Generalized morphea following the COVID vaccine: A series of two patients and a bibliographic review.

The appearance of morphea after vaccination has been reported to date as single and deep lesions that appear exactly at the site of the skin puncture. It was therefore postulated that the origin could be the trauma related to the injection. The aim of this article is to review the various hypotheses offered in the published literature about generalized morphea following vaccination. We present two cases of generalized morphea after COVID-19 vaccination and review the published literature on immune-related cutaneous reactions. As previously reported, antigenic cross-reactivity between vaccine spike proteins and human tissues could cause certain immune-mediated diseases, including generalized morphea. Herein we report two cases of generalized morphea probably induced by the COVID-19 vaccine, given the temporal relationship with its administration. In summary, environmental factors such as vaccination against SARS-COV-2 could induce an immune system dysregulation, which would have an important role in the pathogenesis of morphea. We present two cases of generalized morphea probably induced by the COVID-19 vaccine, given the time elapsed between vaccination and the onset of the skin lesions.

A case of unilateral linear capillaritis.

Unilateral linear capillaritis is a rare variant of the pigmented purpuric dermatoses that can be misdiagnosed due to confusion with other cutaneous diseases that follow a linear distribution. We present the case of an 8-year-old boy with hyperpigmented patches linearly distributed on the right arm, initially diagnosed with segmental neurofibromatosis.

Facial solitary morphea profunda presenting with painful trigeminal neuropathy: A case report.

BACKGROUND: Localized facial scleroderma usually presents as frontal linear morphea or progressive hemifacial atrophy. Only isolated cases of trigeminal painful neuropathy have been described. CASE REPORT: A 43-year-old woman developed an oval lesion on the right cheek. After 1 year, she noticed constant "pulling" pain and episodes of lancinating pain, both spontaneous and triggered by chewing and cold drinks. She was diagnosed with solitary morphea profunda and CT scan, ultrasonography, cranial MRI and biopsy were completed. Methylprednisolone (1 gr/day for 3 days) was prescribed. For pain, gabapentin, oxcarbazepine, amitryptiline, pregabalin and eslicarbacepine were all ineffective. A capsaicin patch was placed with prolonged benefit. Later on, the pain slightly worsened; occipital blockade was effective and methotrexate was recommended. CONCLUSION: This is the first case of solitary morphea profunda associated with painful trigeminal neuropathy. Treatment should include immunosuppressants and treatment of neuropathic pain, in which local therapies seem particularly beneficial.

Recalcitrant granulomatous periorificial dermatitis with good response to low-dose oral isotretinoin.

Granulomatous periorificial dermatitis is a clinical variant of periorificial dermatitis. We present the case of an 18-year-old girl with several reddish papular lesions in the perioral, perinasal, and periorbital regions unresponsive to conventional therapy. After 6 months of therapy with low-dose oral isotretinoin, the lesions fully remitted.

Ocular involvement in pemphigus vulgaris - a retrospective study of a large Spanish cohort.

BACKGROUND AND OBJECTIVES: Ocular/periocular involvement in pemphigus vulgaris (OPV) has rarely been reported. The objective of the present study was to investigate the pattern of OPV and define the prognostic value of its manifestation. PATIENTS AND METHODS: From 1985 to 2014, a total of 167 patients with pemphigus vulgaris (PV) were treated at four tertiary Spanish hospitals. In this retrospective study, we included all patients with OPV. Clinical data and information on associated symptoms were obtained from patients' medical records. RESULTS: Only 24 (14.3 %) of all PV patients had ocular lesions. In most cases, -ocular involvement was preceded by PV lesions at various other sites (mean duration: 33.7 months). Ocular PV lesions occurred during flares of mucocutaneous pemphigus, and was never the only mucosal manifestation. The most common clinical signs were conjunctival hyperemia (87.5 %), erosions on the eyelids (41.6 %) as well as of the palpebral/bulbar conjunctiva (33.3 %) and at the medial epicanthus (20.8 %). The most relevant associated symptoms included local pain/stinging (71.4 %), irritation (47.6 %), photophobia (38.1 %), and epiphora (23.9 %). Ocular PV improved with systemic and adjuvant topical therapies. Only two patients experienced sequelae. CONCLUSIONS: In patients with PV, ocular involvement is an exception. Ocular PV is associated with greater disease activity, and usually follows a benign course. Sites affected are the conjunctiva, the eyelids, or both.

Augenbeteiligung beim Pemphigus vulgaris - retrospektive Studie an einer großen spanischen Kohorte.

HINTERGRUND UND ZIELE: Es gibt nur wenige Berichte zur Beteiligung der okulären/periokulären Region beim Pemphigus vulgaris (PV). Ziel der vorliegenden Studie war es, das Krankheitsbild des okulären PV (OPV) zu untersuchen und seinen prognostischen Wert zu definieren. PATIENTEN UND METHODIK: Zwischen 1985 und 2014 wurden insgesamt 167 Patienten mit Pemphigus vulgaris an vier tertiären spanischen Krankenhäusern behandelt. Wir haben alle Patienten mit OPV in diese retrospektive Studie aufgenommen. Klinische Daten sowie Informationen zu Begleitsymptomen wurden den Krankenakten der -Patienten entnommen. ERGEBNISSE: Lediglich 24 (14.3 %) PV-Patienten hatten okuläre Läsionen. Meist gingen dem okulären Befall PV-Läsionen an verschiedenen anderen Stellen voraus (durchschnittliche Dauer: 33,7 Monate). Okuläre PV-Läsionen traten während der Schübe eines mukokutanen Pemphigus auf und waren niemals die einzige Mukosa-Manifestation. Die häufigsten klinischen Symptome waren konjunktivale Hyperämie (87,5 %) und Erosionen an den Augenlidern (41,6 %), sowohl an der palpebralen/bulbären Konjunktiva (33,3 %) als auch am medialen Epikanthus (20,8 %). Zu den wichtigsten Begleitsymptomen gehörten lokale Schmerzen/Brennen (71,4 %), Reizung (47,6 %), Photophobie (38,1 %) und Epiphora (23,9 %). Der OPV besserte sich unter systemischer und unterstützender topischer Behandlung. Lediglich bei zwei Patienten traten Spätfolgen auf. SCHLUSSFOLGERUNGEN: Bei Patienten mit PV sind die Augen nur in Ausnahmefällen betroffen. Ein okulärer PV ist mit größerer Krankheitsaktivität assoziiert und hat in der Regel einen gutartigen Verlauf. Betroffen sind die Konjunktiva und/oder die Augenlider.

Association between adipophilin expression and risk of dyslipidaemia in patients with granuloma annulare.

BACKGROUND: An association between granuloma annulare (GA) and dyslipidaemia has been reported. Adipophilin expression may play a plausible role as a cutaneous biomarker for dyslipidaemia in patients with GA; however, this potential link remains to be explored. METHODS: Patients with GA were identified at our hospital between January 1, 1990, and December 31, 2021, with a thorough review of their clinical and histological characteristics. Adipophilin staining was assessed in biopsies of GA lesions. RESULTS: A total of 107 patients with GA were included. The prevalence of dyslipidaemia in patients with positive adipophilin staining was clearly higher than in those with negative labelling (62.3% vs 13.3%). Relative to the dyslipidaemia risk for patients with negative adipophilin expression, the odds for patients with positive adipophilin expression were increased 10-fold (OR: 10.8; p-value<.01). We identified 23 incident cases of dyslipidaemia over a median follow-up period of 91 months among 54 patients with no history of dyslipidaemia. The patients with positive adipophilin expression showed a higher risk of developing dyslipidaemia (HR: 8.9; p-value<.01). CONCLUSIONS: Patients with positive adipophilin staining in their GA biopsies were found to be associated with a higher risk for both baseline and incident dyslipidaemia.

Neural nitric oxide synthase participates in pemphigus vulgaris acantholysis through upregulation of Rous sarcoma, mammalian target of rapamycin and focal adhesion kinase.

Pemphigus vulgaris (PV) is an autoimmune blistering skin disease characterized by suprabasal acantholysis produced as a consequence of desmoglein (Dsg) and non-Dsg autoantibodies binding to several targeting molecules localized on the membrane of keratinocytes. Nitric oxide (NO) may exert a pathogenic function in several immunological processes. We have previously demonstrated that neural nitric oxide synthase (nNOS) plays part in PV acantholysis. Also, our group has described a relevant role for HER [human epidermal growth factor receptor (EGFR) related] isoforms and several kinases such as Src (Rous sarcoma), mammalian target of rapamycin (mTOR) and focal adhesion kinase (FAK), as well as caspases in PV development. Using a passive transfer mouse model of PV, we aimed to investigate the relationship between the increase in nNOS and EGFR, Src, mTOR and FAK kinase upregulation observed in PV lesions. Our results revealed a new function for nNOS, which contributes to EGFR-mediated PV acantholysis through the upregulation of Src, mTOR and FAK. In addition, we found that nNOS participates actively in PV at least in part by increasing caspase-9 and caspase-3 activities. These findings underline the important issue that in PV acantholysis, caspase activation is a nNOS-linked process downstream of Src, mTOR and FAK kinase upregulation.

Cephalalgia alopecia or nummular headache with trophic changes? A new case with prolonged follow-up.

Cephalalgia alopecia is a rare and recently described headache syndrome in which recurrent, burning head and neck pain is associated with hair loss from areas of scalp affected by the pain. We here report the case of a 33-year-old woman with continuous unilateral occipital pain and colocalized alopecia, only responsive to onabotulinumtoxin A injections. We hypothesize whether this clinical phenotype may correspond to either cephalalgia alopecia or nummular headache with trophic changes, conditions that might represent 2 manifestations of the same spectrum of disorders.