Pemphigus Herpetiformis: A Report of an Unusual Type of Pemphigus in a Three-Year-Old Female.

Pemphigus herpetiformis (PH) is a rare autoimmune blistering disorder that typically presents in adults. However, its occurrence in paediatric patients, especially in very young children, is exceedingly rare. It presents with clinical features resembling dermatitis herpetiformis (DH) and immunologic characteristics similar to pemphigus, belonging to the group of intraepidermal autoimmune bullous diseases. We present the case of a three-year-old female with a history of annular and vesicular lesions on both forearms and legs. A skin biopsy revealed epidermal acanthosis, marked spongiosis, numerous intra-epidermal blisters, and exocytosis of eosinophils and neutrophils. A superficial perivascular lymphocytic infiltrate, accompanied by eosinophils and neutrophils, was also observed in the dermis. The diagnosis was also supported by direct and indirect immunofluorescence. The patient was treated with clobetasol ointment and dapsone, which showed significant improvement in the skin lesions. This case underscores the importance of considering PH in the differential diagnosis of vesicobullous diseases in children and the need for further research to elucidate its pathogenesis and optimal management.

White Fibrous Papulosis of the Neck in a 70-Year-Old Female: A Case Report.

White fibrous papulosis of the neck (WFPN) manifests through the presence of numerous solid, persistent, and asymptomatic yellowish-white papules, displaying a distinctive asymmetrical distribution primarily localized on the neck and antecubital fossa. This case report describes the clinical presentation of a 70-year-old female diagnosed with WFPN, highlighting the significant finding of collagen fiber thickening upon histopathological analysis. Despite its predilection for specific anatomical sites, the elusive pathogenesis of WFPN adds diagnostic complexity, emphasizing the need for further research in this unique condition that generally follows a benign course.

Clinicopathological Pattern of Nonmelanoma Skin Cancer in Kuwait: A Retrospective Study.

OBJECTIVE: One in every three diagnosed malignancies is skin cancer, making it the most prevalent type of cancer in the world. As skin cancer is not commonly reported in Kuwait, this study was conducted to analyze the clinicopathological characteristics of nonmelanoma skin cancers (NMSC), primarily basal cell carcinoma (BCC) and squamous cell carcinoma (SCC), during the last 13 years in a tertiary dermatology center in Kuwait. MATERIALS AND METHODS: Data were searched for patients with NMSC, primarily BCC and SCC, from 2010 to 2022. A retrospective review was conducted and descriptive data analysis was performed. RESULTS: Of 7,645 cases, a total of 146 patients had NMSC. The patient's average age was 64.9 years. 123 cases (84.2%) had BCC, whereas 23 (15.8%) had SCC. Most of the tumors were seen on the face (35.6%), scalp (20.8%), and nose (17.8%), followed by the back (6.2%), trunk (5.5%), and ear (5.5%). Well-differentiated Cutaneous SCCs were detected in 82.6% of cases. Ulceration was observed in (21.9%) of tumors. The nodular BCC subtype was observed in 50.4% of patients. CONCLUSION: BCC is the most common type of NMSC detected in Kuwait, with the scalp and face being the most common sites of involvement. Any suspicious lesions should be biopsied to rule out skin malignancy.

The Histomorphologic Profile of Skin Diseases in Kuwait.

Background Although dermatological disorders are common in all countries, their spectrum varies greatly, with a wide histological variation. This study aimed to investigate the frequency and spectrum of different histopathological patterns of skin lesions in relation to age and gender in Kuwait. Methodology This was a retrospective descriptive study. Skin biopsy samples collected over a five-year period from 2018 to 2022 at the dermatopathology department of a tertiary dermatology center in Kuwait were included in this study. The distribution of lesions according to age and gender was analyzed. Results Of the 1,796 skin tissues reviewed, the ages ranged from one month to 93 years, with a mean age of 38.9 years. A female predominance was noted, with a female-to-male ratio of 1.8:1. Most patients belonged to the 30-39-year age group. The most frequent diagnostic categories were neoplasms and papulosquamous diseases. The five most common diseases were psoriasis, lichen planus, mycosis fungoides, benign melanocytic nevus, and epidermal inclusion cysts. The most commonly encountered diseases were similarly distributed according to gender (p > 0.05). Conclusions Neoplasms and papulosquamous lesions dominated this investigation. Therefore, understanding the genetic and environmental factors that contribute to psoriasis is crucial for developing effective treatment strategies and comprehensively managing the condition. Additionally, the community should be educated to prevent repeated unprotected ultraviolet light sun exposure and early diagnosis of any suspicious lesions to reduce the prevalence of neoplastic skin diseases. Histopathological research on cutaneous lesions is rare, with none reported from Kuwait. Our histopathology-based retrospective analysis provides a baseline for population-specific skin disease studies.

Pseudoxanthoma Elasticum With Comedones in a 12-Year-Old Female Patient: A Case Report.

Pseudoxanthoma elasticum (PXE) is a rare multisystem disease characterized by progressive calcification and disintegration of elastic fibers. The disorder is attributed to a genetic mutation occurring in the ABCC6 gene, which encodes for the ATP-binding cassette transporter C6. This gene is located on chromosome 16. Patients commonly present with cutaneous, ophthalmic, and cardiovascular manifestations. However, there is a significant degree of phenotypic diversity. The diagnosis is determined by clinical manifestations, histological analysis of the lesions, and genetic analysis. The present study includes a case report of a 12-year-old female patient who presented with a chief complaint of painless, mildly pruritic yellow papules located on her neck for a period of one year. These papules were accompanied by comedones.

Facial Granulomatous Rosacea: A Case Report.

Granulomatous rosacea is a chronic inflammatory skin disease. We present the case of a 30-year-old woman with a three-month history of erythematous monomorphic papules and nodules on the cheeks and forehead. Histopathological examinations revealed tuberculoid granulomas with multinucleated giant cells. Granulomatous rosacea should be differentiated from other similar granulomatous skin diseases such as cutaneous sarcoidosis and Lupus miliaris disseminates faciei.

Dermatomyositis Following BNT162b2 mRNA COVID-19 Vaccination.

Dermatomyositis (DM) is one of the uncommon multi-organ idiopathic inflammatory myopathies that has been reported following the hepatitis B, Influenza, tetanus toxoid, H1N1, and BCG vaccines. However, an association with the coronavirus disease 2019 (COVID-19) vaccine is yet to be reported. In this case, we present the case of a 43-year-old Asian Indian female who was diagnosed with DM 10 days after receiving the second dosage of BNT162b2 mRNA COVID-19 vaccination, in the absence of any additional triggering factors. The diagnosis was established based on physical examination, serological antibodies, magnetic resonance imaging of the muscles, skin biopsy, and electromyography. She received standard treatment for DM, including oral high doses of prednisolone, hydroxychloroquine, mycophenolate, and physiotherapy. The treatment successfully reversed skin changes and muscle weakness. This is the first reported case of classic DM complicated by interstitial lung disease following COVID-19 vaccination. More clinical and functional studies are needed to elucidate this association. Clinicians should be aware of this unexpected adverse event following COVID-19 vaccination and arrange for appropriate management.

Granulomatous reaction following bacillus Calmette-Guérin vaccination: Successful response to clarithromycin.

We report a 3-year-old girl with a delayed nontuberculous granulomatous reaction on a bacillus Calmette-Guérin injection site with dissemination to distant sites who showed a favorable response to clarithromycin used for 12 weeks with no recurrence on a follow-up of more than 2 years.

Neutrophilic Panniculitis in a child with MYSM1 deficiency.

Neutrophilic panniculitis (NP) with myelodysplasia has been described in adults but not in children. We report a case of NP associated with myelodysplasia in a child with MYSM1 deficiency, a newly described syndrome with primary immunodeficiency (PI), bone marrow failure, and developmental aberrations.

Chronic cutaneous graft-versus-host disease in children: A report of 14 patients from a tertiary care pediatric dermatology clinic.

BACKGROUND/OBJECTIVES: Allogeneic hematopoietic stem cell transplantation (HSCT) is a treatment option for many life-threatening disorders in children. Chronic graft-versus-host disease (cGVHD) is a significant complication of HSCT, and its treatment is challenging. Skin is the most common organ affected in cGVHD, with protean manifestations posing a challenge in diagnosis and management. The objective was to have a better understanding of the spectrum of chronic cutaneous GVHD (cc-GVHD) in children. METHODS: Hospital records of 14 children with cc-GVHD, registered over 9 years, were reviewed. RESULTS: All the patients had received HSCT from related donors. Median duration between HSCT and onset of cc-GVHD was 7.5 months. Eighty-six percent of the patients had a prior history of aGVHD, and 14% had de novo onset of cc-GVHD. Of 14 patients, 71% had classic cc-GVHD. Overlap syndrome was observed in 29%. Tandem occurrence of multiple morphologies was noticed in 6 (43%) patients. Of classic cc-GVHD, lichen planus-like cc-GVHD was most common (57%) followed by scleroderma-like (29%) and poikiloderma (7%). Rare variants included eczema-like (14%) and psoriasis-like (7%) cc-GVHD. Mucosal involvement was seen in 78.6% of the patients, nail involvement in 50%, and hair abnormalities in 43%. After a median follow-up of 4.8 years, complete remission was observed in 50% and mortality in 14%. CONCLUSION: The study signifies the diverse nature of cc-GVHD and indicates the need for multicenter surveys including larger number of patients to have proper insight into and develop treatment guidelines for cc-GVHD in children.

Cutaneous Lupus Erythematosus in Children: Experience from a Tertiary Care Pediatric Dermatology Clinic.

BACKGROUND/OBJECTIVES: The manifestations of cutaneous lupus erythematosus (CLE) and their relevance to systemic disease are well characterized in adults, but data are limited in children. The objective of the current study was to examine the spectrum of CLE and its relationship to systemic disease in children from a tertiary care pediatric dermatology clinic. MATERIALS AND METHODS: An analysis of 26 children with CLE registered consecutively over 14 years was performed. RESULTS: Ninety-six percent of the patients were of Arab ethnicity. They included seven (27%) cases with neonatal lupus erythematosus (LE) (71% females and 29% males). Of the other 19 children with CLE, 95% were female. The mean and median age at diagnosis was 11 years. Eighty-nine percent of the patients fulfilled the criteria for systemic LE. All patients had LE-specific lesions and 83% had LE-nonspecific manifestations. Atypical initial presentations were recorded in 28% of the patients, and 22% of the patients had the rare LE variants. Of the LE-specific manifestations, acute CLE was seen in 83%, subacute in 44%, and chronic in 22%. Autoimmune associations were recorded in 44% and a positive family history of autoimmune diseases in 61%. CONCLUSION: This study highlights a striking female predominance, higher risk of systemic disease in children presenting with CLE, higher prevalence of atypical presentation and rare CLE variants, and underrepresentation of discoid LE in children and signifies the need for more surveys to delineate the spectrum of pediatric CLE in different parts of the world.

Noninfectious cutaneous granulomas in primary immunodeficiency disorders: report from a national registry.

: The association of noninfectious cutaneous granulomas with primary immunodeficiency disorders (PIDs) is a rare but well-recognized phenomenon. With the recent advent of new classification and broadening of the list of PIDs, there is now ever-growing number of PIDs having being reported with noninfectious cutaneous granulomas or granulomatous tissue reactions. The authors observed 4 patients with cutaneous granulomas associated with PIDs that constitute 2% of total PIDs registered with them. In this report, the authors describe these 4 patients with cutaneous granulomas/granulomatous skin reactions associated one each with common variable immunodeficiency, Omenn syndrome, combined immunodeficiency, and Blau syndrome (BS), and briefly review the literature on various clinicopathological patterns of cutaneous granulomas with possible underlying pathogenetic mechanisms responsible for such tissue reactions in patients with PID.

Febrile ulceronecrotic Mucha-Habermann disease (pityriasis lichenoides et varioliformis acuta fulminans) associated with parvovirus infection.

Febrile ulceronecrotic Mucha-Habermann disease is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta, characterized by a rapidly progressive course with predominant ulceronecrotic lesions associated with fever and systemic manifestations. It carries a great morbidity and is potentially fatal. The exact pathogenesis is not clear, and it has been proposed to be the result of hypersensitivity reaction to an infection. We report a patient with febrile ulceronecrotic Mucha-Habermann disease in a 12-year-old boy in whom the condition was most likely precipitated by parvovirus infection, and he showed a favorable response to a combination of prednisolone with narrow band ultraviolet B (NB-UVB) phototherapy.

Linear immunoglobulin a bullous disease of childhood responsive to intravenous immunoglobulin monotherapy.

We report a case of linear immunoglobulin A bullous disease in a 9-year-old boy who presented with rapidly progressive severe disease and could not tolerate dapsone because of high liver enzymes within a week after a low dose of dapsone in association with an underlying fatty liver. He showed remarkable improvement with intravenous immunoglobulins used as monotherapy, with a rapid clearance and a sustained remission after stopping the treatment.

Gerodermia osteodysplastica/wrinkly skin syndrome: report of three patients and brief review of the literature.

Gerodermia osteodysplastica and wrinkly skin syndrome are rare autosomal recessive disorders. Due to the many phenotypic similarities in these two conditions, it has been proposed that they represent the same disorder. Both conditions are well delineated in the genetic literature, but despite skin involvement being a striking feature, they are rarely reported in dermatology journals. In this report, we describe three Arab children from two consanguineous families who exhibit overlapping features of gerodermia osteodysplastica and wrinkly skin syndrome. All the patients had dysmorphic facial features, wrinkled skin more marked on the hands and feet, hyperextensible joints, intrauterine growth retardation, developmental delay, congenital dislocation of hips, and osteoporosis. Our observations also support the contention that gerodermia osteodysplastica and wrinkly skin syndrome have the same clinical spectrum; however, this needs to be confirmed at the molecular level.

Unusual malignant transformation of recurrent sebaceoma. A case report.

Sebaceoma is a benign tumor composed of incompletely differentiated sebaceous cells of varying degrees of maturity. Sebaceomas was never reported as a known premalignant lesion.This is a report of a sixteen year old boy who presented with a malignant transformation of a recurrent sebaceoma which was excised twice by Moh's surgery. Excision was done with a free margin of 1 cm down to the parotid fascia. Reconstruction was performed on the same set by using cervicofascial flap extending down to the supra-clavicular area. The patient had an uneventful postoperative period apart from distal marginal necrosis of the flap, which healed nicely with conservative measures and daily dressing and was sent to our cancer centre to start his adjuvant radiotherapy.Previous literature stated that sebaceoma is a distinctive benign tumor. We have presented a case of an unusual malignant transformation of a preauricular recurrent sebaceoma. This indicates that sebaceoma does have a potential risk of malignant transformation. We believe that managing recurrent sebaceoma more aggressively with wide local excision and postoperative adjuvant radiotherapy would provide better prognosis.