Flexor Surface Research Articles

Pruritus: An Overlooked Symptom of Spinal Tumors

Background Chronic itch is often associated with pain due to its complex nature. Neuropathic pruritus is defined as a debilitating form of chronic itching, about which not much is known to date. This condition may develop as a result of various neurological events and is associated with damage to the somatosensory nervous system, accounting for approximately 10% of chronic itching cases. Syringomyelia, transverse myelitis, radiculopathies, thoracic spine masses, brain tumors, strokes, and abscesses are among the conditions associated with neuropathic itching to date. Case presentation A 65-year-old female patient, with no known diseases and no regular medication usage, presented with a complaint of itching that had been ongoing for the past two years. Itching initially started in approximately five cm areas on the bilateral below-knee flexor surfaces. The patient, whose complaints persisted, was referred to the internal medicine outpatient clinic by dermatology. On physical examination, erythematous excoriated papules were observed in the areas affected by itching. The patient's blood sugar, liver and kidney function tests, complete blood count, erythrocyte sedimentation rate, thyroid function tests, urinalysis, stool parasitology were all normal or negative. The patient reported a sensation of coldness in the same area, was referred to neurosurgery to investigate the etiology of possible neuropathic itching. The patient's spinal imaging, revealed a spinal mass. She underwent surgery performed by a neurosurgeon, during which the spinal mass was completely removed. She reported that her itching had completely disappeared post-operatively. Conclusions The concept of itching as a variant of pain is not very new. Any damage occurring in the central nervous system or peripheral nervous system that affects the neurons responsible for transmitting and processing itch can lead to neuropathic itching. Focusing on spinal cord pathologies, any condition that damages the spinal cord may cause itching, depending on the level of damage. In cases of itching with dermatomal localization, where pain, hot or cold sensations, and paroxysmal itching are present, additional imaging methods or investigations for etiology should be performed.

Clinical characteristics and response to biological therapies for inverse psoriasis: a real-life comparison between the therapeutic effects of anti-IL-23 and anti-IL-17 agents.

Inverse psoriasis (IP) is a variant of plaque psoriasis involving flexor surfaces. A clear definition of IP is still lacking. Therapy is based on topical and systemic treatments, including classic systemic drugs and biologic agents, but a well-defined therapeutic strategy is absent. This retrospective study investigated the general characteristics of patients with IP or vulgar psoriasis and compared the effectiveness of anti-interleukin-17 or anti-interleukin-23 agents in the same groups. Second, treatment effectiveness and the demographic characteristics of IP patients treated with IL-23 and IL-17 inhibitors were also compared. IP patients were included if they had specific psoriatic involvement of the axillary, inguinal, or submammary lines, breast folds, antecubital and popliteal pits, intergluteal fold, and perianal area. Patients with vulgar plaque psoriasis and concomitant intertriginous involvement were included in the vulgar psoriasis cohort. Patients with IP were prevalently female and treated with IL-17 inhibitors compared to those with vulgar psoriasis. They also had a greater risk of drug discontinuation and subsequent therapeutical switch (32.1% vs. 18.1%, P = 0.002). At later time points, those with IP showed progressively slower achievement of PASI100 and 90 compared to the cohort with vulgar psoriasis. In the IP cohort, there was greater joint involvement in patients treated with an anti-IL-17 agent (P = 0.011), who also had a lower median age of onset (P = 0.011) compared to patients treated with an anti-IL-23 agent. Patients with IP treated with an anti-IL-23 agent initiated with a lower mean PASI and showed a slower response than patients on an anti-IL-17 agent. At later time points, progressively greater effectiveness of IL-23 inhibitors was observed compared to IL-17 inhibitors. Patients with IP responded less to biologic agents than those with vulgar psoriasis. In the IP cohort, IL-17 inhibitors had a faster onset than IL-23 inhibitors, but long-term anti-IL-23 agents seem to be associated with better outcomes.

Subcorneal pustular dermatosis: Clinical characteristics and long-term follow-up of seventeen patients

Background/Aim. Subcorneal pustular dermatosis (SPD) is a rare, relapsing vesiculopustular disease predominantly presenting on the flexor surfaces. Since data about the course and duration of the disease is limited, the aim of the study was to analyze the clinical features and long-term follow-up data on patients with SPD. Methods. A hospital database was searched for medical records of patients with SPD hospitalized at the institution between 1985 and 2014. The focus was on clinical characteristics, associated diseases, disease courses, and outcomes. Results. Seventeen patients with clinical features of SPD were analyzed ? nine females and eight males with a median age at presentation of 45 years (range 18?90 years). Follow-up data were available for 12 patients. The median follow-up time was 9.5 years (1?28 years). In order to establish a histological diagnosis, repeated biopsies were required (on average 1.7 biopsies per patient). In one male patient, IgA pemphigus was diagnosed by direct immunofluorescence. In most patients, skin eruption was widespread, while in a smaller number of patients, the changes were present only on the flexor surfaces. Dysproteinemia was evident in three patients. The disease was self-limiting in three patients; five patients had mild flare-ups occurring 1?3 times a year without the need for treatment; four patients had continuous flare-ups requiring treatment. Most patients with SPD responded positively to dapsone. Conclusion. SPD is a rare disease, and it usually occurs in a person?s fifth decade. While it is self-limiting in some patients, approximately one-third of patients require continuous therapy for continuous flare-ups.

Clinico-etiological profile of atopic dermatitis in Northeast India

Objectives: Atopic dermatitis (AD) is a chronic, inflammatory skin disorder with relapsing and remitting course affecting all age groups and has multifactorial pathogenesis. As multiple genetic and environmental factors contribute to the pathogenesis of AD, its prevalence and presentation vary among different ethnic groups residing in different geographical areas. This study was conducted to study the clinical profile and aggravating factors in cases of AD attending a tertiary hospital in Meghalaya, India, and to find a correlation of disease with serum immunoglobulin E (IgE) levels. Material and Methods: Patients suspected of AD were diagnosed on the basis of Hanifin and Rajka criteria and included in this study. All relevant demographic, anthropometric, clinical, and biochemical data were collected as per a preset pro forma. Serum IgE levels were tested for every patient using electrochemiluminescence immunoassay. Results: A total of 50 diagnosed cases of AD were included in our study with ages ranging from 5 months to 61 years, and the median age for the study population was 7.9 years. Male-to-female ratio was 1:1.17. Urban patients (66%) outnumbered rural patients and the majority of these patients (90%) belonged to the upper middle class (class II) according to the modified Kuppuswamy scale. The disease was aggravated in winters in 70%, in summer in 10%, due to wool in 48%, and due to food items in 22% of patients. Allergic rhinitis was found to be coexisting in 8% of patients, whereas asthma was found in 2% of patients. The predominant site of involvement was the face (91.9%) in children and the flexor surface (92.3%) among adults. The most common clinical presentation included pruritus (100%) and xerosis (98%). Serum IgE was raised in 58% of patients. Conclusion: Atopic dermatitis of chronic type with predominant facial involvement in children and predominant flexural involvement in adults was common in our study population. Seasonal changes, food items, and woolen clothes were common causative and exacerbating factors. Some atypical presentations included posterior thigh eczema, infra-auricular fissures, retroauricular fissures, eyelid eczema, genital dermatitis, juvenile plantar dermatoses, infranasal fissure, and follicular variant of the disease.

A new approach for reconstruction of the gunshot defect of the flexor surface of the ungual (distal) phalanx by the proper transverse branch of the digital artery: a case report of combat patient injured in the Russo-Ukrainian war

BackgroundGunshot injury to the hand is severe trauma, requiring complicated reconstruction surgery for the damaged anatomic site to restore all the hand functions. The aim of this study was to show the example of the distal phalanx reconstruction by using a flap with distal transverse digital artery (DTDA) blood supply as well as to demonstrate the utility of the audio Doppler application at the reconstruction stage in the combat patient injured in the Russo-Ukrainian war.Case presentationIn this report, we present a case of a 26-year-old service member of the Ukrainian Armed Forces delivered to the Military Medical Clinical Centre on the fourth day after the gunshot gutter shrapnel wound of the distal flexor of the 2nd digit with a gunshot fracture of the ungula (distal) and middle phalanges of the 2nd digit of the right hand along with a bone deficiency of the osseous structure of the distal and middle phalanges, volar soft tissues. The dorsal metacarpal artery (DMCA) flap is a universal variant among the tools of the reconstructive plastic surgeon engaged in reconstructing defects of the digital dorsum and flexors with a limited range of flaps. We consider this to be a key that conforms with the majority of the reconstructive principles, such as ‘analog replacement’, and which is simple, adequate, and easy for operating with a minimal sequela of the donor site.ConclusionsThe distal transverse digital artery (DTDA) could be considered for hand reconstructive surgery for repairing defects of the flexor surface of the digit injury and hands after severe gunshot injury.

Microbiological assessment of the effectiveness of standard therapy in atopic dermatitis

Background. Atopic dermatitis is an inflammatory skin disease characterized by recurrent lesions and intense pruritus. Nowadays there is a stepwise approach to the treatment of atopic dermatitis, which is defined by disease intensity and complications such as secondary skin infections. However, the current management of atopic dermatitis may not always lead to the expected outcome due to not only immune dysregulation of both adaptive and innate immunity but also imbalance of the skin microbiome.
 Aims. The aim of the study was to evaluate changes in the composition of the skin microbiome in both lesional and non-lesional skin in patients with atopic dermatitis during standard treatment.
 Materials and methods. Twenty patients with atopic dermatitis and twenty six healthy controls over 18 years old were included into the study. All microbiome samples were obtained from lesional and non-lesional skin sites of atopic dermatitis patients before and after therapy. Whereas samples from healthy controls were taken once from a flexor surface of the elbow. Species identification of clinical isolates were identified using MALDI Biotyper Sirius (Bruker Daltonics).
 Results. At baseline, the prevalence of S. aureus colonization among patients with atopic dermatitis was 34.20% in lesional skin and 32.50% in non-lesional skin. After treatment, there was a significant decrease in the prevalence of S. aureus carriage in both lesional and non-lesional skin areas (р 0.05). However, no significant difference was observed in the proportion of all other staphylococci (р 0.1). Interestingly, S. aureus was not found in healthy controls.
 Conclusions. The results of the study demonstrated the effectiveness of standard therapy for managing patients with atopic dermatitis as it had a positive impact on the skin microbial community and showed a decrease in S. aureus proportion after the treatment.

P12 A flare of dermatitis herpetiformis precipitated by etanercept

Abstract We report the case of a 79-year-old man who presented with an acute, intensely pruritic, widespread rash, which, on examination, consisted of symmetrical, haemorrhagic nodules and vesicles with some desquamation affecting the extensor surfaces of the arms, flexor surfaces of the legs and the natal cleft. He had a history of coeliac disease (for which he had adhered to a strict gluten-free diet since 1997) and rheumatoid arthritis. He had his first dose of the anti-tumour necrosis factor (TNF) agent etanercept prior to this episode to manage his arthritis. A punch biopsy for histopathology showed subepidermal blisters with a predominance of neutrophils. Eosinophils were also present to the level of the eccrine coils within the dermis, with eosinophilic flame figures but no eosinophil spongiosis at the edges of the blister. Direct immunofluorescence showed granular IgM and IgA in the dermal papillae, and a similar reaction was seen with fibrinogen. These histological findings were strongly suggestive of dermatitis herpetiformis (DH). Owing to diagnostic uncertainty on presentation, initial treatment was with high-dose topical steroids and emollient. and a yellow card was completed. Since this presentation, he has had no further flares and has not required dapsone. Multidisciplinary team discussion decided that the patient should avoid other anti-TNF treatments, but he has found relief for multiple months with rituximab and has had no cutaneous adverse events on this agent. The aetiology of DH following anti-TNF treatment is not immediately clear. Increased immunogenicity and cytokine release have been hypothesized to be part of the pathology of many adverse reactions to anti-TNF agents. It is conceivable that the cytokine release ordinarily seen in DH is replicated by the administration of anti-TNF agents. There have been many cases of anti-TNF agents leading to worsening eczema and psoriasis. Interestingly, these conditions have been found to upregulate TNF-α, a feature that is also seen in DH. This appears to be the first case described in the literature of etanercept-induced DH. A reaction with infliximab was reported in 2008, with a similar rash and histology consistent with DH, but it differed as the patient had no prior diagnosis of coeliac disease and presented after their second dose (Marakli SS, Uzun S, Ozbek S, Tuncer I. Dermatitis herpetiformis in a patient receiving infliximab for ankylosing spondylitis. Eur J Dermatol 2008; 18:88–9).

CT of pancreatic schwannoma

Schwannomas are a type of nerve sheath tumor formed by Schwann cells. They typically occur in the head and neck area, the trunk, and flexor surfaces of the upper and lower extremities. Schwannomas are often benign, and pancreatic schwannomas are extremely uncommon. Given the rarity of these tumors and their clinical similarity to other pancreatic lesions, however, pancreatic schwannomas are challenging to diagnose preoperatively. In this article, we report the case of a 69-year-old female who was diagnosed with a pancreatic schwannoma. We focus on optimizing diagnosis and management through the application of radiological imaging modalities, specifically computed tomography scans with cinematic rendering.

Ancient schwannoma: a rare retroperitoneal tumor

Schwannoma is a tumor of soft tissue, originating from Schwann cells which rarely appears in the retroperitoneal region. Retroperitoneal ancient schwannomas are rare tumors, more usually found in the head, neck and flexor surfaces of the extremities. Ancient schwannomas are a subtype of classic schwannoma with a predominance of degenerative changes like calcifications, hemosiderin deposition, interstitial fibrosis, vascular hyaline degeneration. We report a case of 60 years old female with complaints of vague pain in upper abdomen with discomfort since 6 months. Following imaging studies, retroperitoneal mass was found. The patient underwent surgery and excision of mass was done. Histopathological examination showed degenerative changes which was consistent with ancient schwannoma. No evidence of recurrence appeared during follow up period.

Schwannoma: Another dermoscopic pitfall?

Schwannoma: Another dermoscopic pitfall?

Bullous Pemphigoid in a Native American/ American Indian Patient

Globally, bullous pemphigoid affects less than fifteen people per million per year worldwide. This autoimmune disease occurs most frequently amongst those of northern European ancestry and, most predominantly, in the elderly over sixty-five. While bullous pemphigoid can present as a paraneoplastic syndrome, it can occasionally occur alone. Bullous pemphigoid typically presents with several large blister-like lesions on the skin of flexor surfaces and the trunk. These generally vary in size and stage and are formed after an autoimmune attack on the hemidesmosomes, causing a separation between the basement membrane and the basement membrane cells. Here we present, to our knowledge, the first known case of non-paraneoplastic bullous pemphigoid in an elderly Native American / American Indian patient.

Bullous Pemphigoid in a 37-Year Old Female: A Case Report and Literature Review

Bullous pemphigoid is a blistering disorder which mainly affects the geriatric population predominantly older than 70 years. It is caused by an autoimmune reaction to the hemidesmosomal proteins in basal keratinocytes, causing an inflammatory cascade and subsequent bullae formation. It is rarely encountered in infants, children, and middle-aged adults. Herein, a case of Bullous pemphigoid in a 37-year old female patient is reported. The patient presented with a three-month history of multiple serous fluid-filled tense blisters on the face, neck, trunk, flexor and extensor surfaces of the extremities up to the lower thigh, with areas of excoriation, peeling, erosion and crusting. No involvement of the mucous membranes noted. The lesions were associated with intense pruritus and pain upon rupture. Patient had no other subjective complaints and had an unremarkable past medical history. Patient was initially treated with antibiotics but noted no improvement in her condition. Histologic evaluation of the skin was done and revealed a subepidermal vesicular dermatitis with prominent neutrophilic infiltrates. No hyphae or spores were seen. With these findings, patient was admitted and treated with systemic steroids, antibiotics, and antipruritic medications. Direct Immunofluorescence was done and yielded findings consistent with the diagnosis of Bullous Pemphigoid. The overall response of the patient to therapy was satisfactory. The differentiation of Bullous pemphigoid from other subepidermal bullous diseases is important due to the potential of systemic manifestations and complications of the other diseases. The importance of clinical, histopathologic and immunologic findings in confirming the diagnosis of Bullous Pemphigoid is highlighted in this case report.

BRIGATINIB INDUCED PHOTOSENSITIVITY

<h3>Introduction</h3> Drug-induced photosensitivity is suspected when dermatitis develops in a sun-exposed distribution. Reactions occur by phototoxic, photoallergic, or immune-mediated mechanisms. Brigatinib is an oral tyrosine kinase inhibitor used in the treatment of non-small cell lung cancer. It causes skin rash in 24-40% of patients but is rarely linked with photosensitivity. We present a case of blistering dermatitis suspected secondary to brigatinib. <h3>Case Description</h3> A 65-year-old female with metastatic non-small cell lung cancer on brigatinib for one month presented with a pruritic maculopapular rash without systemic findings. She denied other symptoms or recent illnesses. Vitamin D supplement was the only other medication. Exam was notable for a maculopapular erythematous, partially blanchable rash in a malar distribution, a "V" pattern on the upper chest, flexor surface of arms, dorsal surface of hands with vesicular changes, and erythema of the vaginal labia. Laboratory workup was unremarkable including unchanged chronic mild transaminitis and no peripheral eosinophilia. The rash resolved with brigatinib cessation and mild to moderate potency topical corticosteroids over 2-3 weeks. A month later, brigatinib was reintroduced with sun exposure precautions and without recurrence of rash. <h3>Discussion</h3> We describe a case of suspected photosensitivity drug rash temporally related to use of brigatinib. Photosensitivity has been reported with signal transduction inhibitors, but only one other report is found in literature for brigatinib currently. Given the importance of treatment for this patient, brigatinib was reintroduced at full dose one month following the episode, with careful instructions regarding sun exposure, without rash recurrence.

S2790 A Sweet Response: Crohn’s Flare Leading to Sweet Syndrome

Introduction: Sweet’s syndrome, or acute febrile neutrophilic dermatosis (AFND), is a rare extraintestinal manifestation of inflammatory bowel disease (IBD). It presents with fever, malaise, and erythematous papules that are tender, edematous, and, rarely, bullous. It is associated with underlying diseases including IBD, infections, cancer, autoimmune disorders, medications, and pregnancy. Case Description/Methods: A 79-year-old male with colonic Crohn’s disease (on Infliximab), NSAID-related peptic ulcer disease, and recurrent Clostridium Difficile infection presented with sudden onset, exquisitely painful, bullous rash with fevers to 102oF. The blistering eruptions started on his left wrist and spread to his arms and legs, including extensor and flexor surfaces and soles of his feet. His laboratory results were significant for white blood cell count of 18.5 K/uL, erythrocyte sedimentation rate of 130 mm/hr, c-reactive protein of 185.5 mg/L, and positive ANA titer 1:320. Gastroenterology was consulted for concern for IBD associated dermatological manifestation such as erythema nodosum (EN) or pyoderma gangrenosum (PG); however, given atypical appearance and distribution, dermatology consultation was recommended, and punch biopsy was obtained. Skin biopsy revealed nodular dermal neutrophilic infiltrate, consistent with Sweet’s syndrome. Given association with IBD, colonoscopy was performed revealing significant inflammation with large ulcers throughout the colon. Simple Endoscopic Score for Crohn’s disease was 25 with biopsies consistent with severe chronic active colitis. He was started on IV steroids with immediate improvement in pain and flattening of lesions. Ultimately, his infectious workup was negative, and it was concluded that his presentation was from severe Crohn’s flare. (Figure) Discussion: EN and PG are commonly associated with IBD; however, Sweet’s syndrome is rarely associated with IBD itself, and can also develop as a result of immunosuppressant medications or opportunistic infections. This case of Sweet’s syndrome was unique as it was secondary to uncontrolled IBD, the lesions were bullous, and the patient was male, as classically it is more common in females. It is prudent in patients with atypical appearance of skin lesions, to consider a multidisciplinary approach with dermatology consultation to diagnose and treat appropriately. Understanding the vast array of skin manifestations of IBD is key in providing comprehensive care to IBD patients.Figure 1.: Left: Bullous lesions noted on patient's arm Right: Fingernail changes.

INTERCOSTAL NERVE SCHWANNOMA IN THE LATERAL CHEST WALL: AN UNCOMMON OCCURENCE

INTERCOSTAL NERVE SCHWANNOMA IN THE LATERAL CHEST WALL: AN UNCOMMON OCCURENCE

Z-Plasty with Skin Grafting for Post-Burn Contracture - Study of 30 Cases

Introduction: Postburn contracture is a very common problem in case of undertreated and neglected cases of burns involving the flexor surface of joints of limbs. Satisfactory treatment of postburn contracture needs surgical excision of scar followed by Z-plasty. In case of a severe contracture of joints, Z-plasty is combined with skin grafting for the tension-free treatment of post-burn contracture. Methods: The study was conducted in Sadar Hospital, Sunamgonj and several private clinics in selected districts during the period from 2008 to 2020. Thirty (30) patients with contractures involving flexor surface of Elbow, Knee, and Metacarpophalangeal joints of hands of mild to severe degrees and various durations were treated with Z-plasty plus Split thickness Skin grafts. The operated limbs were immobilized with POP casts. Perioperative Parenteral Antibiotics were prescribed along with adequate Analgesics, Autiulcerants and vitamins, and proper nutrition. Both the operative sites (Recipient and donor areas) were examined by check dressing after the 7th to 14th postoperative days. Result: 5 and 12 patients had complications like infection, flap tip necrosis, and recurrent contractures respectively. But overall results were satisfactory in terms of joint extension, mobility, cosmetic appearance, and patient satisfaction. Very naturally in case of severe contractures, full correction was not possible by single-stage operation. Conclusion: Tension-free flap must be used in patients with mild to severe contractures. Combining skin Grafting with Z-plasty is easy and has better results than Z-plasty alone. It is recommended in patients of Z-plasty with intraoperative flap tension.

Standardized uptake values and attenuation correction in 18 F-sodium fluoride PET of the equine foot and fetlock.

Maximal standardized uptake values (SUVmax ) are commonly used for the interpretation of PET studies. Limited information regarding the SUVmax of 18 F-NaF PET in horses is currently available in the literature. The goals of this retrospective secondary analysis study were to provide reference values for 18 F-NaF SUVmax in the equine distal extremity and assess the effect of attenuation correction. Nonattenuation corrected (NAC) and CT-based attenuation corrected (CTAC) SUVmax were obtained from 19 feet and 19 fetlocks. Twenty regions of interest (ROIs) were defined for the foot and 22 for the fetlock. Areas presenting abnormal uptake were excluded. The overall NAC and CTAC SUVmax were 3.6 +/- 1.5 (mean +/- sd) and 5.0 +/- 1.8 for the feet and 2.9 +/- 1.1 and 3.8 +/- 1.4 for the fetlocks, respectively. The 3 ROIs showing the highest attenuation correction were the navicular center (83.4%), navicular flexor surface (74.9%) and distal phalanx flexor surface (81.3%), whereas attenuation correction was only 5.2% at the dorsal aspect of the proximal phalanx. Significant SUVmax differences were observed between the different ROIs (P<0.0001), with the toe (CTAC SUVmax 7.7 +/- 3.7), dorsal (7.5 +/- 1.9) and central (6.1 +/- 2.2) ROIs of the distal phalanx being significantly higher than those of the other areas. This study demonstrates that attenuation correction affects SUVmax in the equine distal extremity and should be performed if CT data are available. However, as the maximal attenuation correction results in less than doubling the signal intensity, nonattenuation corrected images likely remain relevant for subjective clinical interpretation.

Etiopathological Study of 50 Patients of Cutaneous Lichen Planus in Ayurveda

Lichen planus is a distinctive dermatosis characterized by pruritic violaceous flat topped papule or plaque with a network of white lines called the Wickham’s striae. First introduced by Erasmus Wilson in 1869, it an idiopathic inflammatory, autoimmune disorder affecting the skin, the mucous membrane, hairs and nails. Sites involved in skin are the flexor surface of wrists and forearms, dorsal surface of hands, anterior aspect of lower legs, neck and pre sacral area. In Ayurveda, though there is no direct reference of cutaneous lichen planus like diseases, there are certain skin lesions mentioned under Kusthas like Charmakustha, Kitibha Kustha, Vicarchika and Alasa having signs and symptoms similar to cutaneous lichen planus. An attempt has been made to study the aetiopathogenesis of cutaneous lichen planus in Ayurveda and to evaluate the common Nidanas mentioned under Kustha, which are commonly responsible for causation of the disease. For this purpose, a study was done on 50 patients suffering from cutaneous lichen planus. The patients were selected randomly from OPD &amp; IPD; Govt. Ayurvedic College and Hospital, Guwahati, Assam. Proper history was taken in a specially designed proforma to evaluate the most common Nidanas. From this study it was found that there is a definite role of Aharaja, Viharaja and Manasik Nidanas in cutaneous Lichen planus. The Nidanas were mostly Pitta-Kapha Vardhak Ahar-Viharas like Gramya Anupa Udaka Mamsa, Ati Lavana Sevana, Diwaswapna, Vegdharana, etc. This study can be beneficial in preventing the occurrence and recurrence of lichen planus on adopting Nidana Parivarjanam.

Lichen Planus and the Vulvovaginal Gingival Syndrome. Report of a Case and Review of the Literature

<h3>Introduction</h3> Lichen planus is a dermatologic disease that affects both skin and mucosa. Cutaneous lesions of lichen planus present as small polygonal violaceous papules that present most often on the flexor surfaces of the extremities. These lesions may also be present on the vulvar skin and the hair-bearing aspects of the vulva. Lichen planus of the mucous membranes may affect buccal or gingival mucosa and the vulvar vestibule and vagina. Examination of the mouth reveals the white, lacy pattern of hyperkeratotic striae present on buccal mucosa, often bilaterally. The gingiva may be diffusely swollen, erythematous, and exhibit desquamation. In 1982 Pelisse first reported the vulvovaginal gingival syndrome and lichen planus. The main clinical manifestations of the syndrome are a triad of vulvar, vaginal and gingival lichen planus. <h3>Case Report</h3> A sixty-five year old female was referred to the oral surgeon for evaluation of red patches in her mouth. The patient reported that she had a biopsy of her vulvar for which a diagnosis of lichen sclerosis et atrophicus was made. A biopsy of her maxillary gingiva was submitted to the oral pathology laboratory and diagnosed as lichen planus. The vulvar biopsy was sent for comparison. After consultation with Dermatopathology the diagnosis of lichen planus and the vulvovaginal gingival syndrome was made. <h3>Conclusion</h3> We report a case of severe erosive vulvovaginal lichen planus that preceded the onset of gingival lichen planus by nine months. Not uncommonly, patients with severe oral lesions displayed mild genital disease and patients with asymptomatic gingival disease suffered with severe erosive vulvovaginal disease. The diagnosis of vulvovaginal gingival syndrome requires multidisciplinary evaluations by various medical specialists. We have reviewed the literature of this rather rare condition and report an additional case, with the intention of making the practicing clinician aware of this unusual clinical entity.

Abstract 60: Rickets in congenital bullous ichthyosiform erythroderma

Introduction: Congenital ichthyosiform erythroderma (CIE), also known as bullous congenitalichthyosiform erythroderma is a rare type of the ichthyosis family of skin diseases which occurs in 1 in 200,000 to 300,000 births. CIE comes under the umbrella term autosomal recessive congenital ichthyosis (ARCI), which include non-syndromic congenital ichthyosis such as harlequinichthyosis and lamellar ichthyosis. Ichthyosiform erythroderma due to keratinizing disordersmaysuppress cutaneous vitamin D synthesis, leading to vitamin D deficiency and rickets.Description: A 8 year 10 months old female child presented with poor height gain from 5 years and inward bending of knees from 5 years. h/o hyperpigmented hyperkeratotic scaly dry skinfrombirth. She was diagnosed with Bullous ichythosiform erythroderma and was prescribed emollients by a dermatologist. She is born out of non-consangienous marriage, second in birth order has an elder and youngersibling who are apparently normal. Ichythyosis present from birth more during winter season. h/o delayed dentition present. No h/o constipation or weight gain. h/o delayed motor milestones present. Language and social skills normal. o/e height is 78 cms @ < -10.8 SDS; height age <2 years weight 10 kg @ < - 6 SDS ; weight age < 2 years inward bending of knees + ; forward bending of tibia + ; ichthyosis over lower limbs, abdomen and upper limb. Her labs showed low Vit D levels of 8.7 ng/ml; calcium of 7.5 mg/dl (8- 10 mg/dl); phosphorus 2.6 mg/ml (2.5-4.8 mg/dl); elevated serum Alkaline phosphatase of 1450 IU/L; serum PTH of 181 pg/ml; normal thyroid and renal profile. She was started on oral vitamin D3 supplements and calcium supplements.Discussion: Ichthyosiform erythroderma is of two types, one inherited as an autosomal dominant and the other as an autosomal recessive trait. The recessive form is nonbullous and is associated with growth retardation, oligophrenia, spastic paralysis, genital hypoplasia, hypotrichia and shortened life expectancy. In the autosomal dominant or bullous form, however, life expectancy is not shortened, and the associated symptoms include only seborrhoea of the head an probably polydipsia. Both types begin at birth and are localized mostly on the flexor surfaces. Nonbullous congenital ichthyosiform erythroderma is characterized by prominent erythroderma and fine white, superficial semiadherent scales. The majority of affected individuals are present at birth as collodion babies. Skin histology shows hyperkeratosis, an increase in stratum corneum thickness, a normal or prominent granular layer and increased mitoses. Development of skeletal features in this condition can be explained by the fact that the maintenance of healthy skeleton requires vitamin D, the main source of which is biosynthesis in the skin. Epidermal hyperproliferation disturbs this synthesis and results in rickets.