A Case of Variant Turner Syndrome with Multiple Café-au-lait Spots / 대한피부과학회지

The phenotypic variability of variant Turner syndrome is wide, ranging from characteristic clinical features to those that are hardly distinguishable from the general population. A 4-year-old girl presented with multiple brownish macules and patches on the trunk and upper extremities as well as axillary freckles. Exome sequencing and chromosomal microarray testing revealed a microdeletion at Xp22.33p22.11 leading to a diagnosis of Turner syndrome. Here we describe an unusual case of variant Turner syndrome with multiple café-au-lait spots.

Unilateral Psoriasis on the Left Leg and Poliomyelitis of the Right Leg: A Case Report and Review of the Etiology

Psoriasis is a common immune-mediated, inflammatory skin disease. However, unilateral psoriasis is rare and few cases have been reported. A 59-year-old male with a polio-affected right leg presented with exfoliative skin on the left leg for three months. He had been treated under the diagnosis of contact dermatitis with secondary infection. After the exfoliative skin lesions improved, psoriatic papules were noted, which was ultimately diagnosed as psoriasis and successfully treated with topical steroid and vitamin D agents. We propose that the Koebner phenomenon has acted as the triggering and aggravating factor of unilateral psoriasis in this patient.

Which Areas Are Still Left in Biologics Responsive Korean Patients with Moderate to Severe Plaque Psoriasis

Background@#Psoriasis localized to certain body areas, such as the scalp, nails, palms, soles, intertriginous regions, and genital regions, is reportedly difficult to treat. @*Objective@#To investigate the biologics-resistant areas in South Korean patients with psoriasis treated with biologics. @*Methods@#The study included 50 patients with chronic moderate to severe plaque psoriasis from the Pusan National University Hospital and Chosun University Hospital between October 2019 and September 2020. The patients had at least one psoriatic lesion, were treated with biologics for more than six months, and exhibited a partial or good response (reaching a Psoriasis Area and Severity Index [PASI] score of 1~5 after biologics treatment). @*Results@#A total of 50 patients with psoriasis (32 male, mean±standard deviation 47.8±11 years), with a median PASI score of 1.8, were included. The most common biologics-resistant areas were the anterior lower leg (56.0%), followed by the knee (48.0%) and posterior lower leg (42.0%). The proportion of biologics-resistant areas were obtained for body regions traditionally considered as difficult-to-treat entities, including the fingernails (10.0%), toenails (14.0%), scalp (38.0%), palm (12.0%), sole (14.0%), and genital areas (10.0%). @*Conclusion@#This study determined the biologics-resistant areas in South Korean patients, successfully treated with biologics, in a real-world clinical setting.

Prescription Patterns of Topical Medications in Patients with Atopic Dermatitis: An Investigative Review Using Big Data from the National Health Insurance Corporation

Background@#Topical medications play a crucial role in the treatment of atopic dermatitis (AD). Topical corticosteroids (TCSs) remain the main treatment of choice and topical antibiotics have also been used. However, with the new topical calcineurin inhibitors (TCIs), the prescription patterns of topical agents have changed over time. @*Objective@#To characterize the prescription patterns of topical medications in Korean patients with AD. @*Methods@#We investigated topical medications prescribed to Korean patients with AD using the National Health Insurance Sharing System (NHISS) database over a 14-year period (2002~2015). Additionally, the potency of prescribed TCSs was compared with AD and psoriasis patients. @*Results@#The annual prescription of TCSs showed a slightly decreasing trend without significant change. In particular, in terms of steroid class, prescription of moderate-to-low potency TCSs were increased and the use of high potency TCSs were decreased. TCSs were the most commonly prescribed topical medications for AD. Tertiary hospitals had a higher prescription rate for TCIs than secondary or primary hospitals (16.2%, 3.1%, and 1.9%, respectively). Additionally, dermatologists prescribed TCIs more frequently than pediatricians and internists (4.3%, 1.2%, and 0.6%, respectively). Among TCSs, Class 5 was prescribed the most (40.6%) followed by Class 7, 6, 4, 3, 1, and 2. When we compared the potency of TCSs prescribed for AD with psoriasis patients, moderate-to-low-potency TCSs were more commonly prescribed in AD. @*Conclusion@#Prescription patterns of topical medications had changed from 2002 to 2015 and differed according to the type of institution and specialty of the physician.

Giant Cellulitis-Like Sweet Syndrome Following Varicose Vein Surgery: A Rare Variant of Sweet Syndrome Mimicking Cellulitis

Giant cellulitis-like Sweet syndrome (GCS) is the most recently defined variant of Sweet syndrome (SS) which could clinically mimic wide-spreading cellulitis. Although there has been only paucity of reports in the literature, it mostly appears at lower half of the body and histologically shows dense infiltration of neutrophils with occasional histiocytoid mononuclear cells. Although its exact etiology has not been clarified, abnormal conditions (e.g., infection, malignancy and drugs) could be related triggering factors and trauma itself can be one of the causative elements as a ‘pathergy phenomenon’. GCS could be confusing manifestation especially when appeared in postoperative condition. A 69-year-old female presented with an erythematous edematous papules and plaques on the right thigh after varicose vein surgery. Skin biopsy revealed diffuse neutrophilic infiltrates that was consistent with SS. To our knowledge, there has been no report of GCS as a postoperative complication after varicose vein surgery. Physicians should be aware of this uncommon reactive neutrophilic dermatoses mimicking infectious cutaneous disease.

A Case of Plaque Psoriasis Associated with Myasthenia Gravis and Graves’ Disease / 대한피부과학회지

Psoriasis is a common immune-mediated skin disease. Myasthenia gravis and Graves’ disease are autoimmune diseases caused by autoantibodies against receptors located at the neuromuscular junction and thyroid-stimulating hormone receptor, respectively. A 29-year-old male presented with recurrent multiple erythematous scaly papules and plaques on his legs that had persisted for 3 years. A diagnosis of plaque psoriasis was made based on clinicopathological findings. The patient had a 26-year history of ocular myasthenia gravis, for which he had not been received any specific treatment. He also had a long history of Graves’ disease. To the best of our knowledge, psoriasis with myasthenia gravis and Graves’ disease has not been reported previously in the literature. Herein, we describe our examination of the causes of concurrent psoriasis, myasthenia gravis, and Graves’ disease.

Scar Sarcoidosis: A Retrospective Investigation into Its Peculiar Clinicopathologic Presentation

Background@#Scar sarcoidosis (SS), a rare form of cutaneous sarcoidosis, develops from preexisting scars. Owing to its rarity, the clinicopathologic features and its significance in clinical prognosis have been obscure. @*Objective@#This study aimed to investigate clinical, laboratory and histopathologic findings and to clarify characteristics associated with the development of SS and systemic involvement. @*Methods@#We retrospectively assessed clinical, laboratory and histopathologic findings of SS. Clinical factors including demographics, anatomic area, number of lesion (single, multiple), presence of symptoms, latent period, injury types related to scar and the proportion of systemic involvement were investigated. @*Results@#Of the 21 patients with SS, skin lesions appeared predominantly in females (85.7%) and in the head and neck (57.1%). The mean latent period was 163.5 months and 13 patients (61.9%) had multiple lesions. Injury types were varied, with no specific type identified as associated with SS. Histologically, discrete sarcoidal granulomas surrounded by densely packed collagen bundles with a thickening of numerous fibers were observed. Ten patients (47.6%) had systemic involvement and showed significantly more of the multiple lesions, longer latent period and higher level of mean serum angiotensin-converting enzyme than those without systemic involvement. @*Conclusion@#Various causes of scar were related to SS, but no specific injury type was identified as leading to SS. Although the exact pathomechanism remains unclear, the possibility of systemic involvement could be considered when the patients have multiple lesions, longstanding scars, and elevated serum angiotensin-converting enzyme.

Venous Insufficiency is a Clear Provoker of Pigmented Purpuric Dermatosis

Background@#Pigmented purpuric dermatosis (PPD) is a chronic disorder characterized by distinct petechial hemorrhage and brownish pigmentation. The cause of PPD is unclear, but several underlying conditions are associated with it. Previous reports suggest that venous insufficiency (VI) might be related to PPD; however, a clear correlation remains unelucidated. @*Objective@#To elucidate the causal relationship between PPD and VI. @*Methods@#A total 118 patients diagnosed with PPD in the Department of Dermatology, Pusan National University Hospital from November 2006 to July 2019 were retrospectively reviewed. Doppler ultrasonography of the lower extremities was performed in 56 PPD patients, who were then divided into two groups: PPD with and without VI. We compared the clinical features between the two groups. In the PPD with VI group, we assessed the correspondence ratios between PPD and VI lateralities, and between the PPD distribution and the veins involved. @*Results@#VI was detected in 35 of the 56 patients (62.5%). The PPD with VI group was significantly associated with wider distribution, darker coloration and longer disease duration. There was a positive correlation of laterality between PPD and VI, and between PPD distribution and the vein involved. @*Conclusion@#This findings suggest that VI is a clear provoker of PPD.

A Novel Mutation in the MBTPS2 Gene Resulting in Ichthyosis Follicularis, Atrichia, and Photophobia Syndrome

Ichthyosis follicularis, atrichia, and photophobia (IFAP) syndrome is a rare genetic disorder caused by mutations in the MBTPS2 gene. It is characterized by ichthyosis and alopecia from birth. Photophobia may be present in infancy or early childhood. Its mode of inheritance is X-linked recessive; thus, it mostly affects male. The disease severity varies, ranging from mild cases limited to the skin to the severe variant involving multiple extracutaneous features. A 7-year-old boy presented with scanty hair on scalp and eyebrows at birth. On physical examination, scaly patches were observed on the whole body and spiky follicular hyperkeratotic papules were observed on the face and trunk. He also suffered from severe photophobia. Histopathological examination of the scalp showed miniaturized hair follicles without perifollicular fibrosis. Genetic analysis revealed a novel mutation in the MBTPS2 gene which was a homozygous missense mutation of c.245T>C leading to an amino-acid substitution from phenylalanine to serine (p.Phe82Ser). We diagnosed this patient with IFAP syndrome. To date, 25 pathogenic MBTPS2 gene mutations have been identified. To our knowledge, c.245T>C is a novel homozygous missense mutation in the MBTPS2 gene, which has not been reported in Human Gene Mutation Database, ClinVar Database, and Leiden Open Variation Database. Previous reports suggested genotype-phenotype correlations in the MBTPS2 gene mutations. Supported by a previous notion that genotype correlates with phenotype, this novel mutation can be a predictive factor for the mild form of IFAP syndrome, restricted to the classic symptom triad.

The Importance of Ultrasonography in the Diagnosis of Epidermal Cysts and Lipomas in the Field of Dermatology / 대한피부과학회지

Background@#Ultrasonography (US) is a simple and reliable tool that can aid in the diagnosis of benign subcutaneous lesions such as epidermal cysts and lipomas. @*Objective@#This study aimed to determine the efficacy of US for the differential diagnosis of epidermal cysts and lipomas. @*Methods@#A retrospective study of 100 patients with epidermal cysts (n=45) or lipomas (n=55) who underwent US and then received a pathological diagnosis in the dermatology department of Pusan National University Hospital was conducted. @*Results@#US and clinical impressions accurately diagnosed the epidermal cysts in 90.0% and 88.0% of the patients, respectively, and the lipomas in 92.0% and 88.0% of the patients, respectively. US combined with clinical impression significantly increased the diagnostic yield of epidermal cysts (98%) and lipomas (99%). On US, the frequency of posterior acoustic enhancement was higher for the epidermal cysts than for the lipomas (82.2% and 29.1%, respectively; p＜0.001), and striated echoes such as internal linear echogenic reflections and dark clefts were more frequently found in the lipomas than in the epidermal cysts (90.9% and 6.7%, respectively; p＜0.001). @*Conclusion@#This study implies that the diagnostic role of US can be greatly enhanced by knowledge of the clinical presentation.

CD30＋ Large-Cell Transformation of Mycosis Fungoides Treated with Brentuximab Vedotin / 대한피부과학회지

Large-cell transformation of mycosis fungoides (LCT-MF) is an advanced stage of primary cutaneous T-cell lymphoma with a poor prognosis. Therapeutic options for these patients are often limited, and so far, they are not promising. An 81-year-old woman with a previous history of mycosis fungoides presented with aggravation of generalized erythematous scaly patches and new onset of ulcerated tumor on the abdomen for 3 months.Histopathological examination revealed a dense dermal infiltrate composed of atypical large lymphocytes. Immunohistochemically, the tumor cells were positive for CD30 expression. A diagnosis of CD30＋ LCT-MF was established. She was intensively treated with methotrexate (1 month), acitretin (1 month), and rituximab with dose-modified cyclophosphamide, doxorubicin, and prednisone (1 cycle). Despite such treatments, the improvement was minimal. Subsequently, the patient was started on brentuximab vedotin, 1.8 mg/kg intravenously once every 3weeks. She responded well to brentuximab therapy, and the skin lesions completely subsided within 12 weeks of treatment.

Reconstruction of large facial defects using a combination of forehead flap and other procedures

Background@#Reconstruction of large facial defects is challenging as both functional and cosmetic results must be considered. Reconstruction with forehead flaps on the face is advantageous; nonetheless, reconstruction of large defects with forehead flaps alone results in extensive scarring on the donor site. In our study, the results of reconstruction using a combination of forehead flaps and other techniques for large facial defects were evaluated. @*Methods@#A total of 63 patients underwent reconstructive surgery using forehead flaps between February 2005 and June 2020 at our institution. Reconstruction of a large defect with forehead flaps alone has limitations; because of this, 22 patients underwent a combination of procedures and were selected as the subjects of this study. This study was retrospectively conducted by reviewing the patients’ medical records. Additional procedures included orbicularis oculi musculocutaneous (OOMC) V-Y advancement flap, cheek advancement flap, nasolabial V-Y advancement flap, grafting, and simultaneous application of two different techniques. Flap survival, complications, and recurrence of skin cancer were analyzed. Patient satisfaction was evaluated using questionnaires. @*Results@#Along with reconstructive surgery using forehead flaps, nasolabial V-Y advancement flap was performed in nine patients, local advancement flap in three, OOMC V-Y advancement flap in two, grafting in five, and two different techniques in three patients. No patient developed flap loss; however, cancer recurred in two patients. The overall patient satisfaction was high. @*Conclusion@#Reconstruction with a combination of forehead flaps and other techniques for large facial defects can be considered as both functionally and cosmetically reliable.

Clinical Features and Outcome of Juvenile Dermatomyositis: A Retrospective Study and Literature Review / 대한피부과학회지

Background@#Juvenile dermatomyositis (JDM) is a rare but common childhood idiopathic inflammatory myopathy. Proximal muscle weakness and pathognomonic skin rash, Gottron papules, and heliotrope rash are characteristic clinical features of JDM. However, clinical analysis of JDM has rarely been reported in the Korean dermatologic literature. @*Objective@#This study aimed to investigate the clinical features and outcomes of JDM in Korea and previous studies. @*Methods@#We retrospectively reviewed the medical records and clinical photographs of patients diagnosed with JDM at Pusan National University Hospital (Busan and Yangsan) for 17 years (2005∼2021). @*Results@#We encountered 12 patients with JDM (male to female ratio=7:5) with a mean age of 7.2 years. The most common clinical features were Gottron papules (100%), followed by Gottron sign (83.3%), malar rash (58.3%), heliotrope rash (41.7%), shawl sign (16.7%), calcinosis cutis (8.3%), and ulcer (8.3%). In all cases, there was no concurrent interstitial lung disease or an underlying malignancy. Only 1 of 12 patients complained of proximal muscle weakness, and four patients showed an increase in muscle enzymes in the laboratory test. The skin lesions gradually improved after systemic steroid or topical treatment in all cases. No additional proximal muscle weakness was found during the follow-up. @*Conclusion@#Although proximal muscle weakness is a common symptom in JDM, clinically amyopathic JDM is commonly found in dermatologic clinics. Regardless of subtype, all patients responded well to treatment and rarely encountered recurrence.

Omalizumab for Cyclosporine-resistant Chronic Spontaneous Urticaria:A Retrospective Study of a Single Center / 대한피부과학회지

Background@#Cyclosporine is a recommended third-line treatment for chronic spontaneous urticaria (CSU) that is resistant to H1-antihistamines according to the EAACI/GA 2 LEN/EDF/WAO guidelines for management of urticaria. However, some patients with refractory urticaria do not respond to cyclosporine or antihistamines. Omalizumab, a humanized anti-immunoglobulin E antibody, has been shown to be effective and safe for antihistamine-resistant CSU. However, there are few reports on the efficacy of omalizumab in patients with CSU who are resistant to cyclosporine. @*Objective@#To evaluate the efficacy of omalizumab in patients with cyclosporine-resistant CSU. @*Methods@#Recalcitrant CSU patients who had symptoms (seven-day urticaria activity score, UAS7≥7) despite being administered cyclosporine (3∼5 mg/kg/day) and H1-antihistamine at up to a four-fold increased dose for 4 weeks were included in this study. Omalizumab was administered at 150 mg or 300 mg by subcutaneous injection every 4 weeks. Efficacy was assessed using UAS7 12 weeks after the initial administration of omalizumab. @*Results@#A total of 28 patients (18 women, 10 men) with an average age of 43.8 years were included in the study. The mean duration of CSU was 40.0 (2∼288) months, and the mean UAS7 at baseline was 14.2 (9∼35) months. Overall, 22 patients (78.6%) showed a complete (UAS7=0) or partial response (0＜UAS7≤6) at 12 weeks. Patients who were administered 300 mg of omalizumab had a more complete response (9/15, 60%) than those who were treated with 150 mg (3/13, 23.1%). @*Conclusion@#Omalizumab is an effective therapy for CSU patients who do not respond to cyclosporine.

Clinical Characteristics of Pseudolymphoma after Ear Piercing: A Report of Four Cases and Literature Review / 대한피부과학회지

Cutaneous pseudolymphoma, also referred to as cutaneous lymphoid hyperplasia or lymphocytoma cutis, is an uncommon lymphoproliferative condition. Most lesions are idiopathic in origin; however, triggers such as tattoos, acupuncture, or venipuncture are known to be associated with pseudolymphomas. Owing to the widespread popularity of cosmetic piercings, post-piercing pseudolymphomas are being reported in recent times. Anatomically, most lesions appear on the ears and present with a variety of morphological features, although diagnosis is based on distinctive clinical findings. In this case series, we report four cases of post-piercing pseudolymphoma on the earlobe together with a literature review. We emphasize close and careful monitoring to differentiate this rare condition from other lesions of the earlobe. In view of the diverse clinical presentations and treatment approaches available for pseudolymphomas, we highlight some preliminary clinical indicators to enable early diagnosis and prompt treatment of this condition.

Infantile Hemangiomas with Minimal and Arrested Growth: Clinical Features and Treatment Outcomes with 0.5% Topical Timolol Maleate

Background@#A minority of infantile hemangiomas showing minimal or arrested growth (IH-MAGs) have been recognized in the literature. Nevertheless, the clinical features and treatment outcomes of IH-MAGs have not been well investigated. @*Objective@#This study aimed to understand the clinical characteristics of IH-MAGs better and their response to treatment with topical timolol maleate. @*Methods@#We retrospectively reviewed medical records and clinical images of patients with IH-MAGs. Treatment response with topical timolol was assessed in both IH-MAGs and classic infantile hemangiomas (IHs) groups. @*Results@#Of the 1,038 patients with IHs, only 31 (3.0%) were diagnosed with IH-MAGs. Lesions with non-proliferative components were more frequently distributed in the lower half of the body (61.5%) than those with proliferative components (16.7%). In 14 patients treated with topical timolol, the global assessment scale showed more significant and rapid improvement than in those with classic IHs. @*Conclusion@#Although the prevalence of IH-MAGs may be relatively low, understanding their clinical features will help in differential diagnosis. Furthermore, these type of lesions might be more responsive to topical timolol than classic IHs.

Infantile Hemangiomas with Minimal and Arrested Growth: Clinical Features and Treatment Outcomes with 0.5% Topical Timolol Maleate

Background@#A minority of infantile hemangiomas showing minimal or arrested growth (IH-MAGs) have been recognized in the literature. Nevertheless, the clinical features and treatment outcomes of IH-MAGs have not been well investigated. @*Objective@#This study aimed to understand the clinical characteristics of IH-MAGs better and their response to treatment with topical timolol maleate. @*Methods@#We retrospectively reviewed medical records and clinical images of patients with IH-MAGs. Treatment response with topical timolol was assessed in both IH-MAGs and classic infantile hemangiomas (IHs) groups. @*Results@#Of the 1,038 patients with IHs, only 31 (3.0%) were diagnosed with IH-MAGs. Lesions with non-proliferative components were more frequently distributed in the lower half of the body (61.5%) than those with proliferative components (16.7%). In 14 patients treated with topical timolol, the global assessment scale showed more significant and rapid improvement than in those with classic IHs. @*Conclusion@#Although the prevalence of IH-MAGs may be relatively low, understanding their clinical features will help in differential diagnosis. Furthermore, these type of lesions might be more responsive to topical timolol than classic IHs.

Cryosurgery as a Minimally Invasive Alternative Treatment for a Patient with Erosive Adenomatosis of the Nipple

Erosive adenomatosis of the nipple (EAN), also known as nipple adenoma, florid papillomatosis, or papillary adenoma of the nipple, is a benign neoplasm originating from a lactiferous duct of the breast. Although the potential for malignant change is invariably negligible, the nature of the disease is quite intractable despite several treatment methods. Surgical excision is known as the treatment of choice, but this invasive approach is generally not acceptable to the vast majority of patients due to the cosmetic outcomes. Cryosurgery could be an alternative choice to preserve the structure of the nipple-areola complex, though its application has not been studied due to the paucity of cases. A 22-year-old female presented with a unilateral, crater-like erosion of the left nipple with serosanguineous discharge. The skin biopsy revealed proliferation of tubular structures, which corresponded to EAN. She was treated with 4 sessions of cryosurgery (open cryospray with liquid nitrogen) over 6 months, and the skin lesion resolved completely without any recurrence for 12 months. Although further study is required to determine the optimal treatment regimen for EAN, cryosur-gery should be considered as an effective option to surgical excision.

Omalizumab for Cyclosporine-resistant Chronic Spontaneous Urticaria:A Retrospective Study of a Single Center / 대한피부과학회지

Background@#Cyclosporine is a recommended third-line treatment for chronic spontaneous urticaria (CSU) that is resistant to H1-antihistamines according to the EAACI/GA 2 LEN/EDF/WAO guidelines for management of urticaria. However, some patients with refractory urticaria do not respond to cyclosporine or antihistamines. Omalizumab, a humanized anti-immunoglobulin E antibody, has been shown to be effective and safe for antihistamine-resistant CSU. However, there are few reports on the efficacy of omalizumab in patients with CSU who are resistant to cyclosporine. @*Objective@#To evaluate the efficacy of omalizumab in patients with cyclosporine-resistant CSU. @*Methods@#Recalcitrant CSU patients who had symptoms (seven-day urticaria activity score, UAS7≥7) despite being administered cyclosporine (3∼5 mg/kg/day) and H1-antihistamine at up to a four-fold increased dose for 4 weeks were included in this study. Omalizumab was administered at 150 mg or 300 mg by subcutaneous injection every 4 weeks. Efficacy was assessed using UAS7 12 weeks after the initial administration of omalizumab. @*Results@#A total of 28 patients (18 women, 10 men) with an average age of 43.8 years were included in the study. The mean duration of CSU was 40.0 (2∼288) months, and the mean UAS7 at baseline was 14.2 (9∼35) months. Overall, 22 patients (78.6%) showed a complete (UAS7=0) or partial response (0＜UAS7≤6) at 12 weeks. Patients who were administered 300 mg of omalizumab had a more complete response (9/15, 60%) than those who were treated with 150 mg (3/13, 23.1%). @*Conclusion@#Omalizumab is an effective therapy for CSU patients who do not respond to cyclosporine.

Clinical Characteristics of Pseudolymphoma after Ear Piercing: A Report of Four Cases and Literature Review / 대한피부과학회지

Cutaneous pseudolymphoma, also referred to as cutaneous lymphoid hyperplasia or lymphocytoma cutis, is an uncommon lymphoproliferative condition. Most lesions are idiopathic in origin; however, triggers such as tattoos, acupuncture, or venipuncture are known to be associated with pseudolymphomas. Owing to the widespread popularity of cosmetic piercings, post-piercing pseudolymphomas are being reported in recent times. Anatomically, most lesions appear on the ears and present with a variety of morphological features, although diagnosis is based on distinctive clinical findings. In this case series, we report four cases of post-piercing pseudolymphoma on the earlobe together with a literature review. We emphasize close and careful monitoring to differentiate this rare condition from other lesions of the earlobe. In view of the diverse clinical presentations and treatment approaches available for pseudolymphomas, we highlight some preliminary clinical indicators to enable early diagnosis and prompt treatment of this condition.