milky red areas"). Moreover, during the first round, panelists proposed a change of three existing items and the introduction of four new items, i.e., "black, small clods" ("black globules"), "follicular plugs", "erosions/ulcerations", and "white color around vessels" ("perivascular white halo"). All such proposals achieved agreement, thus being included in the final list, for a total of 79 items. There was consistency between the descriptive and metaphoric approaches in terms of scoring. CONCLUSIONS: Albeit most of the original items were considered applicable even for skin of color, there are some points of differences that physicians need to know. No significant preference was found between descriptive and metaphoric terminology among panelists.

Clinicodermoscopic and immunopathological profile of non-infectious non-eczematous inflammatory tattoo reactions: A retrospective study from a tertiary care centre of East India.

Introduction Tattoo-associated complications are on the rise due to the popularity of decorative tattoos in recent years. The exact pathogeneses of various tattoo reaction patterns are still unclear, and their dermoscopic details are sparsely reported. Aim We aimed to retrospectively study the clinical, dermoscopic and immunopathological details of patients with non-infectious, non-eczematous inflammatory tattoo reaction patterns in a tertiary care centre of East India. Method The clinical, dermoscopic and pathological details of all the patients who had non-infectious, non-eczematous inflammatory tattoo reactions were collected. In all the cases, immunohistochemistry was done for CD1a, CD3, CD4, CD8, FoxP3, CD20 and CD56. Results A total of five patients of skin phototypes IV and V and six tattoo reactions were analysed. Five lesions had reactions at the site of a black tattoo, and one at the site of red tattoo. Clinically, the patients presented with erythematous or blue-grey flat-topped to verrucous papules and plaques. Dermoscopic features were dominated by a central white to pink-white structureless area, a peripheral grey-white to bluish-white structureless area, white scales, comedo-like opening with keratotic plugging, milia-like cysts and shiny white structures. Pathologically, except for one lesion that only showed a lichenoid reaction pattern in the red tattoo, all had a combination of reaction patterns. Immunohistochemistry showed increased epidermal and dermal Langerhans cells, predominantly CD8 positive T cells in the epidermis and dermis, sparse dermal B cells and CD4 positive T cells, reduced T regulatory cells and a complete absence of CD56 positive NK cells. Limitations Small sample size was the limitation of the study. Conclusion The clinical morphology and dermoscopy may not differentiate between various types of non-infectious non-eczematous inflammatory tattoo reactions. The immunological profile supports a delayed hypersensitivity reaction due to contact sensitisation to tattoo pigment, and CD8 positive T cells play a central role in executing various pathological reaction patterns, both in the epidermis and dermis.

Dermoscopic features of basal cell carcinoma in skin of color: A retrospective cross-sectional study from Puducherry, South India.

BACKGROUND: Dermoscopy is useful in the diagnosis of basal cell carcinoma (BCC). However, most descriptions of the dermoscopic features of BCCs are in Caucasians (skin types I-III) and there is a paucity of data in dark-skinned Indian patients. AIMS: The aim of this study was to describe the various dermoscopic features of BCC in dark-skinned patients from South India and correlate these with the histopathologic subtypes. METHODS: A retrospective observational study of biopsy-proven cases of BCC was conducted at a tertiary care center in South India using nonpolarized contact dermoscopy. RESULTS: Sixty BCCs in 35 patients predominantly of skin phototypes IV or V were studied. These included 32 nodular, 27 superficial and 1 infiltrative type of BCC. The most common dermoscopic features noted were maple leaf-like areas (61.7%), blue-white veils (53.4%), ulceration (48.4%) and short fine telangiectases (46.7%). Ulceration, blue-white veils and arborizing vessels were significantly associated with nodular BCCs, while maple leaf-like areas, red-white structureless areas, multiple small erosions and spoke wheel areas were noted with superficial BCCs. LIMITATIONS: The limitations of this study include its retrospective nature, the use of only nonpolarized light for examination, the lack of other histopathological variants of BCC as well as the lack of a comparison group. CONCLUSION: We report a dermoscopic study of BCC in dark-skinned patients from Puducherry, South India. The blue-white veil was observed in half of the patients and was significantly associated with nodular BCCs. The addition of the blue-white veil to the diagnostic criteria for pigmented BCC could improve the diagnostic accuracy of dermoscopy in Indian patients.

Clinico-Dermoscopic-Pathological Features of a Rare Case of Locally Invasive Multifocal Primary Cutaneous Diffuse Large B Cell Lymphoma-Leg Type Over the Face and Scalp.

Primary cutaneous diffuse large B-cell lymphoma-leg type (PCDLBCL-LT) is characterized by diffuse monotonous proliferation of centroblasts and immunoblasts. It commonly presents as erythematous to violaceous nodules on one or both the legs and has a poor prognosis. We report the clinico-dermoscopic-pathological features and therapeutic response of a rare case of PCDLBCL-LT in a 62-year-old diabetic man, who presented with multifocal plaques, one lobulated and two arcuate-shaped, on the face and scalp. During the investigation, one of the plaques had eroded the underlying bone without any evidence of malignant cells in the cerebrospinal fluid. He was successfully treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) along with intrathecal methotrexate.