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Radiol Case Rep
Radiol Case Rep
Radiology Case Reports
1930-0433
Elsevier

S1930-0433(24)00672-1
10.1016/j.radcr.2024.07.086
Case Report
Dermatophytic disease: A rare case report
Ez-zaky Sara MD sara92ezzaky@gmail.com
⁎
Imrani Kaoutar MD
Boutatleb Joud MD
Moatassim Billah Nabil PHD
Nassar Ittimade PHD
Central Radiology Department, Ibn Sina Hospital, Mohammed V University, Rabat, Morocco
⁎ Corresponding author. sara92ezzaky@gmail.com
16 8 2024
11 2024
16 8 2024
19 11 49254928
1 5 2024
15 7 2024
16 7 2024
© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Dermatophytic disease is a rare condition, primarily described in North Africa. This orphan disease owes its name to Hadida and Schousboe. Although the pathophysiology remains poorly understood, it is often associated with immune deficiency and occurs in the context of high consanguinity, as in our case. We report the case of a 24-year-old man who presented a trichophytic disease due to Trichophyton rubrum, admitted for the appearance of numerous subcutaneous nodules, prompting consultation at our institution. This dermatophytic disease typically begins with recurrent ringworm of the scalp or involvement of hairless skin. The dermatophytes involved are of anthropophilic and zoophilic origin, with T. violaceum being the most frequently isolated species in ringworm cases in the Maghreb. To date, no treatment regimen has been established. However, improving immune status and using antifungals have delayed or prevented the visceral dissemination of the disease.

Keywords

Dermatophytic disease
Trichophyton rubrum
North Africa
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pmcIntroduction

Dermatophytic disease, identified as a rare condition in the late 1940s by Hadida and Schousboe, represents a chronic cutaneovisceral dermatophytosis primarily observed in North Africa. These pioneers reported the first Algerian case and contributed to defining its clinical characteristics [1]. On one hand, the presence of familial cases and consanguinity suggests an autosomal recessive transmission, while on the other hand, the chronicity and partial resistance to antifungals suggest a cellular immune deficiency. This disease primarily affects males, with the first signs typically appearing during childhood [1,2].

Case report

We present the case of a 24-year-old man,with a history of trichophytic disease caused by Trichophyton rubrum. The patient, from a consanguineous background and with no significant medical history (specifically no immunosuppression or exposure to tuberculosis), was admitted due to worsening symptoms. The disease onset occurred at age 3, initially presenting as an alopecic plaque treated as ringworm, showing improvement, but subsequent recurrences necessitated various antifungal treatments. Progression of the disease manifested with the development of numerous subcutaneous nodules, prompting consultation at our facility. Clinical examination revealed multiple subcutaneous formations in the cervical, thoracic, abdominal, and cephalic regions, some of which exhibited skin fistulization (Fig. 1). Skin biopsy with mycological analysis confirmed T. rubrum infection (Fig. 2). To assess the extent of the nodules and plan surgical intervention, a thoraco-abdomino-pelvic CT scan was performed. The scan revealed multiple soft tissue collections in the subcutaneous parietal region, Fig. 3, Fig. 4 oval in shape, with enhanced walls postcontrast injection. Some of these collections showed calcification, primarily located in the cervical, thoracic, abdominal, pelvic, and axillary regions, indicating superficial involvement without deeper tissue penetration.Fig. 1 Cervico-thoracic and cephalic subcutaneous formations, some of which are fistulized into the skin.

Fig 1:

Fig. 2 Microscopic examination showing:Fine, regular, tangled mycelial filaments with acladium microconidia

Fig 2:

Fig. 3 Axial and sagittal sections of a CT scan showing superficial subcutaneous collections without muscular extension or deep collections of impure fluid density, some of which are calcified.

Fig 3:

Fig. 4 3D reconstruction showing subcutaneous formations.

Fig 4

Our patient was placed on antifungal treatment: terbinafine 500 mg/day and griseofulvin 1g/day, combined with surgical treatment for some subcutaneous nodules as shown in the images (Fig. 1). The progress is marked by the regression and reduction in the number of subcutaneous formations and the persistence of the scaly condition.

Discussion

Dermatophytic disease is rare, with approximately fifty cases reported, almost exclusively from the Maghreb region, led by Algeria followed by Morocco, with a male predominance [1,2]. This orphan disease owes its name to Hadida and Schousboe in 1949. Although the pathophysiology remains poorly understood, it is often caused by immune deficiency transmitted by a recessive gene deficiency in CARD9, as recently described [3], often occurring in contexts of high consanguinity, as in our case.

This dermatophytic disease typically begins with recurrent ringworm of the scalp or involvement of hairless skin. Various manifestations may occur, including alopecic plaques affecting all hair-bearing areas, erythematous-scaly lesions leading to erythroderma, intense pruritus, papulonodules or even subcutaneous abscesses, palmoplantar keratoderma, and pachyonychia affecting all twenty nails. However [2,4,5], mucous membranes are typically spared.

Lymphadenopathy is found in 58.3% of cases, affecting various lymph node regions such as axillary, inguinal, maxillary, and cervical, but deep involvement is generally delayed. Lymph nodes vary in size and can become inflamed, fistulize, ulcerate, and mimic tuberculous lymphadenitis [6,7]. Late in the disease course, lesions can spread to deeper organs (bones, brain...). Four cases of cerebral involvement have been reported, 3 of which were in Algeria. In the first reported case by Hadida and Schousboe, autopsy confirmed cerebral invasion [8]. Additionally, lymphedema affecting the limbs and genital organs has been reported [9]. Delayed growth and hormonal disturbances have also been noted. These various manifestations are characterized by frequent resistance or relapse, especially upon treatment cessation, as seen in our patient.

At the epidermal level, damage can lead to hyperplasia, hyperacanthosis, parakeratosis with foci of spongiosis, and even keratinocyte necrosis [6]. However, the granuloma, present in almost all deep lesions (tubercles, nodules, subcutaneous nodules, lymph nodes, etc.), is the most suggestive feature. The granuloma may demonstrate a highly tuberculoid follicular organization centered on foci of necrosis rich in mycelial filaments, although it may sometimes be less well organized. Yeast-like formations and even mycetoma grains may be present. Parasitism of hair follicles and sweat ducts can be observed in some histological sections [6]. At the lymph node level, the architecture of the lymph node parenchyma is completely altered by the presence of large areas of tissue necrosis, significant reticular hyperplasia with Langhans giant cells surrounded by lymphocytes, plasma cells, and histiocytes [7,6].

Direct examination relies mainly on superficial and deep samples (pus from fistulas, lymph node aspirations, biopsies...), confirming the diagnosis of disseminated dermatophytosis. The dermatophytes involved are of anthropophilic origin in 84% and zoophilic in 16% of cases, with T. violaceum being the most frequently isolated species in ringworm cases in the Maghreb [10].

The disease course varies in duration [11], with an average of 25 years (ranging from 5-47 years), conditioned by the patients' immune status [6], but it remains serious, often involving the risk of death.

Imaging plays a crucial role, particularly in assessing lesions and studying the depth of skin involvement, as in our case. Soft tissue ultrasound is used to evaluate skin thickness, detect subcutaneous collections, or guide drainage of these collections. Additionally, cross-sectional imaging, such as MRI and CT scans, may be requested when dermatophytic infection extends deeply into subcutaneous tissues or when complications are suspected.

To date, no treatment regimen has been established, although improving immune status and using antifungals have delayed or prevented the visceral dissemination of the disease. Griseofulvin is the most commonly administered antifungal at a dose of 1g/day, often combined with local treatments. Azoles have been indicated as second-line treatment after griseofulvin failure, with the most commonly used being fluconazole (200-800 mg/day), ketoconazole (200 mg-800 mg/day), and itraconazole (200 mg/day). For better results, immune intervention is essential, hence the introduction of interferon-gamma alongside antifungals, albeit at a high cost. Hepatic monitoring is necessary to avoid hepatotoxicity due to prolonged use of antifungals over several years [11].

Surgical excision is indicated in cases of cerebral involvement, swellings, and large lymphadenopathy [[12], [13], [14]].

Conclusion

Dermatophytic disease is a rare condition, primarily described in North Africa, with its pathogenesis still poorly understood and carrying a reserved prognosis despite the emergence of new antifungals and immunostimulants.

Patient consent

Written informed consent was obtained from the patient for the publication of this case report.

Competing Interests: The authors have declared that no competing interests exist.
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References

1 Fatma Cheikhrouhou Fattouma Makni Ali Ayadi La maladie dermatophytique: revue de la littérature Journal de Mycologie Médicale 20 1 2010 61 69
2 Amel Souissi Nadia Ezzine Sebai Rim Benmously Mokhtar Incaf, Fazaa Becima, Chaker Emna, Kamoun Mohamed Ridha. La maladie dermatophytique: à propos d'une observation familiale tunisienne Med Trop 65 5 2005 482 486
3 Souissi, M., Karray I., Chelly, A., Zehani W., Hafsi, D. El Euch, N., et al. Mokni Maladie dermatophytique : à propos d'un cas A. https://www.sciencedirect.com/science/article/abs/pii/S0248866317304186.
4 Badreddine Hassam Karima Senouci Farida Bennouna Bachir Lazrak Abdelaziz Agoumi Maladie dermatophytique: approche épidémiologique Med Maghreb 35 5 1992 5 8 [Google Scholar]
5 Robert Baran Nabla McLoone Roderick Hay Could proximal white subungual onychomycosis be a complication of systemic spread? The lessons to be learned from maladie dermatophytique and other deep infections Br J Dermatol 153 5 2005 1023 1025 16225618
6 Boudghène Stambouli O Mérad boudia A. La maladie dermatophytique en Algérie : nouvelle observation et revue de la littérature Ann Dermatol Venereol 118 1991 17 21 2018301
7 Marton K Cherid A. Mycose généralisée due au Trichophyton verrucosum : à propos de deux cas Inter J Dermatol 12 1973 295 301
8 Hadida E Schousboe A. Aspects de la maladie dermatophytique Algerie Med  63 1959 303 337
9 Ben Salem N Ben Ismail R Tiouri H Kchouk MC Bouzouia N Zribi A La maladie dermatophytique. À propos d'un cas tunisien Bull Soc Fr Mycol Med 16 1987 277 280
10 Makni F Neji S Sellami A Cheikhrouhou F Sellami H Marrekchi S État actuel des teignes du cuir chevelu dans la région de Sfax (Tunisie) J Mycol Med 18 2008 162 165
11 Boudghène Stambouli O Belbachir A Bariout A Dermatophytic disease: 42 years after its description Ann Dermatol Venereol 129 2002 145 208
12 Souissi A Ezzine Sebai N Benmously R Mokhtar I Fazaa B Chaker E La maladie dermatophytique : à propos d'une observation familiale tunisienne Med Trop 65 2005 482 486
13 Liautaud B Marill FG. La maladie dermatophytique. Observations algériennes récentes Bull Soc Pathol Exot Filiales 77 1984 637 648 6525721
14 Bouncer F, Otsmane F, Hammoutene A, Leclou, Abida, Bouadjar B. Maladie dermatophytique avec atteinte tronculo-corticale. Ann Dermatol Venereol.
