pacinioma: an unusual digital tumor. case report

1
P8588 Nutritional deficiency in a fortified nation Matthew Palmer, MD, Geisinger Medical Center, Danville, PA, United States; Victor Marks, MD, Geisinger Medical Center, Danville, PA, United States Background: Nutritional deficiency is most often caused by the inadequate intake or absorption of essential dietary elements. The clinical manifestations of nutritional deficiency are diverse and subtle at times. Scurvy is characterized by perifollicular petechiae, hyperkeratosis of the hair follicles, cork screw hairs, edema, purpura, hemorrhagic gingivitis, and delayed wound healing. Case report: A 30-year-old otherwise healthy woman presented to outpatient clinic with 2 years of chronic asymptomatic erythema and scaling of the nose. She denied any past medical problems, associated symptoms, or medications. Complete review of systems was negative. Prior attempted treatments with mild topical steroids and emollients had provided no improvement. On exam she had a 2-cm erythematous, atrophic plaque with telangiectasia on the nasal tip. Focal gingival hyperplasia and poor dentition were also noted. Biopsy of nasal tip demonstrated telangiectasia and perifolliculitis. Biopsy of the gingiva demonstrated gingival hyperplasia. Laboratory evaluation was significant for vitamin C (ascorbic acid), B 2 (riboflavin), and B 3 (niacin) at undetectable levels. Basic metabolic panel and complete blood count were unremarkable. Further questioning regarding her diet revealed that she completely subsided on fast food. Staple meals consisted of ‘‘hamburgers and chicken fingers’’ and included no fruits or vegetables. After her condition failed to improve with standard education on diet modifications, a referral to a nutritionist was made. Eventually, she was lost to follow-up. Discussion: Nutritional deficiencies are most common in underdeveloped regions of the world. When observed in developed countries, it is most commonly associated with alcoholism, fad diets, chronic diseases including malignancies, institutionaliza- tion, and eating disorders. It is also seen in those with anatomic or physiologic abnormalities limiting absorption of nutrients including iatrogenic states, such as gastric bypass patients. In our experience, isolated nutritional deficiencies—for example, deficient in zinc only—is uncommon, and therefore a thorough evaluation is required. Our patient only had clinical findings of scurvy yet was found to be deficient in vitamins B 2 and B 3 as well. Conclusion: Nutritional deficiency, while uncommon in developed countries, must still be including when evaluating otherwise healthy patients in the general dermatology clinic. Commercial support: None identified. P8619 Oral mucosal and cutaneous findings in Fanconi’s anemia patients Silvia Mendez-Flores, MD, Dermatology Department. Instituto Nacional de Ciencias M edicas y Nutrici on ‘‘Salvador Zubir an,’’ Mexico D.F., Mexico; Amparo Hernandez-Salazar, MD, Instituto Nacional de Ciencias Medicas y Nutrici on, Mexico D.F., Mexico; Judith Dominguez-Cherith, MD, Instituto Nacional de Ciencias Medicas y Nutrici on, Mexico D.F., Mexico; Lilly Esquivel-Pedraza, MD, Dermatology Department. Instituto Nacional de Ciencias M edicas y Nutrici on ‘‘Salvador Zubir an,’’ Mexico D.F., Mexico Background: Fanconi anemia (FA) is a rare autosomal recessive disorder related to defective caretaker genes that is clinically characterized by congenital malforma- tions, progressive bone marrow failure, and increased incidence of malignancies, especially acute myeloid leukemia and squamous cell carcinomas of the head and neck (HNSCCs) and anogenital regions. Objective: To describe the clinical findings in 2 patients with FA seen in a tertiary care center in Mexico City, focusing on mouth and skin manifestations. Cases: A 22 year-old man was referred to the oral diseases clinic of the Dermatology Department at the INCMNSZ. At oral examination, red and white patches affecting palatal and tongue were detected, and an ulcer in right border of the tongue, which was diagnosed as squamous cell carcinoma, treated with surgical resection. A 17- year-old female patient with FA was examined in May 2013 because of a burning sensation in the mouth. Xerostomia, atrophy of the mouth mucosa mainly affecting the dorsum of the tongue, erythemathous candidosis, and angular cheilitis were seen. Discussion: FA is an inherited condition of chromosomal instability. Prevalence of oral cancer has been reported in almost 50% of patients. Cancer therapy in such patient is frequently difficult due to systemic conditions. A very close follow-up should be made in FA patients, considering the increased incidence of malignancies observed and the considerably reduced life expectancy in such patients. Commercial support: None identified. P8599 Pacinioma: An unusual digital tumor. Case report Dalia Rodriguez Acosta, MD, Instituto Nacional de Ciencias Medicas y Nutrici on ‘‘Dr. Salvadar Zubir an,’’ Mexico City, Mexico; Jose Manuel Diaz Gonzalez, MD, Instituto Nacional de Ciencias Medicas y Nutrici on ‘‘Dr. Salvadar Zubir an,’’ Mexico City, Mexico; Judith Dominguez-Cherit, MD, Instituto Nacional de Ciencias Medicas y Nutrici on ‘‘Dr. Salvadar Zubir an,’’ Mexico DF, Mexico; Julio Jasso Olivares, MD, Instituto Nacional de Ciencias Medicas y Nutrici on ‘‘Dr. Salvadar Zubir an,’’ Mexico City, Mexico; Linda Garcia Hidalgo, MD, Mexico DF, Mexico Pacinian corpuscles are mechanical receptors usually located in the palmar and plantar aponeurosis. In the subcutaneous tissues of the volar surface of the proximal phalanges, they are normally found adjacent to a nerve or tendon. Hypertrophy and hyperplasia of pacinian corpuscles in the hand are very rare, with only 34 cases previously published. We describe a case in a 50-year-old housewife who complained of progressive tenderness and pain during prehension with the right hand.A palmar tenosynovial cyst of the index finger was suspected, but we discovered a small mass along the fourth collateral nerve, consisting of an unusual grape-like structure of white granules. Postsurgical evolution was uneventful. The biopsy shown within the adipose tissue several enlarged pacinian corpuscles, some corpuscles measured up to 2.5 mm in their long axis (normal, 1-1.5 mm on paraffin- embedded material). Each corpuscle had a central axis (;15 m in diameter) surrounded by almost 35-50 lamellae (normal, no more than 15 to 35 lamellae) and was limited by a fibrous capsule. Immunohistochemical and electronmicroscopic investigations indicated that the lump replicates the structure of enlarged pacinian corpuscles and should not be considered as a real tumor, nor connected to neurofibromatosis. No recurrence was observed after surgical excision. Although local trauma was encountered in 55% of the reported cases, the pathogenesis of such a lesion is still speculative. Commercial support: None identified. P7863 Pain associated with lipomas Leigh Sutton, MD, Scott and White Texas A&M, Temple, TX, United States; Katherine Fiala, MD, Scott and White Texas A&M, Temple, TX, United States; Kathleen Nyugen, Scott and White Texas A&M, Temple, TX, United States Lipomas are common benign tumors that frequently present in the subcutaneous tissue. They may slowly change in size but are often asymptomatic. However, rarely patients with lipomas present with associated pain symptoms. Most of these cases are presented in orthopedic and neurology literature. We report 2 patients whose pain symptoms significantly improved upon excision of the adjacent lipomas. The first case is a 70-year-old female who presented with a 13 3 12 mm subcutaneous nodule on her right postauricular neck. The patient also stated she had severe episodic aching right ear pain. Brain MRI ordered by neurology was negative. Pathology of the subcutaneous nodule was consistent with lipoma. The second case is a 40-year-old female who presented to clinic with a 15 3 15 mm nodule on her glabella. The second patient also stated she had noticed new onset headaches occurring in the region near her nodule. Excision was performed and pathology revealed an intramuscular lipoma. In both cases, the patients’ pain symptoms were relieved after removal of the lipomas. Previously proposed mechanisms of pain associated with lipomas include nerve entrapment, mechanical irritation, stretching of nerve fibers, or increased pressures on soft tissue. Because of the superficial location of the patients’ lipomas described above, the etiology of pain is likely caused by pressure on surrounding soft tissues or stretching of nearby nerve fibers. Although the exact mechanism of associated pain cannot be determined, excision of lipomas may be a safe and simple treatment for pain symptoms. Commercial support: None identified. MAY 2014 JAM ACAD DERMATOL AB51

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Page 1: Pacinioma: An unusual digital tumor. Case report

P8588Nutritional deficiency in a fortified nation

Matthew Palmer, MD, Geisinger Medical Center, Danville, PA, United States;Victor Marks, MD, Geisinger Medical Center, Danville, PA, United States

Background: Nutritional deficiency is most often caused by the inadequate intake orabsorption of essential dietary elements. The clinical manifestations of nutritionaldeficiency are diverse and subtle at times. Scurvy is characterized by perifollicularpetechiae, hyperkeratosis of the hair follicles, cork screw hairs, edema, purpura,hemorrhagic gingivitis, and delayed wound healing.

Case report: A 30-year-old otherwise healthy woman presented to outpatient clinicwith 2 years of chronic asymptomatic erythema and scaling of the nose. She deniedany past medical problems, associated symptoms, or medications. Complete reviewof systems was negative. Prior attempted treatments with mild topical steroids andemollients had provided no improvement. On exam she had a 2-cm erythematous,atrophic plaque with telangiectasia on the nasal tip. Focal gingival hyperplasia andpoor dentition were also noted. Biopsy of nasal tip demonstrated telangiectasia andperifolliculitis. Biopsy of the gingiva demonstrated gingival hyperplasia. Laboratoryevaluation was significant for vitamin C (ascorbic acid), B2 (riboflavin), and B3(niacin) at undetectable levels. Basic metabolic panel and complete blood countwere unremarkable. Further questioning regarding her diet revealed that shecompletely subsided on fast food. Staple meals consisted of ‘‘hamburgers andchicken fingers’’ and included no fruits or vegetables. After her condition failed toimprove with standard education on diet modifications, a referral to a nutritionistwas made. Eventually, she was lost to follow-up.

Discussion: Nutritional deficiencies are most common in underdeveloped regions ofthe world. When observed in developed countries, it is most commonly associatedwith alcoholism, fad diets, chronic diseases including malignancies, institutionaliza-tion, and eating disorders. It is also seen in those with anatomic or physiologicabnormalities limiting absorption of nutrients including iatrogenic states, such asgastric bypass patients. In our experience, isolated nutritional deficiencies—forexample, deficient in zinc only—is uncommon, and therefore a thorough evaluationis required. Our patient only had clinical findings of scurvy yet was found to bedeficient in vitamins B2 and B3 as well.

Conclusion: Nutritional deficiency, while uncommon in developed countries, muststill be including when evaluating otherwise healthy patients in the generaldermatology clinic.

MAY 201

cial support: None identified.

Commer

P8619Oral mucosal and cutaneous findings in Fanconi’s anemia patients

Silvia Mendez-Flores, MD, Dermatology Department. Instituto Nacional deCiencias M�edicas y Nutrici�on ‘‘Salvador Zubir�an,’’ Mexico D.F., Mexico; AmparoHernandez-Salazar, MD, Instituto Nacional de Ciencias Medicas y Nutrici�on,Mexico D.F., Mexico; Judith Dominguez-Cherith, MD, Instituto Nacional deCiencias Medicas y Nutrici�on, Mexico D.F., Mexico; Lilly Esquivel-Pedraza, MD,Dermatology Department. Instituto Nacional de Ciencias M�edicas y Nutrici�on‘‘Salvador Zubir�an,’’ Mexico D.F., Mexico

Background: Fanconi anemia (FA) is a rare autosomal recessive disorder related todefective caretaker genes that is clinically characterized by congenital malforma-tions, progressive bone marrow failure, and increased incidence of malignancies,especially acute myeloid leukemia and squamous cell carcinomas of the head andneck (HNSCCs) and anogenital regions.

Objective: To describe the clinical findings in 2 patients with FA seen in a tertiarycare center in Mexico City, focusing on mouth and skin manifestations.

Cases: A 22 year-old man was referred to the oral diseases clinic of the DermatologyDepartment at the INCMNSZ. At oral examination, red and white patches affectingpalatal and tongue were detected, and an ulcer in right border of the tongue, whichwas diagnosed as squamous cell carcinoma, treated with surgical resection. A 17-year-old female patient with FA was examined in May 2013 because of a burningsensation in the mouth. Xerostomia, atrophy of the mouth mucosa mainly affectingthe dorsum of the tongue, erythemathous candidosis, and angular cheilitis wereseen.

Discussion: FA is an inherited condition of chromosomal instability. Prevalence oforal cancer has been reported in almost 50% of patients. Cancer therapy in suchpatient is frequently difficult due to systemic conditions. A very close follow-upshould be made in FA patients, considering the increased incidence of malignanciesobserved and the considerably reduced life expectancy in such patients.

cial support: None identified.

Commer

4

P8599Pacinioma: An unusual digital tumor. Case report

Dalia Rodriguez Acosta, MD, Instituto Nacional de Ciencias Medicas y Nutrici�on‘‘Dr. Salvadar Zubir�an,’’ Mexico City, Mexico; Jose Manuel Diaz Gonzalez, MD,Instituto Nacional de Ciencias Medicas y Nutrici�on ‘‘Dr. Salvadar Zubir�an,’’Mexico City, Mexico; Judith Dominguez-Cherit, MD, Instituto Nacional deCiencias Medicas y Nutrici�on ‘‘Dr. Salvadar Zubir�an,’’ Mexico DF, Mexico; JulioJasso Olivares, MD, Instituto Nacional de Ciencias Medicas y Nutrici�on ‘‘Dr.Salvadar Zubir�an,’’ Mexico City, Mexico; Linda Garcia Hidalgo, MD, Mexico DF,Mexico

Pacinian corpuscles are mechanical receptors usually located in the palmar andplantar aponeurosis. In the subcutaneous tissues of the volar surface of the proximalphalanges, they are normally found adjacent to a nerve or tendon. Hypertrophy andhyperplasia of pacinian corpuscles in the hand are very rare, with only 34 casespreviously published. We describe a case in a 50-year-old housewife whocomplained of progressive tenderness and pain during prehension with the righthand.A palmar tenosynovial cyst of the index finger was suspected, but wediscovered a small mass along the fourth collateral nerve, consisting of an unusualgrape-like structure of white granules. Postsurgical evolution was uneventful. Thebiopsy shown within the adipose tissue several enlarged pacinian corpuscles, somecorpuscles measured up to 2.5 mm in their long axis (normal, 1-1.5 mm on paraffin-embedded material). Each corpuscle had a central axis (;15 �m in diameter)surrounded by almost 35-50 lamellae (normal, no more than 15 to 35 lamellae) andwas limited by a fibrous capsule. Immunohistochemical and electronmicroscopicinvestigations indicated that the lump replicates the structure of enlarged paciniancorpuscles and should not be considered as a real tumor, nor connected toneurofibromatosis. No recurrence was observed after surgical excision. Althoughlocal traumawas encountered in 55% of the reported cases, the pathogenesis of sucha lesion is still speculative.

cial support: None identified.

Commer

P7863Pain associated with lipomas

Leigh Sutton, MD, Scott and White Texas A&M, Temple, TX, United States;Katherine Fiala, MD, Scott and White Texas A&M, Temple, TX, United States;Kathleen Nyugen, Scott and White Texas A&M, Temple, TX, United States

Lipomas are common benign tumors that frequently present in the subcutaneoustissue. They may slowly change in size but are often asymptomatic. However, rarelypatients with lipomas present with associated pain symptoms. Most of these casesare presented in orthopedic and neurology literature. We report 2 patients whosepain symptoms significantly improved upon excision of the adjacent lipomas. Thefirst case is a 70-year-old female who presented with a 13 3 12 mm subcutaneousnodule on her right postauricular neck. The patient also stated she had severeepisodic aching right ear pain. Brain MRI ordered by neurology was negative.Pathology of the subcutaneous nodule was consistent with lipoma. The second caseis a 40-year-old female who presented to clinic with a 15 3 15 mm nodule on herglabella. The second patient also stated she had noticed new onset headachesoccurring in the region near her nodule. Excision was performed and pathologyrevealed an intramuscular lipoma. In both cases, the patients’ pain symptoms wererelieved after removal of the lipomas. Previously proposed mechanisms of painassociated with lipomas include nerve entrapment, mechanical irritation, stretchingof nerve fibers, or increased pressures on soft tissue. Because of the superficiallocation of the patients’ lipomas described above, the etiology of pain is likelycaused by pressure on surrounding soft tissues or stretching of nearby nerve fibers.Although the exact mechanism of associated pain cannot be determined, excision oflipomas may be a safe and simple treatment for pain symptoms.

cial support: None identified.

Commer

J AM ACAD DERMATOL AB51