Pemphigus Erythematosus with an unusual presentation Article in - PDF document

See discussions, stats, and author profiles for this publication at: https://www.researchgate.net/publication/272023776 Pemphigus Erythematosus with an unusual presentation Article in Sudanese Journal of Dermatology · November 2007 DOI: 10.4314/sjd.v5i1.32897 CITATION READS 1 125 6 authors , including: Mahdi M.A. Shamad University of Bahri 9 PUBLICATIONS 39 CITATIONS SEE PROFILE All content following this page was uploaded by Mahdi M.A. Shamad on 09 February 2015. The user has requested enhancement of the downloaded file.

CASE REPORT Pemphigus Erythematosus with an unusual presentation 1 Bakri S. El Agraa; 2 Mahdi M.A. Shamad; 3 Nahid A. El Bashir; 3 Muna S. Jamal; 4 Suzan I. Ayed; 4 Muna O. Abd Elaziz,. 1 Associate Professor of Dermatology, College of Medicine – Bakht El Rudha University, Sudan. General Director of Khartoum Dermatology Teaching Hospital. 2 Assistant Professor of Dermatology, College of Medicine – University of Juba, Sudan. Pemphigus erythematosus, unusual presentation … Bakri El Agraa et al Dermatologist, Khartoum Dermatology Teaching Hospital. 3 Dermatologist, Khartoum Dermatology Teaching Hospital. 4 Registrar of Dermatology, Khartoum Dermatology Hospital Correspondence: Bakri S. El Agraa, General Director Khartoum Dermatology Teaching Hospital, Khartoum – Sudan. E- mail: agraab@hotmail.com Abstract This is an unusual presentation of Pemphigus erythematosus in a 32- year old Sudanese male. The pustular eruption the patient presented with, guided to many differential diagnoses till the final diagnosis was reached. The case is presented here in details. Sudan J Dermatol 2007;Vol 5 (1) : 36 - 39 Introduction Pemphigus Erythematosus (PE ) or Senear-Usher syndrome is a localized variety of Pemphigus foliaceus (PF) largely confined to seborrheic sites. Erythematous, crusted, and erosive lesions in the “butterfly” area of the face, forehead, presternal, and interscapular regions. Despite clinical, histopathologic, and immunopathologic similarity to PF, PE may be unique, since patients have immunoglobulin and complement deposits at the dermal-epidermal junction, in addition to inter- cellular pemphigus antibody in the epidermis, and antinuclear antibodies, as is the case in lupus erythematosus. In addition, PE may be associated with thymoma and myasthenia gravis [1]. The Case report A 32- year old male presented to us with a generalized itchy skin lesions for about two years in a remission and relapse course. He has been treated as a case of psoriasis, for which different forms of treatment were given without improvement. About one year later a histological examination was performed and the picture was compatible with Senear–Usher syndrome. Dapsone 100 mg and Dexamethasone 0.5mg/kg/day were prescribed but the patient was not taking his treatment regularly. Then the condition relapsed aggressively to present this time with generalized superficial pustulations. On examination the patient was found to be anxious, irritable, with LL edema. The lesions were wide spread over the body with no specific pattern of distribution. They were in the form of superficial pustules of different sizes; some of them were over erythematous base with areas of erosions and crustation. Palms, soles, scalp, and genitalia were involved but mucous membranes 36 Sudan J Dermatol 2007;Vol 5 (1)

and nails were not affected. A Gram stain, and microscopy of the pustule contents, did not reveal any organisms, and cultures for fungi and bacteria were negative. On the other hand blood culture for bacteria was also negative. Thinking about the possibility of pustular psoriasis, subcorneal pustular dermatosis, and pustular drug eruption a new skin biopsy was taken for histopathology and the result was suggestive of pustular psoriasis. Treatment then started with methotrexate but the condition get worse and worse. At this level a new biopsy was taken and the result showed: “a blister formed intra-epidermally by separation of the granular and horny layers; the underlying epidermal cells show acantholysis with loss of intercellular bridges; the picture consistent with Pemphigus erythematosus”. The immunoflorescence was not available at the time of admission, so it was not done for this patient. The patient started on Dapsone 100 mg/day and Prednisolone 80mg/day in a tapering manner. Pemphigus erythematosus, unusual presentation … Bakri El Agraa et al All the lesions resolved and the oedema subsided and his condition improved. Discussion When we first saw this patient our differential diagnosis included: infection, subcorneal pustular eruption, pustular psoriasis, and pustular eruption due to drugs. Infection was found to be unlikely because all cultures of the pustules and stains for bacteria and fungi were negative. Cutaneous reactions to systemically administered medications are common. The combination of skin pustules and fever may be a manifestation of drug hypersensitivity rather than infection, pustular psoriasis, or subcorneal pustular dermatosis. Reports of pustular drug eruptions are infrequent. Drugs implicated include chloramphenicol, pyrimethamine, furosemide, piperazine [2], iodides and bromides [3], streptomycin [4], carbazepine [5], isoniazid [6], cephradine [7], cephalexin[8], cephazolin [9], ampicillin [10], diltiaze [11], naproxen [12], norfloxacin [13] and co-trimoxazole and bactrim [14]. So, acute generalized pustular rash accompanied by fever together with the presence of eosinophils in the dermal infiltrate is diagnostic for pustular drug eruption. This is not the case in our patient where there was no history of any offending drug and the dermal infiltrates in all biopsy specimens were devoid of eosinophils. Subcorneal pustular dermatosis occurs mainly in middle-aged women as chronic relapsing, pustular, erythematous eruption that affects the trunk, particularly in the axillae, groins and under the breasts, and may be on the abdomen. The face is never affected nor is the mucous membrane [1]. This was not the situation in our patients where the rash was wide spread involving even the face. Acute generalized pustular psoriasis was first described by Von Zumbusch [15]. This disorder can be a life-threatening medical problem with an abrupt onset. The skin involvement is distinctive and starts with a burning erythema that spreads in hours to result in large areas of fiery-red skin. Pinpoint pustules appear in clusters, peppering the red areas of skin; these pustules become confluent and form “lakes” filled with purulent fluid. Fever, generalized weakness, severe malaise, and a leukocytosis are prominent features in almost every patient. Baker and Ryan [16], found that generalize pustular psoriasis is usually preceded by psoriasis vulgaris, and that the acute pustular flare is typically recalcitrant, and requires vigorous therapy. The possibility of pustular psoriasis in this case was more likely especially in the presence of past history of psoriasis, but deterioration of his condition on treatment raise a big question. 37 Sudan J Dermatol 2007;Vol 5 (1)

Pemphigus erythematosus, unusual presentation … Bakri El Agraa et al Figure 1: The patient before treatment References 1. Mohammed El-Ghamriny. Blistering of bullous dermatosis. In: Manual of Clinical dermatology, 6 th edn. Egypt: University Book Centre, 2007:579-648. 2. MacMillan AL. Generalized pustular drug rash. Dermatologica 1973; 146: 285-91. 3. Baker H. Drug reactions. In: Rook A, Ebling FJG, Wilkinson DS, eds. Textbook of Dermatology, 3 rd edn. Oxford: Blackwell Scientific Publications,1979:1111-1149. 4. Kushimoto H, Aoki T. Toxic erythema with generalized follicular pustules caused by streptomycin. Arch Dermatol 1981; 117: 444-5. 5. Staughton RCD, Rowland-Payne CME, Harper JI, McMichen H. Toxic Pustuloderma - a new entity?. J R Soc Med 1984; 77 (Suppl. 4): 6-8. 6. Yamasaki R, Yamasaki M, Kawasaki Y, Nagasako R. Generalized pustular dermatosis caused by isoniazid. Br J Dermatol 1985; 112: 504-6. 7. Kalb RE, Grossman ME. Pustular eruption following administration of cephradine. Cutis 1986; 38-60. 8. Jackson H, Vion B, levy PM. Generlized eruptive pustular drug rash due to cephalexin. Dermatologica 1988; 177: 292-4. 9. Fayol J, Bernard P, Bonnetblanc JM. pustular eruption following administration of cefazolin: a second case report. J Am Acad Dermatol 1988; 19:571. 38 Sudan J Dermatol 2007;Vol 5 (1)

10. Beylot C, Bioulac P, Doutre MS.. Pustuloses exanthematiques aigues generalisees. A propos de 4 cas. Ann Dermatol Venereol 1980; 107: 37-48. 11. Lambbert DG, Dalac S, Beer F et al. Acute generalized exanthematous pustular dermatitis induced by diltiazem. Br J Dermatol 1988;118: 308-9. 12. Grattan CE. Generalized eruptive pustular drug rash due to naproxen. Dermatologica 1989; 179: 57-8. 13. Shelley ED, Shelley WB. The subcorneal pustular drug eruption: an example induced by norfloxacin. Cutis 1988; 42: 24-7. 14. MacDonald KJS, Green CM, Kenicer KJA. Pustular dermatosis induced by co- trimoxazole. Br Med J 1986;293: 1279-80. 15. Von Zumbusch L Psoriasis und pustuloses Exanthem. Arch f. Dermatol u. Syphilol 1910: 335-46. Pemphigus erythematosus, unusual presentation … Bakri El Agraa et al 16. Baker H, Ryan TJ. Generalized pustular psoriasis: a clinical and epidemiological study of 104 cases. Br J Dermatol 1969; 80: 771-93. 39 Sudan J Dermatol 2007;Vol 5 (1) View publication stats View publication stats