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

1

READS

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.

36

Sudan J Dermatol 2007;Vol 5 (1)

Pemphigus Erythematosus with an unusual presentation

1Bakri S. El Agraa; 2Mahdi M.A. Shamad; 3Nahid A. El Bashir; 3Muna S. Jamal; 4Suzan

• I. Ayed; 4Muna O. Abd Elaziz,.

1Associate 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.

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.

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

• f 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

Sudan J Dermatol 2007;Vol 5 (1) : 36 - 39

CASE REPORT

Pemphigus erythematosus, unusual presentation … Bakri El Agraa et al

Sudan J Dermatol 2007;Vol 5 (1)

37

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. 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

• f 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

• f 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. Pemphigus erythematosus, unusual presentation … Bakri El Agraa et al

38

Sudan J Dermatol 2007;Vol 5 (1)

References

1. Mohammed El-Ghamriny. Blistering of bullous dermatosis. In: Manual of Clinical dermatology, 6th 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, 3rd 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. Pemphigus erythematosus, unusual presentation … Bakri El Agraa et al Figure 1: The patient before treatment

Sudan J Dermatol 2007;Vol 5 (1)

39

• 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.

• 16. Baker H, Ryan TJ. Generalized pustular psoriasis: a clinical and epidemiological study
• f 104 cases. Br J Dermatol 1969; 80: 771-93.

Pemphigus erythematosus, unusual presentation … Bakri El Agraa et al

View publication stats View publication stats