Multiple Papules Research Articles

CPC06 Congenital urticaria pigmentosa mistaken for nonaccidental injury

Abstract A male infant presented to his general practitioner for a routine 6-week check. His parents reported the presence of tan–brown lesions since birth. The marks were uniform in colour, were present on the trunk, scalp and face, and were not overlying bony prominences. There was concern that these lesions represented bruising as a manifestation of nonaccidental injury (NAI), and urgent paediatric review was arranged. He was admitted for investigation of potential NAI, and dermatology consultation was sought. On examination, scattered reddish brown-to-tan macules and papules were noted on the torso and limbs, not overlying bony prominences. Darier sign was negative, although some reactive cutis marmorata was appreciable on the trunk following rubbing of multiple lesions. A diagnosis of urticaria pigmentosa (UP), or cutaneous mastocytosis, was made clinically. Skin biopsies demonstrated an infiltrate of spindled and round mast cells and scattered eosinophils within the superficial and mid-dermis, with immunohistochemistry positive for CD117, consistent with UP. Blood tests during admission showed a normal complete blood count and coagulation screen (including von Willebrand factor), normal renal and liver function, normal lactate dehydrogenase, normal immunoglobulins and normal mast cell tryptase. Cranial ultrasound and liver ultrasound were normal. Mastocytosis is a spectrum of disease characterized by pathological accumulation of mast cells in tissues, including skin, bone marrow and the gastrointestinal tract. Cutaneous mastocytosis can be further classified as maculopapular cutaneous mastocytosis, also known as UP, diffuse cutaneous mastocytosis and localized cutaneous mastocytomas. With UP, children typically present in the first 2 years of life with multiple reddish-brown macules, papules and/or plaques distributed anywhere on the body, most commonly the trunk. In darkly pigmented skin, lesions may be hyperpigmented and erythema less appreciable. Darier sign, a transient urticarial response upon gentle rubbing of the skin, is classic and supportive of diagnosis, although it is not always demonstrable. UP has rarely been reported to be misdiagnosed as NAI. Other ‘bruising’ mimics include disorders of coagulation, Valsalva petechiae, vasculitis, acute haemorrhagic oedema, incontinentia pigmenti, phytophotodermatitis, coin rubbing, spooning, cupping, Mongolian spots, morphoea, neuroblastoma and ink stains, some of which may be unfamiliar to paediatricians or dermatologists. Dermatologists have a crucial role in recognizing cutaneous manifestations of NAI in children, which commonly includes bruising, particularly in infants who are not yet mobile. While consideration of NAI is important in all children presenting for medical care, this case highlights the importance of considering underlying dermatoses to avoid unwarranted distress and unnecessary investigations.

DP22 Cutaneous lymphoid hyperplasia: clinicopathological correlation and a therapeutic option

Abstract Cutaneous reactive lymphoid hyperplasia, also known as cutaneous pseudolymphoma, is an uncommon entity in which there are skin lesions that histologically, and sometimes clinically, mimic cutaneous lymphoma but are not, both morphologically and immunohistochemically. Correlating clinical, pathological, immunohistochemical and, occasionally, molecular findings is essential for the diagnosis (Romero-Perez D, Blanes Martinez M, Encabo-Duran B. Pseudolinfomas cutaneous. Actas Dermosifiliogr 2016; 107:640–51). Although the majority of cases can be regarded as idiopathic, certain infections and inflammatory conditions can precipitate cutaneous reactive lymphoid hyperplasia, examples of which include Borrelia infection, insect bite reactions and folliculitis (Mitteldorf, C, Kempf, W. Cutaneous pseudolymphoma – a review on the spectrum and a proposal for a new classification. J Cutan Pathol 2020; 47:76–97). The first case was an 18-year-old woman who presented with deep dermal and subcutaneous nodules for the last 5 years that started on her limbs and were slightly itchy. The nodules were firm and deep, with minimal surface changes (hyperpigmentation and adherent scale) but did not ulcerate. She also had nodules involving her buccal mucosa, inner lips and soft tissue in the nasal bridge and a couple of deep nodules on the cheeks. Pathological examination showed a dense lymphocytic infiltrate with plasma cells admixed with other inflammatory cells involving the deep dermis with an extension to the dermal/subcutaneous junction. The pathological picture was in the spectrum of deep tumid lupus/lupus panniculitis overlap and cutaneous lymphoid hyperplasia. Immunohistochemical stains showed a mixture of inflammatory cells with CD3+ and CD20+ cells. Lip mucosal biopsy showed florid reactive lymphoid follicular hyperplasia. Her condition was resistant to multiple systemic treatments. Eventually, she underwent treatment with rituximab and had satisfactory results. The second patient is an elderly female who had multiple erythematous papules, plaques and nodules on her face. Pathological examination showed a dense dermal lymphocytic infiltrate with periadnexal predilection and reactive follicles that also were highlighted by CD20, with Bcl2 and CD3+ cells around germinal centres. She was diagnosed as having cutaneous reactive lymphoid hyperplasia. Topical calcineurin inhibitors were initiated, with a good response. In conclusion, this paper highlights the term cutaneous pseudolymphoma/cutaneous reactive lymphoid hyperplasia, presenting a few cases with clinicopathological correlation and exploring some uncommon treatment options.

DP15 ‘Unwell water’: the potential importance of environmental factors in skin cancer in people of colour

Abstract Ultraviolet radiation (UVR) is the most common environmental carcinogen to cause cutaneous squamous cell carcinoma (cSCC). However, other carcinogens may also play a role and should be suspected when cSCC occurs in people with skin of colour (SOC) in whom ultraviolet (UV)-induced cSCC is less common. We present the case of a South Asian female who presented with an aggressive cSCC, which we hypothesize was related to previous arsenic exposure. A 70-year-old Bangladeshi woman (skin phototype V) presented with a 4-cm diameter, tender, keratotic plaque at the base of her left thumb, which had been present for 12 years and was slowly enlarging. There was no associated lymphadenopathy. She also had multiple palmoplantar keratotic papules and two hyperkeratotic plaques in UV-protected areas on her trunk. Histology of the lesion on her thumb confirmed a cSCC with high-risk features: poorly differentiated, invasion 9.5 mm into fibromuscular tissue, perineural invasion (nerve diameter > 0.1 mm) and vascular invasion. The truncal plaques were confirmed to be SCC in situ. The cSCC was incompletely excised and subsequently showed extensive involvement of periosteal soft tissues with invasion into bone marrow. Staging computed tomography showed no evidence of nodal or distant metastatic disease. She subsequently underwent ray amputation and remains under close clinical surveillance. Given the cSCC location and her skin phototype, potential non-UV radiation (UVR) causes were explored. In view of the palmoplantar keratotic papules and SCC in situ, chronic arsenic exposure was suspected. Further questioning revealed a history of drinking well water in a rural village in Bangladesh throughout childhood and early adulthood, although exposure had stopped > 40 years ago. Arsenic exposure from contaminated groundwater was therefore considered to be the most likely diagnosis. She was started on long-term chemoprevention with acitretin. Arsenic is a potent human carcinogen and a well-recognized cause of cSCC; the latency period may be 30–40 years. Most cases are likely to be in people exposed through environmental arsenic exposure, such as contaminated groundwater, with some cases of exposure to arsenic-containing traditional medicines also reported. Carcinogenic mechanisms are uncertain but may involve dysregulation of the Hippo signalling pathway. In summary, although the incidence of UVR-related cSCC is lower, this case highlights the importance of considering risk factors, other than UVR, in cSCC arising in people with SOC. Identifying relevant carcinogens may require a detailed travel, social and cultural history and, more importantly, an awareness of the non-UVR causes of cSCC.

Dermatodaxia (wolf-Biter) of Both Hands

A 14-year-old healthy boy presented to the Dermatology clinic with asymptomatic multiple lichenified firm papules with calluses formation, and skin dyspigmentation, involving the dorsum of both hands and the distal part of the forearms (Panel A and B). The condition was associated with fine scales and sluffing of the cuticles and the proximal nail fold of several fingers (Panel C). The condition started 2 years ago, as a result of history of a frequent compulsive habit of eating and chewing the involved parts of the body without ingestion. The patient became relaxed and enjoyed himself, especially after a stressful event. He demonstrated a skin thickening in this instance without a wound or an ulcer. The compulsion affected the cuticles of several fingers and did not associate with other known body-focused repetitive performances. A diagnosis of dermatodaxia was made. Dermatodaxia describes as an individual who eats his skin. It is a body-focused repetitive behavior and is classified in the group of obsessive-compulsive disorders. Previously named wolf-biter, because angry or trapped wolves will bite themselves. Other synonyms are chewing pads and dermatophagia. The patient was referred to a psychiatrist for further behavioral management. Meanwhile, he advised keeping the involved skin clean and enclosed with dressings like bandages until it heals.

MOLLUSCUM CONTAGIOSUM AFFECTING THE LIPS

Molluscum contagiosum is a cutaneous and mucosal viral infection that can affect the oral cavity and lips on rare occasions. The aim of this study is to report a case of molluscum contagiosum affecting the lips. A 53-year-old female was referred for evaluation of multiple papules on the skin and lips. Oral and cutaneous examination revealed the presence of multiple yellowish papules in the skin of the shoulders and neck and in the vermillion border. Clinical diagnosis included molluscum contagiosum and impetigo and a biopsy of a lip lesion was performed under local anesthesia. Hematoxylin and eosin-stained histological slides showed a localized proliferation of the stratified squamous epithelium with keratinocytes presenting nuclear basophilic inclusions, compatible with molluscum contagiosum. The patient was submitted to surgical removal of additional lesions and remains in clinical follow-up. Molluscum contagiosum is a cutaneous and mucosal viral infection that can affect the oral cavity and lips on rare occasions. The aim of this study is to report a case of molluscum contagiosum affecting the lips. A 53-year-old female was referred for evaluation of multiple papules on the skin and lips. Oral and cutaneous examination revealed the presence of multiple yellowish papules in the skin of the shoulders and neck and in the vermillion border. Clinical diagnosis included molluscum contagiosum and impetigo and a biopsy of a lip lesion was performed under local anesthesia. Hematoxylin and eosin-stained histological slides showed a localized proliferation of the stratified squamous epithelium with keratinocytes presenting nuclear basophilic inclusions, compatible with molluscum contagiosum. The patient was submitted to surgical removal of additional lesions and remains in clinical follow-up.

BIRT-HOGG-DUBÉ SYNDROME: A CASE REPORT WITH ORAL MANIFESTATION

Birt-Hogg-Dubé Syndrome (BHDS) is a rare autosomal dominant disorder characterized by cutaneous papules (fibrofolliculomas, trichodiscomas, and acrochordons), pulmonary cysts, and renal tumors. A 38-year-old man presented with pain in the right upper molars. His medical history included lung cysts due to BHDS, which also affected his mother. Extraoral examination revealed multiple skin-colored dome-shaped papules on his face, neck, ears, and upper thorax. Intraorally, there was mandibular prognathism, abfraction mainly in the posterior teeth, and asymptomatic whitish papules located on the ventral tongue. The panoramic radiograph revealed bone loss around the upper molars. The oral papules were conservatively removed, and microscopic diagnosis was consistent with fibrous hyperplasia covered by stratified epithelium with prominent rete pegs. The patient was submitted to periodontal treatment and long-term follow-up was recommended. We present an illustrative case of BHDS with oral manifestation, which is considered very rare in the literature. Birt-Hogg-Dubé Syndrome (BHDS) is a rare autosomal dominant disorder characterized by cutaneous papules (fibrofolliculomas, trichodiscomas, and acrochordons), pulmonary cysts, and renal tumors. A 38-year-old man presented with pain in the right upper molars. His medical history included lung cysts due to BHDS, which also affected his mother. Extraoral examination revealed multiple skin-colored dome-shaped papules on his face, neck, ears, and upper thorax. Intraorally, there was mandibular prognathism, abfraction mainly in the posterior teeth, and asymptomatic whitish papules located on the ventral tongue. The panoramic radiograph revealed bone loss around the upper molars. The oral papules were conservatively removed, and microscopic diagnosis was consistent with fibrous hyperplasia covered by stratified epithelium with prominent rete pegs. The patient was submitted to periodontal treatment and long-term follow-up was recommended. We present an illustrative case of BHDS with oral manifestation, which is considered very rare in the literature.

Een pleister of zalf op de wonde is niet altijd voldoende, of toch?

A band-aid or ointment on the wound is not always enough, or is it? A healthy 68-year-old man presents with a progressive, ulcerated, livid swelling on the right elbow. After clinicopathological correlation, the diagnosis of a primary cutaneous anaplastic large-cell lymphoma (ALCL) is made. This entity is part of the heterogeneous group of ALCL, which is a subtype of T-cell lymphoma with a similar morphology, but a variable clinical presentation, therapy and prognosis. An ALCL usually occurs in adults, rarely in children and adolescents. Locally or scattered over several sites, large single or multiple slow-growing erythematous papules, nodules or tumours develop. These lesions may ulcerate, exudate and/or itch. Generally, the prognosis is favourable, with a 5-year survival rate of 90%. Spontaneous regression is observed in 25% of the cases. Recurrences are frequent (up to 30% of the cases), but usually remain cutaneous. If technically feasible, a solitary or localized primary cutaneous ALCL can be excised, but if inoperable, radiotherapy is initiated, as in the discussed patient. Case series also describe good responses to a low dose of methotrexate (15-20 mg per week), but recurrences are frequent and the evidence is limited. In case of multifocal skin involvement, combination chemotherapy is used.

Figurate annulare erythemas.

T. Colcott Fox (1849-1916) first introduced in 1889 the term "figurate erythemas." According to the clinical pattern, figurate erythemas are annular, circinate, concentric, polycyclic, or arciform. The most important figurate annulare erythemas are erythema annulare centrifugum, erythema marginatum, erythema gyratum repens, erythema migrans, erythema chronicum migrans, and the pediatric annular erythemas. Erythema annulare centrifugum might be due to fungal, bacterial, or viral infections or drugs. It tends to spread centrifugally while developing central clearing. The most common locations are the trunk and the proximal extremities. Individual lesions last from several days to weeks and may resolve spontaneously. Erythema marginatum is one of the criteria for the diagnosis of acute rheumatic fever, but it also might be seen as a symptom of other diseases such as hereditary angioedema with C1-inhibitor deficiency and psittacosis. The typical clinical picture is presented by serpiginous erythematous macules and plaques with central clearing and accentuated borders. Erythema gyratum repens is a figurate erythema associated with internal malignancy. It has been linked especially to lung, esophageal, and breast cancers. Erythema gyratum repens is characterized by multiple erythematous, rounded macules or papules, rapidly progressing and forming concentric bands with an unique wood-grained appearance with desquamation on the edges of the erythema. Erythema chronicum migrans is the most common sign of infection with Borrelia burgdorferi and other Borrelia species. It is characterized by a round or oval erythematous or livid macule with a central depressed or raised area on the spot of a previous tick bite. Erythema migrans grows centrifugally and slowly in a matter of days or weeks. Central clearing is observed in 60% of patients, thus forming a targetoid appearance of the lesion. Many other figurate erythemas can be observed in infancy (pediatric annular erythemas). To this group belong neonatal lupus, erythema gyratum atrophicans transiens neonatale, annular centrifugal erythema, familial annular erythema, annular erythema of infancy, eosinophilic annular erythema, and figurate neutrophilic erythema of infancy. The treatment of the various types of figurate erythemas should be etiologic, and when the underlying condition is addressed, the therapy usually is successful.

A Very Rare Case of Juvenile Dermatomyositis with Typical Cutaneous Clinical Signs

A Very Rare Case of Juvenile Dermatomyositis with Typical Cutaneous Clinical Signs

Pseudolymphoma to Lymphoma: A Case of Chronic Reactive Lymphoid Hyperplasia Transforming to Primary Cutaneous Marginal Zone Lymphoma.

Primary cutaneous marginal zone lymphoma (PCMZL) is a low-grade malignant B-cell lymphoma that originates from the skin. It often presents as erythematous solitary or multiple papules, nodules, and/or plaques. It is one of the 3 main subtypes of primary cutaneous B-cell lymphomas. PCMZLs are believed to develop from chronic antigenic stimulation such as from tick-borne bacteria, vaccines, tattoo pigment, or other foreign body. In addition, cutaneous lymphoid hyperplasia, a documented precursor to malignant PCMZL, often presents in response to areas of chronic inflammation. Cutaneous lymphoid hyperplasia and PCMZL share several clinical and histological similarities that require clinicopathologic suspicion, immunohistochemical ancillary studies, and histopathologic analysis to accurately differentiate the 2 entities. Although gene rearrangement studies have historically been of limited value in the diagnosis of PCMZL, recent studies investigating molecular markers have identified the presence of multiple genetic abnormalities that have helped to better characterize the disease and aid in diagnosis. In addition, newer studies have found associations between PCMZL and gastrointestinal disorders, including Helicobacter pylori and inflammatory bowel disorders. In this article, we describe a case of a 56-year-old patient with a history of ulcerative colitis presenting with chronic reactive lymphoid hyperplasia that transformed to primary cutaneous marginal zone lymphoma.

Electrosurgery on Giant Condyloma Acuminata

Giant Condyloma Acuminata (GCA) is a sexually transmitted infection caused by Human papillomavirus (HPV) types 6 and 11. GCA lesions can growth up to 10 cm and form like a cauliflower shape. This infection can affect patient’s quality of life. The goal of therapy in GCA is to remove all skin lesions, which can be done by surgical treatment. Many modalities have been tested in GCA cases, one of which is electrosurgery. The aim of this study was to evaluate effectiveness of electrosurgery for GCA. A 43-year-old woman came with complaints of warts appeared in the genital area since 3 months before. Examination showed multiple papules with a verrucous surface, size around 7 cm in the labia majora, labia minor and perineum. The acetowhite examination and histopathology lead to the diagnosis of condyloma acuminata. Electrosurgery (electrodesication) to remove GCA lesions divided into 2 times. Post-operative evaluation, patient did not complain of pain and erythema, edema, and pus. Electrosurgery is a therapeutic modality that can be chosen in cases of GCA. Electrosurgery, like other surgical modalities, is capable of removing the entire lesion, but the advantages of electrosurgery include not injuring large areas, minimal bleeding and a lower recurrence rate when compared to other therapeutic modalities.

Multiple hyperkeratotic papules on the dorsal feet and lower legs.

An 87-year-old Chinese man presented with a 5-year history of multiple scaly, reddish-brown hyperkeratotic papules 1–5 mm in diameter on the dorsal feet and lower legs. Dermoscopic examination under polarized light displayed an erythematous-brownish background and white scales. A biopsy specimen demonstrated hyperkeratosis, focal epidermal atrophy, slight perivascular lymphocytic infiltrate and pigment incontinence in the superficial dermis.

Congenital cutaneous mastocytosis mistaken for non-accidental injury.

A previously well male infant presented to his general practitioner (GP) for his routine 6-week check. His parents reported the presence of tan-brown lesions since birth. The marks were uniform in color, were present on the trunk, scalp, and face, and were not overlying bony prominences. The GP was concerned that these lesions represented bruising as a manifestation of non-accidental injury (NAI), and arranged an urgent review at our pediatric department. Following assessment by the pediatric team he was admitted for investigation of potential NAI, and dermatology consultation was sought. On examination, scattered reddish brown to tan macules and papules were noted on the torso and limbs, not overlying bony prominences (Figure 1A, B). Darier's sign was negative, although some reactive cutis marmorata was appreciable on the trunk following the rubbing of multiple lesions. A diagnosis of urticaria pigmentosa (UP), now known as maculopapular cutaneous mastocytosis (CM), a form of CM, was made clinically. Skin biopsies demonstrated an infiltrate of spindled and round mast cells and scattered eosinophils within the superficial and mid dermis, with immunohistochemistry positive for CD117, consistent with CM (Figure 1C–E). Blood tests during admission showed a normal complete blood count and coagulation screen (including von Willebrand factor), normal renal and liver function, normal lactate dehydrogenase, normal immunoglobulins, and normal mast cell tryptase. Cranial ultrasound and abdominal ultrasound (including liver and spleen) were normal. Mastocytosis is a spectrum of disease characterized by the pathologic accumulation of mast cells in tissues, including skin, bone marrow, and the gastrointestinal tract. The World Health Organisation has outlined three variants: CM, systemic mastocytosis, and mast cell sarcoma. CM can be further classified as maculopapular CM, also known as UP; diffuse CM; and localized cutaneous mastocytomas.1 Pediatric CM is regarded as a benign condition limited to skin, most commonly presenting as UP, which usually spontaneously regresses in puberty.2 In contrast, adult-onset mastocytosis is more often associated with systemic involvement, a persistent course, and activating mutations of the KIT gene.1 With UP, children typically present in the first two years of life with multiple reddish-brown macules, papules, and/or plaques distributed anywhere on the body, most commonly the trunk. In darkly pigmented skin, lesions may be hyperpigmented and erythema less appreciable. Darier sign, a transient urticarial response upon gentle rubbing of the skin, is classic and supportive of the diagnosis, though not always demonstrable. Treatment with antihistamines can be helpful.3 UP has rarely been reported to be misdiagnosed as NAI.4, 5 Other ‘bruising’ mimics include disorders of coagulation, Valsalva petechiae, vasculitis, acute hemorrhagic edema, incontinentia pigmenti, phytophotodermatitis, coin rubbing, spooning, cupping, Mongolian spots, morphea, neuroblastoma, and ink stains, some of which may be unfamiliar to pediatricians or dermatologists.5 CM can be differentiated from bruising by the typically monomorphic appearance; the persistence of lesions with very gradual resolution; the development of new lesions without trauma; the presence of lesions on any part of the body, not specifically overlying bony prominences; the presence of itch; and the presence of Darier sign. Dermatologists have a crucial role in recognizing cutaneous manifestations of NAI in children, which commonly includes bruising, particularly in infants who are not yet mobile.4 While consideration of NAI is important in all children presenting for medical care, this case highlights the importance of considering underlying dermatoses to avoid unwarranted distress and unnecessary investigations. Written informed consent was obtained from the patient's parent. None.

Granulomatous perioral dermatitis

Parents of patient S., born in 2018 applied with complaints of rashes on the skin of her daughter's face, accompanied by itching for a year. Topically used cream methylprednisolone aceponate and methylene blue. Objectively: the pathological process was widespread, localized on the skin of the face. On the skin of the face, congestive hyperemia, multiple miliary pustules, vesicles, already opened in places, covered with serous crusts, multiple pink papules were visualized periorally. When conducting a histological examination of the affected area of the skin of the face, characteristic signs for granulomatous dermatitis were revealed: in the follicles, foci of necrosis, macrophages with an admixture of neutrophilic leukocytes, single eosinophils and giant multinucleated cells. In the deep layers of the dermis, rough, unevenly thickened and in places multidirectional bundles of collagen fibers with signs of necrosis and the presence of giant cell granulomas, as well as numerous giant cell granulomas without necrosis, exocytosis of lymphocytes, which is characteristic of granulomatous dermatitis, were determined. The diagnosis was made: Perioral dermatitis, granulomatous form. We were prescribed a long-term oral administration of an antibiotic of the macrolide group, externally calcineurin blockers, emollients and ointments containing zinc. On the background of the ongoing treatment, the rash resolved. There was a stable remission.

A rare case of viral-associated trichodysplasia spinulosa in a patient with chronic lymphocytic leukemia.

Dear Editors, Viral-associated trichodysplasia spinulosa (VATS) is a cutaneous eruption of folliculo-centric shiny papules and alopecia that most commonly occurs in transplant recipient patients.1 Here, we present an extremely rare case of VATS in a female patient with chronic lymphocytic leukemia (CLL). An informed consent was obtained from the patient for this report. A 63-year-old female presented with a 2-month history of multiple reddish-pinkish papules on her face in the setting of long-standing CLL. Physical examination also showed leonine facies, superciliary madarosis, and an infiltrative nose. Many spiny red papules and spicules were located on her face, neck, chest, arms, and back (Fig. 1A and B). A punch biopsy of one of the lesions on the back revealed prominent trichohyalin granules with disruption of the normal follicular maturation showing basophilic inclusions within the follicular ostia consistent with VATS (Fig. 2A and B).Fig. 1.: Clinical progression of viral-associated trichodysplasia spinulosa treated with topical cidofovir. Spiny red papules and spicules concentrated on face (A) and upper back (B) found on initial presentation. Face showing dramatically decreased spicules and papules, particularly on cheek and forehead, after 9 months of treatment with topical cidofovir (C).Fig. 2.: Pathological findings of viral-associated trichodysplasia spinulosa showing unusual prominent trichohyalin granules with disruption of the normal follicular maturation showing basophilic inclusions within the follicular ostia, shown at magnification 100× (A) and 400× (B).Importantly, the patient was not on any immunosuppressive therapy when she developed the VATS lesions. The most recent treatment for her CLL had been approximately 1 year ago, when she had finished 6 cycles of bendamustine plus rituximab. Prior to that round of treatment, her small lymphocytic lymphoma had been diagnosed over 20 years ago but had not required therapy for 10 years. A complete work up for immunosuppression including HIV testing, T-cell subsets, immunoglobulins, and re-staging of small lymphocytic lymphoma was performed. She was negative for HIV-1/2 antibody/HIV-1 antigen screening. She had evidence of hypogammaglobulinemia with an IgG of 667 mg/dL and low IgG subclasses 2, 3, and 4 (72, 16, and 1.3 mg/dL, respectively). IgG subclass 1 was normal (482 mg/dL). A peripheral blood flow cytometry showed absolute T-cell lymphopenia with low CD3+ and CD3+CD4+ absolute counts (504 and 267.2/µL, respectively), but no evidence of lymphoproliferative disease. Laboratory values showed a low absolute lymphocyte count of 0.8/µL. Prior treatment of the skin lesions by the primary care physician included topical corticosteroids, topical metronidazole, and oral cephalexin, and were not effective. After the diagnosis was established, the patient was treated with 450 mg valganciclovir twice daily to target the underlying polyomavirus, but showed no improvement over 4 months. 3% topical cidofovir twice daily was then added, and the patient showed significant improvement over the following 9 months on both 450 mg valganciclovir twice daily and 3% topical cidofovir treatment (Fig. 1C). During this period, valganciclovir was discontinued by the patient for a period of 3 months due to apparent lack of efficacy but was restarted based on dermatology recommendation. The plan of care is to continue this treatment indefinitely as maintenance therapy. Although VATS has been reported frequently in transplant recipient patients and patients with acute lymphocytic leukemia and non-Hodgkin’s lymphoma, there are only 3 previously reported account of VATS in a patient with CLL in the literature.2–4 Importantly, the previously reported patient developed VATS just 2 months after ceasing fludarabine,3 whereas our patient had been off immunosuppressive agents for nearly 1 year prior to developing VATS. Thus, our unique case highlights the dermatologically significant impact of CLL-induced immunodeficiency. The etiology of the immunodeficiency caused by CLL is multifactorial, including hypogammaglobulinemia, hypocomplementemia, and altered major histocompatibility complex class II antigens on cancer cells.5 Finally, this case emphasizes the important role that topical cidofovir may have in treating patients with treatment-resistant VATS.6 Conflicts of interest None. Funding None. Study approval N/A. Author contributions RC and SB: Writing and editing manuscript. JM and JP: Interpretation of information and editing. JL: Editing manuscript, supervision.

Mycosis Fungoides: A Diagnostic Challenge

Background: Mycosis fungoides (MF) is the most frequent type of cutaneous T-cell lymphomas (CTCL), a heterogeneous group of non-Hodgkin lymphoma of T-cell origin. CTCLs accounts for about 4% of all non-Hodgkin lymphoma. MF mainly involves the skin but in advanced cases it may involve the lymph, blood and other organs. It mimics other skin disorders like erythema necroticans, leprosy, psoriasis etc which leads to delayed diagnosis and subsequent treatment. Case report: A 61 year old male patient visited the OPD of a tertiary care hospital in Eastern India with multiple reddish elevated lesions for last 6 months which later became dome shaped and then ulcerated. He did not have any fever or itching. He informed that he had been taking Homeopathic medicine previously, but the identity of the said medicine could not be confirmed. Physical examination ruled out the presence of pallor, jaundice, oedema and cyanosis. Pulse and BP were within normal range. Systemic examination was unremarkable except mild hepatomegaly. Cutaneous examination revealed multiple erythematous indurated plaques, papules and nodules over chest, abdomen, back, limbs and face. Few of the plaques present on the trunk and left arm had developed ulcers having a necrotic floors. Cervical, axillary and inguinal lymph nodes were palpable, being firm in consistency and mobile in nature. Hair, nail and mucosal examination revealed normal results. Complete blood count (CBC), peripheral blood smear(PBS), serum urea and creatinine level did not deviate from normal findings. Ultrasonography of whole abdomen showed a mildly enlarged liver. A prominent lymph node in the aortocaval groove could be appreciated on the computed tomography(CT) scan of whole abdomen. CT scan of chest revealed bilateral axillary lymphadenopathy. Erythema necroticans, sarcoidosis and CTCL were considered as the differential diagnoses. Lesional biopsy revealed irregular epidermal hyperplasia, parakeratosis and epidermotropism in the absence of spongiosis.There was infiltration of atypical lymphocytes in the epidermis, forming well developed Pautrier’s micro abscesses. The lymphocytes were tagging the dermo-epidermal junction and within the epidermis showing surrounding halo, convoluted nuclei and variable nuclear pleomorphism. Band like papillary dermal lymphoid infiltrate, dermal lymphoid fibroplasia and nodular lymphoid aggregates could be appreciated in the deep dermis. Immunohistochemical tests were performed and the immunophenotypic profile revealed positivity for CD2, CD3, CD5, CD7, CD 8 and TIA1 and negative for CD4, CD20, CD30, CD56 and granzyme B. Based on clinical presentation, histopathology and Immunohistochemical tests, a final diagnosis of MF was made. The patient was then started on systemic antibiotic therapy, due to increased risk of bacterial super infection of lesions due to skin barrier disruption. He was then referred to Oncology department for further treatment. Conclusion: In this case, the male patient was suffering from mycosis fungoides. In the background of its clinical features being similar to other skin disorders like erythema necroticans, clinicians may face a diagnostic dilemma to correctly diagnose it. Timely diagnosis and treatment improves the prognosis in most cases. Histopathological evaluation still remains the investigation of choice.

Penile Intraepithelial Neoplasia (PeIN) in a young man with the clinical appearance of Buschke Lowenstein Tumor

Background: The incidence of penile cancer in the general population is rare. Epidemiologically, penile cancer occurs less than 1% compared to other types of cancer. Penile Intraepithelial Neoplasia (PeIN) is a precancerous lesion of invasive squamous cell carcinoma characterized by dysplastic changes of squamous epithelium with the intact basement membrane. This case report aims to increase understanding of Penile Intraepithelial Neoplasia (PeIN), especially with the clinical appearance of the Buschke Lowenstein Tumor, diagnosis, examination, and appropriate management. Case Presentation: A 21-year-old Balinese man presented with a lump on his genitalia without itching, pain, and a lesion that bleeds easily, which has appeared since January 2022. He complained that the lump was getting bigger and bigger. Physical examination showed multiple hyperpigmented papules and plaques with well-defined oval and corn shapes that vary in size with a dense consistency and a verrucous surface. Laboratory tests showed negative on Hep-B and HIV tests. The patient also had a biopsy of the lump where the results showed Penile Intraepithelial Neoplasia. The patient was treated with 6 cycles of TCA 80% once a week, but it did not improve, so circumcision and surgical excision were carried out on June 2022. Fourteen days after surgery, multiple lumps appeared, recurred, and increased in number and size. On 1-month observation after the second surgery, the patient denied complaints of the genitals. Venerological status was within normal limits. Conclusion: HPV-related Penile Intraepithelial Neoplasia (Warty subtype) with the clinical appearance of Buschke Lowenstein Tumor was reported in a 21-year-old Balinese man treated with Glans Resurfacing surgical excision with satisfactory results characterized by no functional abnormalities both during urination or erection.

A patient with familial Flegel disease caused by a novel splicing variant in SPTLC1.

Flegel disease (also known as hyperkeratosis lenticular perstans, HLP) is a rare keratinization disorder characterized by multiple asymptomatic papules predominantly distributed on the distal extremities. Very recently, variants in SPTLC1, which encodes serine palmitoyltransferase, long chain base subunit-1 (SPTLC1), have been demonstrated to cause HLP. Herein, we report a patient with familial HLP caused by a novel variant in SPTLC1.

Challenging Patterns of Atypical Dermatofibromas and Promising Diagnostic Tools for Differential Diagnosis of Malignant Lesions.

Dermatofibroma (DF) or fibrous histiocytoma is one of the most frequent benign cutaneous soft-tissue lesions, characterized by a post-inflammatory tissue reaction associated with fibrosis of the dermis. Clinically DFs have a polymorphous clinical aspect from the solitary, firm, single nodules to multiple papules with a relatively smooth surface. However, multiple atypical clinicopathological variants of DFs have been reported and, therefore, clinical recognition may become challenging, leading to a more burdensome identification and sometimes to misdiagnosis. Dermoscopy is considered an important tool in DFs diagnosis, as it improves diagnostic accuracy for clinically amelanotic nodules. Although typical dermoscopic patterns are most frequently seen in clinical practice, there have also been some atypical variants described, mimicking some underlying recurrent and sometimes harmful skin afflictions. Usually, no treatment is required, although an appropriate work-up may be necessary in specific cases, such as in the presence of atypical variants or a history of recent changes. This narrative review's aim is to summarize current evidence regarding clinical presentation, positive and differential diagnosis of atypical dermatofibromas and also to raise awareness about the importance of specific characteristics of atypical variants to better differentiate them from malignant conditions.

Case Report: The Effect of 25% Podophyllin Tincture in Condylomata Accuminata

Introduction: Condylomata accuminata (CA) is one of the most common sexually transmitted infections in the genital area caused by Human Papilloma Virus (HPV) variants 6 and 11. This disease is also known as genital warts. The potential for recurrence is relatively high and the risk for malignancy is high, therefore it requires appropriate treatment and therapy. There is no clear evidence regarding the best treatment choice for patients with CA. Case Presentation: Female, 24 years old with complaints of genital warts that have grown bigger since 1 month before. Physiological examination of the labia minora showed multiple papules with mucosal-like color and verrucous surfaces, varying in shape and size. The acetowhite test result was positive. Patients were treated with 25% podophyllin tincture, with an interval of one week one time. Conclusion: Podophyllin usage as a therapy for CA provides excellent therapeutic response and is relatively affordable, simple, and has minimal side effects. Patients treated with 25% podophyllin tincture, with an interval of one week one time, for two weeks showed significant improvement of the lesions.