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Conference 15 28 January 2009 The Armed Forces Institute of Pathology Department of Veterinary Pathology Conference Coordinator: Todd M. Bell, DVM WEDNESDAY SLIDE CONFERENCE 2008-2009 Conference 15 28 January 2009 Conference Moderator: Michael H. Goldschmidt, BVMS, MRCVS, DACVP of only one or two layers of partly flattened keratinocytes, CASE I – T8944-07 (AFIP 3102252) interpreted as severe epidermal atrophy (Fig. 1-2), and is covered by a layer of lamellar eosinophilic material Signalment: 2-year-old female neutered Siamese cat, (lamellar orthokeratotic hyperkeratosis). Superficial and feline, (Felis catus) periadnexal dermis show a perivascular to interstitial infiltrate composed of moderate numbers of lymphocytes, History: The cat was presented to the clinician with less macrophages, neutrophils and mast cells, and a few a 9 month history of prednisolone treatment due to a eosinophils and plasma cells. Around vessels of the diagnosis of food hypersensitivity. The skin of the dorsum superficial vascular plexus there are moderate numbers resembled wet tissue paper and was sensitive to touch. of extravasated erythrocytes (haemorrhage). Dermal From the left and right thigh, blood vessels were directed collagen is extremely attenuated and shows a pale towards the lesion (figures). The practitioner excised a 13 staining in H&E stained sections. Adnexal structures x 4 x 0.3 cm skin sample, which showed several tears and demonstrate a complete lack of anagen hair follicles, and detachment of the dermis from the panniculus (Fig. 1-1). the perifollicular fibrous sheaths of many telogen follicles The sample was fixed in formalin and routinely processed are thickened. Sebaceous glands are decreased in size and for histopathological evaluation. number, interpreted as atrophy. Follicular infundibula are extended and filled with large amounts of an eosinophilic Gross Pathology: None lamellar material (infundibular hyperkeratosis). Laboratory Results: None Contributor’s Morphologic Diagnosis: 1. Skin and subcutis: Severe atrophy of epidermis and the Histopathologic Description: Haired skin: The subcutaneous panniculus with cleft formation, consistent main lesion is a complete lack of subcutaneous adipose with feline acquired skin fragility syndrome, Siamese cat, tissue which is replaced by a large clear cleft, extending feline throughout the whole tissue sample and leading to dermo- 2. Skin: Subacute suppurative dermatitis, superficial and hypodermal separation. Collagen fibres in this area have perivascular, mild a torn and stretched appearance. The epidermis consists *Sponsored by the American Veterinary Medical Association, the American College of Veterinary Pathologists, and the C. L. Davis Foundation. WSC 2008-2009 Conference 15 1-1 Haired skin, cat. There are multifocal tears which extend through the diffusely thin epidermis and dermis to the level of the panniculus with minimal associated hemorrhage. Photograph courtesy of Institut fuer Veterinaer-Pathologie, Universitaet Giessen, Giessen, Germany. Contributor’s Comment: Different disease entities metabolism.6 A primary form due to aging or unprotected are described in man and animal species with an obvious sun exposure and an iatrogenic form (after corticosteroid fragility of the skin. Ehlers-Danlos syndrome (syn. administration) are distinguished. collagen dysplasia, dermatosparaxia, cutaneous asthenia, cutis hyperelastica) as a congenital disease with abnormal An ectodermal dysplasia with a congenital skin fragility collagen synthesis has been described in man, cattle, sheep, syndrome due to a mutation in the desmosomal protein dogs, cats, mink and rabbits.4 In man, six main types of plakophilin 1 has been reported in man.2 the disease are defined due to biochemical, clinical and molecular studies.7 In addition to skin lesions abnormal Feline acquired skin fragility syndrome is a rare disease collagen synthesis leads to alterations in ligaments, joints, with marked skin lesions of multiple etiologies but without a blood vessels and cardiac valves. In cats with Ehlers- genetic background. The syndrome has been reported only Danlos syndrome skin lesions occur predominantly as in cats. It has been noted in combination with several other cutaneous asthenia or dermatosparaxia. In contrast to diseases, such as diabetes mellitus, cholangiocarcinoma or other species a remarkable joint laxity is not typical in hepatic lipidosis, and administration of different drugs.4,5,8 cats.4 Most commonly the syndrome is associated with iatrogenic or naturally occurring hyperglucocorticism.5 There is one Recently in man the usage of the term “dermatoporosis” report of acquired skin fragility syndrome associated with has been presumed for skin lesions in aged people. In this phenytoin treatment. Since phenytoin inhibits collagen syndrome an increased fragility of the skin is attributed synthesis in vitro the authors assume that cats could acquire to age-related alterations of the extracellular matrix collagen disorders during treatment with phenytoin.1 WSC 2008-2009 Conference 15 1-2 Haired skin, cat. The markedly thin epidermis in some areas is only one cell layer thick. There is a paucity of dermal adnexa and collagen, and existing collagen fibers vary greatly in thickness. (HE 400X) Clinical features of acquired skin fragility syndrome are during the discussion and described ways to differentiate striking. Affected cats show markedly thin skin which feline acquired skin fragility syndrome from Ehlers- tears with minor trauma and commonly leaves great flaps Danlos syndrome (EDS) both grossly and via histologic of loose skin. The lesions are most commonly seen at the evaluation. back. Partial alopecia occurs in most affected regions.5 Cats with feline acquired skin fragility syndrome have Differential diagnosis is not problematic since the lesions extremely thin skin resembling tissue paper that tears very are typical. The patients normally are middle-aged or easily and is not hyperextensible. In contrast to feline older and compared to Ehlers-Danlos syndrome the skin is acquired skin fragility syndrome, EDS is characterized abnormally thin but without evidence of hyperextensibility.5 clinically by the ability to stretch the skin to great lengths Histologically the lesions in Ehlers-Danlos syndrome and without tearing. The skin does not appear to be attached feline acquired skin fragility syndrome are similar or to the underlying subcutis. EDS is also a heritable indistinguishable.4 disease, whereas feline acquired skin fragility syndrome is normally secondary to endocrine disorders, neoplasia, To confirm the histopathologic diagnosis, Masson’s or improper drug administration.5 trichrome stain was performed due to described staining abnormalities (abnormal collagen fibers, presence of As the contributor stated these two entities are histologically segmental red staining defects, birefringence of polarized similar. However, Dr. Goldschmidt pointed out features light).3 Unfortunately fiber abnormalities are not limited that allow them to be differentiated histologically in to acquired skin fragility syndrome. Similar alterations most cases. In feline acquired skin fragility syndrome, can also be seen in cutaneous asthenia.3 In our case no the epidermis is thin and there is also severe dermal staining abnormalities were detectable. atrophy with marked thinning of collagen fibers. Adnexal structures may also be atrophic. In contrast, the epidermis AFIP Diagnosis: Skin: Epidermal and dermal atrophy, in EDS is generally unaffected, and the dermis may be of diffuse, marked with follicular atrophy and loss, dermal normal thickness or partially reduced in total thickness. clefting, and mild subacute dermatitis The dermal collagen is abnormally arranged with affected fibers having red cores when stained with Masson’s Conference Comment: Dr. Goldschmidt concentrated trichrome stain.5 on the gross and histologic features of this disease WSC 2008-2009 Conference 15 Contributing Institution: Institut fuer Veterinaer- Pathologie, Universitaet Giessen Frankfurter Str. 96, 35392 Giessen, Germany, http://www. vetmed.uni-giessen.de/vet-pathologie CASE II – 05-3388 (AFIP 3103426) References: Signalment: 6-year-old male Basset hound 1. Barthold SW, Kaplan BJ, Schwartz A: Reversible dermal atrophy in a cat treated with phenytoin. Vet Pathol History: There was a 3.5 x 3.5 x 1cm raised, red, 17:469-476, 1980 ulcerated subcutaneous nodule on the right dorsal elbow. 2. Ersoy-Evans S, Erkin G, Fassihi H, Chan I, Paller The lesion had been present for months, and was removed AS, Sürücü S, McGrath JA: Ectodermal dysplasia-skin because it limited the range of motion of the elbow. fragility syndrome resulting from a new homozygous mutation, 888delC, in the desmosomal protein plakophilin Gross Pathology: None 1. J Am Acad Dermatol 55:157-161, 2006 3. Fernandez CJ, Scott DW, Erb HN, Minor RR: Staining Laboratory Results: None abnormalities of dermal collagen in cats with cutaneous asthenia or acquired skin fragility as demonstrated with Histopathologic Description: The superficial Masson’s trichrome stain. Vet Dermatol 9:49-54, 1998 and deep dermis contains multiple cystic structures that 4. Ginn PE, Mansell JEKL, Rakich PM: Skin and occasionally compress or displace adnexal structures. The appendages. In: Jubb, Kennedy and Palmer’s Pathology cysts are lined by a stratified squamous epithelium. The of Domestic Animals, ed. Maxie GM, 5th ed., vol 1, pp basal layer of the epithelium frequently forms short rete 389-391, Saunders, Edinburgh, 2007 pegs that
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